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Cao L, Liu E, Yuan X, Dong Y, Yang B, Li H. Clinical characteristics and potential pathology of Moyamoya disease combined with psychiatric disorders in Chinese population - A cross-sectional study. J Affect Disord 2025; 381:1-8. [PMID: 39892754 DOI: 10.1016/j.jad.2025.01.134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2024] [Revised: 12/19/2024] [Accepted: 01/26/2025] [Indexed: 02/04/2025]
Abstract
OBJECTIVE This study aims to identify the clinical characteristics of Moyamoya disease (MMD) combined with psychiatric disorders and explore the potential pathogenesis. MATERIAL AND METHODS Psychiatric disorders between 88 MMD patients and 72 health controls were investigated using psychological testing scales. We subsequently analyzed the clinical features of 72 MMD patients with psychiatric comorbidities retrospectively. Finally, neurotransmitters analysis was conducted to further explore the pathogenesis. RESULTS Results of self-testing scales revealed a significantly higher susceptibility of psychiatric disorders in MMD patients. Compared with MMD group, clinical features of combined group demonstrated significant differences in age (51.3 ± 9.6 vs. 40.9 ± 10.4, p = 0.000), gender of female (68.1 % vs. 51.3 %, p = 0.018), duration time (33.3 ± 44.6mon vs. 8.1 ± 15.3mon, p = 0.000), cerebral infarction (55.2 % vs. 70.8 %, p = 0.025), initial type, Suzuki stage and mRS scores (p < 0.05). The perfusion results revealed significantly decreased time to peak in frontal parietal lobe (1854.14 ± 238.62 vs. 1242.79 ± 115.99, p = 0.025), temporal occipital lobe (1721.55 ± 245.15 vs. 1165.71 ± 111.55, p = 0.040), lateral ventricle (1840.03 ± 256.88 vs. 1221.95 ± 115.53, p = 0.028), and the cerebral blood volume of temporal occipital lobe was found significantly decreased (300.36 ± 34.93 vs. 403.23 ± 19.70, p = 0.026), and remarkable lower incidence of hyperperfusion syndrome and subdural effusion were demonstrated. Differential neurotransmitters of decreased 3-htdroxybutyric acid expression and increased sarcosine, tyrosine, betaine aldehyde chloride, kynurenic acid, glycine, succinic acid and lysine were identified in combined group. CONCLUSIONS Patients of MMD combined with psychiatric disorders presented unique clinical characteristics. Neurotransmitters disorder may be involved in the pathogenesis. These results provided novel clinical management evidence and new insights in pathologic mechanism of MMD combined with psychiatric disorders.
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Affiliation(s)
- Lei Cao
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China
| | - Eryong Liu
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China
| | - Xiaoli Yuan
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China
| | - Yang Dong
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China
| | - Bo Yang
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China.
| | - Hongwei Li
- Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China; Department of Hematology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450003, China.
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Muljadi R, Koesbandono, Pratama TA, Octavius GS. Moyamoya disease and moyamoya syndrome: A case series from multicentre private hospitals in Indonesia. NEUROCIRUGIA (ENGLISH EDITION) 2025; 36:151-160. [PMID: 39577562 DOI: 10.1016/j.neucie.2024.11.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/01/2024] [Revised: 10/24/2024] [Accepted: 10/26/2024] [Indexed: 11/24/2024]
Abstract
BACKGROUND Moyamoya disease (MMD) and moyamoya syndrome (MMS) are considered rare in Indonesia, without any proper epidemiological data backing this claim. Hence, this case series aims to assess all MMD and MMS cases from the perspective of a multicentre private hospital in Indonesia. METHODS This is a descriptive analysis using data from the picture archiving and communication system (PACS) from January 2019 to December 2023. The inclusion criteria included all patients who fulfilled the radiological criteria for MMD and/or MMS, while patients who only underwent brain non-contrast computed tomography (CT) scans were excluded. RESULTS There are 58,905 unique MRI scans from 2019 to 2023. The cohort comprises 8 females and 2 males, with a median age of 37 (7-65) years old. Three cases are probable MMD. with six MMD cases and one MMS case. Therefore, the prevalence rate for the four years is approximately 11.9 cases per 100,000 scans. Out of the confirmed Moya-Moya cases, the majority (4/7) are ischemic subtypes, followed by epileptic (electroencephalography shows slowing brain waves) and TIA in one case each. The most common presenting symptom is weakness in the extremity (N = 7), followed by headache (N = 5). Four patients underwent operative procedures, with three of them being superior temporal artery to middle cerebral artery (STA-MCA) bypass procedures and one of them being encephalo-duro-myo-arterio-pericraniosynangiosis (EDMAPS). CONCLUSION The paucity of confirmed cases either points towards the low prevalence of MMD and MMS in Indonesia or the underdiagnosis of these cases.
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Affiliation(s)
- Rusli Muljadi
- Thoracic and Cardiovascular Imaging Division, Department of Radiology, Faculty of Universitas Pelita Harapan, Tangerang, Indonesia; Department of Radiology of Siloam Hospital Lippo Village, Indonesia.
| | - Koesbandono
- Department of Radiology of Siloam Hospital Lippo Village, Indonesia; Interventional Radiology Division, Department of Radiology, Faculty of Universitas Pelita Harapan, Tangerang, Indonesia
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Muljadi R, Koesbandono, Pratama TA, Octavius GS. Moyamoya disease and moyamoya syndrome: A case series from multicentre private hospitals in Indonesia. Neurocirugia (Astur) 2025; 36:151-160. [DOI: 10.1016/j.neucir.2024.10.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/18/2025]
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Kim MS, Nam S, Lee SU, Park SJ, Woo SJ, Lee J, Joo K. Moyamoya Disease Increased the Risk of Retinal Vascular Occlusion: A Nationwide Cohort Study in Korea. Ophthalmol Retina 2025; 9:386-391. [PMID: 39442651 DOI: 10.1016/j.oret.2024.10.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2024] [Revised: 09/26/2024] [Accepted: 10/14/2024] [Indexed: 10/25/2024]
Abstract
PURPOSE To investigate the risk of retinal vascular occlusion in patients with Moyamoya disease (MMD). DESIGN Retrospective, longitudinal cohort study using the Korean National Health Insurance Service database. PARTICIPANTS Newly diagnosed MMD patients (n = 34 627), who were diagnosed between 2004 and 2022, and their propensity score matched controls (n = 136 945) were included. METHODS We identified retinal vascular occlusion events using diagnostic codes for central retinal artery occlusion, other retinal artery occlusion, and retinal vein occlusion. After a washout period from 2002 to 2003, information on the diagnosis of retinal vascular occlusion was extracted in both MMD and control group during the follow-up period. The association between MMD and the risk of subsequent retinal vascular occlusion was investigated using a time-dependent Cox proportional hazard model and Kaplan-Meier survival analysis with log-rank test adjusted for age, sex, and comorbidities. MAIN OUTCOME MEASURES Hazard ratios (HRs) and 95% confidence intervals (CIs) for retinal vascular occlusion development according to the MMD. RESULTS Moyamoya disease was associated with an increased risk of subsequent retinal vascular occlusion even after adjusting for confounding variables (HR, 1.22; 95% CI, 1.09-1.36). Among the subtypes of retinal vascular occlusion, central retinal artery occlusion showed a highest HR (2.23; 95% CI, 1.35-3.7). Incidence probability of retinal vascular occlusion was significantly higher among MMD patients than controls (P < 0.001, log-rank test). CONCLUSIONS In this nationwide population-based cohort study, patients with MMD in Korea had an elevated risk of retinal vascular occlusion, suggesting that the MMD is one of the risk factors for retinal vascular occlusion. FINANCIAL DISCLOSURE(S) Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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Affiliation(s)
- Min Seok Kim
- Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | | | - Si Un Lee
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Sang Jun Park
- Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Se Joon Woo
- Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | | | - Kwangsic Joo
- Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
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Tan BYQ, Tan SML, Ng MBJ, Chan BP. Ischaemic stroke in young adults: an overview of the classification, risk factors, aetiologies and outcomes. Singapore Med J 2025:00077293-990000000-00183. [PMID: 40084701 DOI: 10.4103/singaporemedj.smj-2024-010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2024] [Accepted: 08/18/2024] [Indexed: 03/16/2025]
Affiliation(s)
- Benjamin Yong-Qiang Tan
- Division of Neurology, Department of Medicine, National University Health System, Singapore
- Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
| | - Sarah Ming Li Tan
- Division of Neurology, Department of Medicine, National University Health System, Singapore
| | - Megan Bi Jia Ng
- Division of Neurology, Department of Medicine, National University Health System, Singapore
| | - Bernard Pl Chan
- Division of Neurology, Department of Medicine, National University Health System, Singapore
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Kim BR, Lee KH, Paik K, Kim M, Bae JM, Choi CW, Youn SW. Comorbid diseases in bullous pemphigoid: A population-based case-control study. J Dermatol 2025; 52:460-471. [PMID: 39670559 DOI: 10.1111/1346-8138.17577] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2024] [Revised: 11/20/2024] [Accepted: 11/25/2024] [Indexed: 12/14/2024]
Abstract
Bullous pemphigoid (BP) is the most prevalent autoimmune blistering disorder, triggered by autoantibodies targeting hemidesmosome components. It is associated with substantial morbidity and increased mortality. No studies comprehensively evaluate all comorbidities before and after diagnosing patients with BP. We aimed to investigate all BP-associated comorbid diseases and their patterns of associations. This nationwide population-based study included 5066 patients with BP and 10 132 controls between 2011 and 2021. We performed an automated mass screening of 546 diagnostic codes to identify BP-associated comorbidities 5 years before and after BP diagnosis, and analyzed associations patterns of comorbidities. Patients with BP had increased odds of having pressure ulcers, intracerebral hemorrhage, scabies, neuropsychiatric disorders, psoriasis, drug eruption, and acute renal failure before BP diagnosis. After BP diagnosis, they had increased odds pneumonia, sepsis, chronic renal disease, and cardiac arrest. Strong interrelationships were observed between five neuropsychiatric conditions before BP diagnosis and a strong bidirectional association between Alzheimer's dementia and pneumonia after BP diagnosis. This large case-control study of patients with BP thoroughly identified all relevant comorbidities before and after BP diagnosis, highlighting their clinical significance as predisposing and prognostic factors in patients with BP.
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Affiliation(s)
- Bo Ri Kim
- Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Republic of Korea
| | - Kun Hee Lee
- Department of Applied Statistics, Yonsei University, Seoul, Republic of Korea
| | - Kyungho Paik
- Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Republic of Korea
| | - Minjae Kim
- Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Republic of Korea
| | - Jung Min Bae
- Department of Dermatology, St Vincent's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Chong Won Choi
- Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Republic of Korea
| | - Sang Woong Youn
- Department of Dermatology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Republic of Korea
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Yu J, Du Q, Hu M, Tao T, Wan G, Zhang J, Chen J. Role of scalp arteries in revascularization after side to side anastomosis in moyamoya disease patients. Sci Rep 2024; 14:29961. [PMID: 39623047 PMCID: PMC11612458 DOI: 10.1038/s41598-024-81362-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2024] [Accepted: 11/26/2024] [Indexed: 12/06/2024] Open
Abstract
In our recent study, we introduced a novel side-to-side (S-S) bypass technique for adult moyamoya disease (MMD) patients. We aimed to validate the potential of this technique in enhancing postoperative revascularization. Patients undergoing S-S or end-to-side (E-S) bypass were enrolled, clinical data and angiography parameters were collected and compared. We included 44 E-S and 40 S-S MMD patients. There were no significant differences in basic characteristics and postoperative Matsushima grades between the two groups. However, in the S-S group, fewer patients had Matsushima grade D (0%) compared to the E-S group (11.4%, P = 0.028). The S-S group showed a greater occipital artery (OA) participation in revascularization (87.5% vs. 56.8%, P = 0.002) and higher postoperative caliber change ratio (CCR) of the superficial temporal artery (STA)-frontal branch (1.26 ± 0.43 vs. 1.04 ± 0.53, P = 0.038) compared to the E-S group. This difference was more pronounced in the subgroup with poor revascularization outcomes: in the S-S group, the CCR of the STA-frontal branch and the degree of participation in revascularization were 1.163 ± 0.168 and 58.8%, respectively, while in the E-S group, they were only 0.798 ± 0.494 and 6.7%. The S-S group also had a higher OA CCR (1.133 ± 0.257) and participation (82.4%) compared to the E-S group (0.941 ± 0.216 and 37.5%, respectively). In conclusion, the S-S bypass technique effectively utilizes scalp arteries, particularly the STA-frontal branch and OA, for direct revascularization via the preserved distal STA in adult MMD patients. Scalp arteries can serve as a supplementary source of donor arteries, especially beneficial for patients with suboptimal revascularization outcomes.
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Affiliation(s)
- Jin Yu
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China
- Wuhan Asia General hospital, Wuhan, 430071, China
| | - Qian Du
- Zhongnan Hospital of Wuhan University, Wuhan, 430071, China
| | - Miao Hu
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China
| | - Tianshu Tao
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China
| | - Guiping Wan
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China
| | - Jianjian Zhang
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China.
- Wuhan Asia General hospital, Wuhan, 430071, China.
| | - Jincao Chen
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Donghu Road 169, Wuhan, 430071, China.
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Byoun HS, Lee SU, Won YD, Choi TW, Lee SH, Kim YD, Ban SP, Bang JS, Kwon OK, Oh CW. Nationwide Cohort observational study on the safety and efficacy of COVID-19 vaccination in patients with Moyamoya disease. Sci Rep 2024; 14:24400. [PMID: 39420039 PMCID: PMC11487014 DOI: 10.1038/s41598-024-73940-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2024] [Accepted: 09/23/2024] [Indexed: 10/19/2024] Open
Abstract
The safety and efficacy of coronavirus disease 2019 (COVID-19) vaccination in patients diagnosed with Moyamoya disease (MMD) have not been established. Using National Health Insurance Service data, this study analyzed the occurrence of stroke-related events and mortality following COVID-19 vaccination among patients diagnosed with MMD from 2008 to 2020. Among 10,297 MMD patients, 296 (2.9%) experienced events and 175 (1.7%) died in 2021. Significant risk factors for events included ages 50-59 (Odds Ratio [OR] 3.29; P = 0.022) and 60 or above (OR 5.20; P = 0.001) (reference group: age in 20s), low BMI (OR 2.00; P = 0.011), previous stroke (OR 1.96; P < 0.001), and COVID-19 infection (OR 2.28; P = 0.034). Female (OR 0.64 [95% CI 0.50-0.82]; p = 0.011), revascularization surgery (RS) (OR 0.38 [95% CI 0.21-0.70]; p < 0.001), and vaccination (OR 0.17 [95% CI 0.13-0.22]; p < 0.001) were associated with reduced odds of stroke-related events. For mortality, significant risks were age over 60 (OR 7.09; P = 0.008), low BMI (OR 3.87; P = 0.001), and prior stroke (OR 1.74; p = 0.004), while being female, RS (OR 0.41; P = 0.022), and vaccination (OR 0.12; P < 0.001) were associated with a lower frequency of mortality. mRNA vaccines were associated with a significantly lower incidence of events, mortality, and COVID-19 infections compared to vector vaccines. COVID-19 vaccination is linked to reduced stroke-related events and mortality in MMD patients, with mRNA vaccines showing a significantly lower incidence compared to vector vaccines. COVID-19 infection raises the risk of events, underscoring the benefit of vaccination.
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Affiliation(s)
- Hyoung Soo Byoun
- Department of Neurosurgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju-si, Korea
| | - Si Un Lee
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea.
| | - Yu Deok Won
- Department of Neurosurgery, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri-si, Korea.
| | - Tae Won Choi
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - Sang Hyo Lee
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - Young-Deok Kim
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - Seung Pil Ban
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - Jae Seung Bang
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - O-Ki Kwon
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
| | - Chang Wan Oh
- Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, Korea
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Xu S, Chen T, Yu J, Wan L, Zhang J, Chen J, Wei W, Li X. Insights into the regulatory role of epigenetics in moyamoya disease: Current advances and future prospectives. MOLECULAR THERAPY. NUCLEIC ACIDS 2024; 35:102281. [PMID: 39188306 PMCID: PMC11345382 DOI: 10.1016/j.omtn.2024.102281] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Indexed: 08/28/2024]
Abstract
Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular disorder that predominantly affecting East Asian populations. The intricate interplay of distinct and overlapping mechanisms, including genetic associations such as the RNF213-p.R4810K variant, contributes to the steno-occlusive lesions and moyamoya vessels. However, genetic mutations alone do not fully elucidate the occurrence of MMD, suggesting a potential role for epigenetic factors. Accruing evidence has unveiled the regulatory role of epigenetic markers, including DNA methylation, histone modifications, and non-coding RNAs (ncRNAs), in regulating pivotal cellular and molecular processes implicated in the pathogenesis of MMD by modulating endothelial cells and smooth muscle cells. The profile of these epigenetic markers in cerebral vasculatures and circulation has been determined to identify potential diagnostic biomarkers and therapeutic targets. Furthermore, in vitro studies have demonstrated the multifaceted effects of modulating specific epigenetic markers on MMD pathogenesis. These findings hold great potential for the discovery of novel therapeutic targets, translational studies, and clinical applications. In this review, we comprehensively summarize the current understanding of epigenetic mechanisms, including DNA methylation, histone modifications, and ncRNAs, in the context of MMD. Furthermore, we discuss the potential challenges and opportunities that lie ahead in this rapidly evolving field.
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Affiliation(s)
- Shuangxiang Xu
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Tongyu Chen
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Jin Yu
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Lei Wan
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Jianjian Zhang
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Jincao Chen
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Wei Wei
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Xiang Li
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan University, Wuhan 430071, China
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
- Frontier Science Center for Immunology and Metabolism, Wuhan University, Wuhan 430071, China
- Medical Research Institute, Wuhan University, Wuhan 430071, China
- Sino-Italian Ascula Brain Science Joint Laboratory, Wuhan University, Wuhan 430071, China
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Cho IS, Lim J, Chang MS, Lee JH. Impact of the coronavirus disease 2019 pandemic on hospital admissions for idiopathic pulmonary fibrosis: a nationwide population-based study. BMC Pulm Med 2024; 24:430. [PMID: 39217306 PMCID: PMC11365253 DOI: 10.1186/s12890-024-03230-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2024] [Accepted: 08/19/2024] [Indexed: 09/04/2024] Open
Abstract
BACKGROUND Previous studies have consistently reported a decrease in hospital admissions for respiratory diseases during the coronavirus disease 2019 (COVID-19) pandemic. However, the impact of the pandemic on idiopathic pulmonary fibrosis (IPF) admissions remains unknown. METHODS This study used data from the Korean National Health Insurance Service database. IPF was defined based on the International Classification of Diseases 10th Revision (ICD-10) and rare intractable disease (RID) codes. The rate of IPF admissions was calculated by dividing the number of IPF admissions by the prevalence of IPF. The rate of IPF admissions during the COVID-19 pandemic (2020-2021) was compared with the mean rate of admissions during the prepandemic period (2017-2019) and presented as the rate ratio (RR). A sensitivity analysis was conducted on patients treated with systemic corticosteroids during IPF admission. RESULTS In patients with IPF defined based on the ICD-10 (analysis 1), the RRs significantly decreased from March in 2020 to December 2021, except for June and September in 2020. Similarly, in patients with IPF defined based on the ICD-10 and RID (analysis 2), the RRs significantly decreased from March 2020 to December 2021, except for June and September 2020. In the sensitivity analysis of analysis 1, the RR significantly decreased in 2020 (0.93; 95%CI: 0.88-0.99; P = 0.029), whereas the RR in 2021 was not significantly different. The RRs in the sensitivity analysis of analysis 2 significantly decreased to 0.85 (0.79-0.92; P < 0.001) in 2020 and 0.82 (0.76-0.88; P < 0.001) in 2021. In the subgroup analysis, the rates of IPF admissions significantly decreased in 2020 and 2021 across both sexes, patients aged ≥ 60 years, and all household income groups. CONCLUSIONS The rate of IPF admissions significantly decreased during the COVID-19 pandemic. This result indicates that preventive measures against COVID-19 may effectively mitigate IPF exacerbation. Therefore, it is assumed that there is a close relationship between respiratory viral infections and IPF exacerbations.
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Affiliation(s)
- In-So Cho
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, 20 Ilsan-ro, Wonju, 26426, Korea
| | - Jihye Lim
- Department of Medical Informatics and Biostatistics, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Min-Seok Chang
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, 20 Ilsan-ro, Wonju, 26426, Korea
| | - Ji-Ho Lee
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, 20 Ilsan-ro, Wonju, 26426, Korea.
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Lim YC, Lee E, Song J. Outcomes of Bypass Surgery in Adult Moyamoya Disease by Onset Type. JAMA Netw Open 2024; 7:e2415102. [PMID: 38842810 PMCID: PMC11157360 DOI: 10.1001/jamanetworkopen.2024.15102] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2023] [Accepted: 04/04/2024] [Indexed: 06/07/2024] Open
Abstract
Importance Moyamoya disease (MMD) is a rare chronic cerebrovascular disease, and the outcomes of bypass management in adult patients remain controversial. Objective To categorize adult MMD based on asymptomatic, ischemic, and hemorrhagic onset and compare the outcomes (death, hemorrhagic stroke [HS], and ischemic stroke [IS]) of bypass surgery (direct or indirect) with those of conservative management. Design, Setting, and Participants This retrospective, nationwide, population-based longitudinal cohort study used Korean National Health Insurance Research data to identify adults (aged ≥15 years) with MMD who were diagnosed between January 1, 2008, and December 31, 2020, and followed up until December 31, 2021 (median follow-up, 5.74 [IQR, 2.95-9.42] years). A total of 19 700 participants (3194 with hemorrhagic, 517 with ischemic, and 15 989 with asymptomatic MMD) were included. Data were analyzed from January 2 to April 1, 2023. Exposures Bypass surgery and conservative management. Main Outcomes and Measures Death constituted the primary outcome; secondary outcomes consisted of HS or IS. Kaplan-Meier survival curve and Cox proportional hazards regression analysis were applied. The propensity score-matching and stratified analyses were performed to control covariate effects. Results A total of 19 700 patients (mean [SD] age, 45.43 [14.98] years; 12 766 [64.8%] female) were included. Compared with conservative management, bypass was associated with a reduced risk of death (adjusted hazard ratio [AHR], 0.50 [95% CI, 0.41-0.61]; P < .001) and HS (AHR, 0.36 [0.30-0.40]; P < .001) in hemorrhagic MMD; reduced risk of IS (AHR, 0.55 [95% CI, 0.37-0.81]; P = .002) in ischemic MMD; and reduced risk of death (AHR, 0.74 [95% CI, 0.66-0.84]; P < .001) in asymptomatic MMD. However, bypass was associated with an increased risk of HS (AHR, 1.76 [95% CI, 1.56-2.00]; P < .001) in asymptomatic MMD. Both direct and indirect bypass demonstrated similar effects in hemorrhagic and asymptomatic MMD, except only direct bypass was associated with a reduced risk of IS (AHR, 0.52 [95% CI, 0.33- 0.83]; P = .01) in ischemic MMD. After stratification, bypass was associated with a reduced risk of death in patients younger than 55 years with ischemic (AHR, 0.34 [95% CI, 0.13- 0.88]; P = .03) and asymptomatic (AHR, 0.69 [95% CI, 0.60-0.79]; P < .001) MMD, but an increased risk of HS in patients 55 years or older with ischemic MMD (AHR, 2.13 [95% CI, 1.1-4.16]; P = .03). Conclusions and Relevance The findings of this cohort study of bypass outcomes for patients with MMD emphasize the importance of tailoring management strategies in adult patients based on onset types.
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Affiliation(s)
- Yong Cheol Lim
- Department of Neurosurgery, Ajou University School of Medicine, Ajou University Hospital, Suwon, Republic of Korea
| | - Eunyoung Lee
- Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston
| | - Jihye Song
- Department of Neurosurgery, Ajou University School of Medicine, Ajou University Hospital, Suwon, Republic of Korea
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Strunk D, Bauer P, Keyvani K, Diehl RR, Veltkamp R, Berlit P, Meuth SG, Timmermann L, Schwitalla JC, Kraemer M. Moyamoya disease in Southeast Asians: genetic and autopsy data, new cases, systematic review, and meta-analysis of all patients from the literature. J Neurol 2024; 271:3328-3339. [PMID: 38478032 PMCID: PMC11136762 DOI: 10.1007/s00415-024-12228-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2023] [Revised: 01/27/2024] [Accepted: 01/27/2024] [Indexed: 05/30/2024]
Abstract
BACKGROUND Moyamoya disease (MMD) is a rare disorder causing ischemic and hemorrhagic juvenile stroke. It is associated with the founder susceptibility variant p.R4810K in the RNF213 gene in East Asia. Our aim was to enhance understanding of MMD in so far poorly characterized Southeast Asians and exploring differences with Caucasian Europeans. METHODS By retrospective analysis of medical records and systematic database search on PubMed for all published cases, we identified Southeast Asian patients with MMD. We extracted and pooled proportions using fixed-effects models. Our own cohort was tested for the East Asian RNF213 founder variant p.R4810K. One of our Southeast Asian patients underwent post-mortem histopathological examination. RESULTS The study cohort comprised 32 Southeast Asians. Mean age at onset in the entire cohort was 32.5 ± 20.3 years (n = 24), 43.4 ± 8.7 years in patients admitted to our center (n = 11), and 23.4 ± 22.4 years in patients from the international literature (n = 13). Female-to-male ratio was 1.6:1. MMD predominantly affected bilateral anterior intracranial vessels. Cerebral ischemia outnumbered transient ischemic attacks (TIAs) and intracranial hemorrhage. TIAs, arterial hypertension and obesity were significantly less frequent in Southeast Asian patients compared to Caucasian Europeans. p.R4810K was absent in all examined Southeast Asians despite of typical histopathological signs of MMD in one autopsy case. CONCLUSION Clinical and histopathological manifestations of MMD in Southeast Asians are similar to those in Caucasian Europeans. The genotype of MMD in Southeast Asians differs from that of most East Asian patients.
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Affiliation(s)
- Daniel Strunk
- Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Straße 21, 45131, Essen, Germany.
- Department of Neurology, University Hospital Gießen and Marburg, Marburg, Germany.
| | | | - Kathy Keyvani
- Institute of Neuropathology, University of Duisburg-Essen, Essen, Germany
| | - Rolf R Diehl
- Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Straße 21, 45131, Essen, Germany
| | - Roland Veltkamp
- Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Straße 21, 45131, Essen, Germany
- Department of Brain Sciences, Imperial College London, London, UK
| | | | - Sven G Meuth
- Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany
| | - Lars Timmermann
- Department of Neurology, University Hospital Gießen and Marburg, Marburg, Germany
| | | | - Markus Kraemer
- Department of Neurology, Alfried Krupp Hospital, Alfried-Krupp-Straße 21, 45131, Essen, Germany.
- Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
- Moyamoya Friends Association, Essen, Germany.
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Liu H, Fukasawa T, Anno T, Takeuchi M, Shimazaki S, Yang T, Kawakami K. Incidence, prevalence, and treatment of Moyamoya disease in Japan: A population-based descriptive study. J Stroke Cerebrovasc Dis 2024; 33:107770. [PMID: 38768667 DOI: 10.1016/j.jstrokecerebrovasdis.2024.107770] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/11/2024] [Revised: 05/10/2024] [Accepted: 05/13/2024] [Indexed: 05/22/2024] Open
Abstract
BACKGROUND Moyamoya disease (MMD) is characterized by progressive stenosis or occlusion of the terminal portions of the bilateral internal carotid arteries. A Japanese survey in 2003 reported an incidence and prevalence of MMD of 0.54 and 6.03 per 100,000 people, respectively, showing an upward trend over previous surveys. An update to these estimates is therefore warranted. Additionally, evidence is lacking on trends in revascularization and antiplatelet therapy in MMD patients. METHODS We conducted a population-based descriptive study using a Japanese claims database. From fiscal year (FY) 2015 to 2019, we standardized the incidence and prevalence estimates of MMD to the 2015 Japanese census population by age and sex. We also estimated the 1-year cumulative incidence of revascularization among incident MMD patients and the proportion of prevalent MMD patients receiving antiplatelet therapy in each FY. RESULTS The age-standardized male-to-female ratio of both incident and prevalent MMD patients was approximately 1:2. Standardized incidence and prevalence of MMD per 100,000 population increased slightly from 1.8 to 2.4 and 14.7 to 17.6, respectively. The 1-year cumulative incidence of revascularization among incident MMD patients varied between 21.9 % and 28.9 %. Among prevalent MMD patients, 36.6 % to 39.0 % received antiplatelet therapy. CONCLUSIONS The incidence and prevalence of MMD in Japan from FY 2015 to 2019 were higher than those estimated in 2003. The trends in revascularization and antiplatelet therapy identified in this study will be useful in further improving the quality of MMD clinical practice.
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Affiliation(s)
- Hao Liu
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan
| | - Toshiki Fukasawa
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan; Department of Digital Health and Epidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan
| | - Takayuki Anno
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan
| | - Masato Takeuchi
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan
| | - Sho Shimazaki
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan
| | - Tao Yang
- Department of Neurosurgery, Osaka Red Cross Hospital, Osaka, Japan
| | - Koji Kawakami
- Department of Pharmacoepidemiology, Graduate School of Medicine and Public Health, Kyoto University, Kyoto, Japan.
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Lee JH, Hong SH, Yu I, Chang MS, Park S, Lee SJ, Kim SH. Incidence, Prevalence, and Mortality of Eosinophilic Granulomatosis With Polyangiitis in Korea: A Nationwide Population-Based Study. ALLERGY, ASTHMA & IMMUNOLOGY RESEARCH 2024; 16:168-178. [PMID: 38528384 DOI: 10.4168/aair.2024.16.2.168] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/10/2023] [Revised: 09/07/2023] [Accepted: 10/18/2023] [Indexed: 03/27/2024]
Abstract
PURPOSE Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis with multiorgan involvement. The incidence and prevalence of EGPA vary geographically and ethnically. This study investigated the incidence, prevalence, and mortality of EGPA in a nationwide population-based cohort in Korea. METHODS This retrospective cohort study used data from the National Health Insurance database that covers almost all Korean residents. EGPA was identified using relevant diagnostic codes from 2007 to 2018. Newly diagnosed EGPA cases since 2007 and patients who visited outpatient clinics for EGPA at least three times were included. Age- and sex-adjusted standardized incidence and prevalence rates were analyzed. RESULTS A total of 843 patients with EGPA were identified. The mean annual standardized incidence between 2007 and 2018 was 1.2 (per 1,000,000 individuals). The incidence of EGPA has increased from 1.1 (per 1,000,000 individuals) in 2007 to 1.6 (per 1,000,000 individuals) in 2017. The standardized prevalence of EGPA has increased from 1.1(per 1,000,000 individuals) in 2007 to 11.2 (per 1,000,000 individuals) in 2018. The incidence and prevalence of EGPA were higher in women than in men. The standardized mortality rate was 1.61 (95% confidence interval [CI], 1.34-1.93) in total population, 1.59 (95% CI, 1.23-2.02) in males, and 1.63 (95% CI, 1.22-2.13) in females. CONCLUSIONS The incidence of EGPA has increased over the past decade. Incidence and prevalence rates were higher in females than in males. The overall mortality rate associated with EGPA was higher than that in the general population.
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Affiliation(s)
- Ji-Ho Lee
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Se Hwa Hong
- Department of Biostatistics, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Iseul Yu
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Min-Seok Chang
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Sunmin Park
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Seok Jeong Lee
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Sang-Ha Kim
- Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.
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Wang Q, Yao Q, Yuan S, Shen Y, Feng Y, Liu L, Zhu Y, Zhao Y, Cui J, Qin J, Tian J, Zhao R, Liu L, Zhou Y, Liu X. Recurrent Cerebral Infarction Due to Moyamoya Disease Complicated With Systemic Lupus Erythematosus: A Case Report and Literature Review. Neurologist 2024; 29:4-13. [PMID: 37582681 DOI: 10.1097/nrl.0000000000000517] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/17/2023]
Abstract
INTRODUCTION We report a rare case of moyamoya disease caused by an RNF213 mutation, complicated with systemic lupus erythematosus. CASE REPORT A 32-year-old woman experienced 4 cerebral ischemia stroke events within 6 months. The main symptom was left limb weakness with blurred vision in the right eye. Results of digital subtraction angiography conducted at another hospital were consistent with moyamoya disease. On genetic testing, we found that the patient carried 2 mutations in the moyamoya disease-related gene RNF213 (p.R4810K, p.T1727M). On the basis of the laboratory immunologic indicators, such as positive antibodies and abnormal immunoglobulin levels and imaging examinations, the patient was finally diagnosed as moyamoya disease complicated with systemic lupus erythematosus. She was treated with aspirin, butylphthalide, urinary kallidinogenase, and sodium methylprednisolone. CONCLUSIONS This was a 32-year-old young patient diagnosed with moyamoya disease carrying RNF213 gene mutation and accompanied by lupus with cerebral ischemic event as the first occurrence. The patient's condition was complex; therefore, comprehensive analysis and in-depth consideration were needed to avoid a missed diagnosis and misdiagnosis. When the primary disease cannot be identified, genetic testing can help to clarify the diagnosis of moyamoya disease.
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Affiliation(s)
- Qisong Wang
- Department of Neurology, The Second Hospital of Hebei Medical University, Shijiazhuang, China
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Park H, Han M, Jang D, Kim D, Huh P, Park H, Park IS, Han Y, Sung JH, Lee K, Lee H, Kim YW. Association of Bypass Surgery and Mortality in Moyamoya Disease. J Am Heart Assoc 2023; 12:e030834. [PMID: 37947101 PMCID: PMC10727291 DOI: 10.1161/jaha.123.030834] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/13/2023] [Accepted: 10/11/2023] [Indexed: 11/12/2023]
Abstract
BACKGROUND Patients with moyamoya disease (MMD) have a high risk of stroke or death. We investigated whether extracranial to intracranial bypass surgery can reduce mortality by preventing strokes in patients with MMD. METHODS AND RESULTS This nationwide retrospective cohort study encompassed patients with MMD registered under the Rare Intractable Diseases program via the Relieved Co-Payment Policy between 2006 and 2019, using the Korean National Health Insurance Service database. Following a 4-year washout period, landmark analyses were employed to assess mortality and stroke occurrence between the bypass surgery group and the nonsurgical control group at specific time points postindex date (1 month and 3, 6, 12, and 36 months). The study included 18 480 patients with MMD (mean age, 40.7 years; male to female ratio, 1:1.86) with a median follow-up of 5.6 years (interquartile range, 2.5-9.3; mean, 6.1 years [SD, 4.0 years]). During 111 775 person-years of follow-up, 265 patients in the bypass surgery group and 1144 patients in the nonsurgical control group died (incidence mortality rate of 618.1 events versus 1660.3 events, respectively, per 105 person-years). The overall adjusted hazard ratio (HR) revealed significantly lower all-cause mortality in the bypass surgery group from the 36-month landmark time point, for any stroke mortality from 3- and 6-month landmark time points, and for hemorrhagic stroke mortality from the 6-month landmark time point. Furthermore, the overall adjusted HRs for hemorrhagic stroke occurrence were beneficially maintained from all 5 landmark time points in the bypass surgery group. CONCLUSIONS Bypass surgery in patients with MMD was associated with a lower risk of all-cause and hemorrhagic stroke mortality and hemorrhagic stroke occurrence compared with nonsurgical control.
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Affiliation(s)
- Hyunjun Park
- Department of Neurosurgery, Incheon St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaIncheonRepublic of Korea
| | - Minkyung Han
- Biostatistics Collaboration Unit, Department of Biomedical Systems InformaticsYonsei University College of MedicineSeoulRepublic of Korea
| | - Dong‐Kyu Jang
- Department of Neurosurgery, Incheon St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaIncheonRepublic of Korea
| | - Dal‐Soo Kim
- Department of NeurosurgeryMyong‐Ji St. Mary’s HospitalSeoulRepublic of Korea
| | - Pil‐Woo Huh
- Department of Neurosurgery, Uijeongbu St. Mary’s Hospital. College of MedicineThe Catholic University of KoreaGyeonggi‐doRepublic of Korea
| | - Hae‐Kwan Park
- Department of Neurosurgery, Eunpyeong St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaSeoulRepublic of Korea
| | - Ik Seong Park
- Department of Neurosurgery, Bucheon St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaGyeonggi‐doRepublic of Korea
| | - Young‐Min Han
- Department of NeurosurgeryNaeun HospitalIncheonRepublic of Korea
| | - Jae Hoon Sung
- Department of Neurosurgery, St. Vincent’s Hospital, College of MedicineThe Catholic University of KoreaGyeonggi‐doRepublic of Korea
| | - Kwan‐Sung Lee
- Department of Neurosurgery, Seoul St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaSeoulRepublic of Korea
| | - Hyung‐Jin Lee
- Department of Neurosurgery, Daejeon St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaDaejeonRepublic of Korea
| | - Young Woo Kim
- Department of Neurosurgery, Uijeongbu St. Mary’s Hospital. College of MedicineThe Catholic University of KoreaGyeonggi‐doRepublic of Korea
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Koo JH, Lee M, Kim EH, Oh HJ, Lim JS, Hyung WJ, Yoon HI, Jung I, Chung YE. Harmful effect of repetitive intravenous iodinated contrast media administration on the long-term renal function of patients with early gastric cancer. Sci Rep 2023; 13:19448. [PMID: 37945805 PMCID: PMC10636198 DOI: 10.1038/s41598-023-46773-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2023] [Accepted: 11/04/2023] [Indexed: 11/12/2023] Open
Abstract
This retrospective study investigated whether repetitive exposure to intravenous iodinated contrast media (ICM) affects long-term renal function in patients who undergo curative surgery for early gastric cancer (EGC) collected from the Korean Health Insurance and Review Assessment (HIRA) database. Patients diagnosed with gastric cancer between January 2010 and December 2013 underwent regular computed tomography (CT) scans to monitor for extragastric recurrence. Patients who already had chronic kidney disease (CKD) before cancer diagnosis or had undergone chemotherapy or repeated surgery were excluded. A nested case-control study design was chosen to analyze the effect of repetitive ICM exposure to long-term renal function by comparing patients who developed CKD 2 years after cancer diagnosis and patients who did not. Among 59,971 patients collected according to inclusion and exclusion criteria, 1021 were diagnosed with CKD 2 years after cancer diagnosis. Using 1:5 matching after adjusting for age, sex and date of cancer diagnosis, 5097 control patients were matched to 1021 CKD patients. Conditional logistic regression showed that the number of CTs taken using ICM slightly increased the odds of CKD (odds ratio, 1.080; 95% confidence interval (CI): 1.059, 1.100; P < 0.0001). Thus, the administration of ICM might contribute to chronic renal function impairment.
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Affiliation(s)
- Ja Ho Koo
- Department of Radiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea
| | - Myeongjee Lee
- Biostatistics Collaboration Unit, Department of Biomedical Systems Informatics, Yonsei University College of Medicine, Seoul, South Korea
| | - Eun Hwa Kim
- Biostatistics Collaboration Unit, Department of Biomedical Systems Informatics, Yonsei University College of Medicine, Seoul, South Korea
| | - Hyung Jung Oh
- Department of Nephrology, Sheikh Khalifa Specialty Hospital, Ras Al-Khaimah, United Arab Emirates
| | - Joon Seok Lim
- Department of Radiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea
| | - Woo Jin Hyung
- Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Hong In Yoon
- Department of Radiation Oncology, Yonsei University College of Medicine, Seoul, South Korea
| | - Inkyung Jung
- Division of Biostatistics, Department of Biomedical Systems Informatics, Yonsei University College of Medicine, Seoul, South Korea.
| | - Yong Eun Chung
- Department of Radiology, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea.
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Kim JW, Hayashi T, Kim SK, Shirane R. Technical evolution of pediatric neurosurgery: moyamoya disease. Childs Nerv Syst 2023; 39:2819-2827. [PMID: 37395784 DOI: 10.1007/s00381-023-06017-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2023] [Accepted: 05/31/2023] [Indexed: 07/04/2023]
Abstract
Moyamoya disease (MMD) is a rare steno-occlusive disease of the bilateral internal carotid arteries that predominantly occurs in East Asia. Since the first description of the MMD by Suzuki and Takaku in 1969, significant advances have been made in both basic and clinical understanding of the disease. The incidence and prevalence of pediatric MMD have increased, potentially due to improved detection rates. The advancement of neuroimaging techniques has enabled MRI-based diagnostics and detailed visualization of the vessel wall. Various methods of surgical treatments are successful in pediatric MMD patients, and recent studies emphasize the importance of reducing postoperative complications since the goal of MMD surgery is to prevent future cerebral infarction and hemorrhage. Long-term outcomes following appropriate surgical treatment in pediatric MMD patients have shown promising results, including favorable outcomes in very young patients. Further studies with a large patient cohort are needed to establish individualized risk group stratification for determining the optimal timing of surgical treatment and to conduct multidisciplinary outcome assessments.
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Affiliation(s)
- Joo Whan Kim
- Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 03080, 101 Daehak-Ro, Jongno-Gu, Seoul, Republic of Korea
| | - Toshiaki Hayashi
- Department of Pediatric Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Japan
- Department of Neurosurgery, Miyagi Children's Hospital, 4 Chome-3-17 Ochiai, Aoba Ward, Sendai, Miyagi, 989-3126, Japan
| | - Seung-Ki Kim
- Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 03080, 101 Daehak-Ro, Jongno-Gu, Seoul, Republic of Korea.
| | - Reizo Shirane
- Department of Neurosurgery, Miyagi Children's Hospital, 4 Chome-3-17 Ochiai, Aoba Ward, Sendai, Miyagi, 989-3126, Japan.
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Mariadas R, Liu S, Sachdeva M, Unnikrishnan S, Foong HY, Stoodley M. Revascularization Surgery for Moyamoya Vasculopathy: An Australian Experience. World Neurosurg 2023; 178:e65-e71. [PMID: 37419316 DOI: 10.1016/j.wneu.2023.06.129] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2023] [Revised: 06/25/2023] [Accepted: 06/26/2023] [Indexed: 07/09/2023]
Abstract
BACKGROUND Moyamoya vasculopathy is a rare steno-occlusive cerebrovascular disorder presenting with ischemia or hemorrhage. There are racial and geographic differences in presentation and outcome. There is little information regarding moyamoya in Australia. METHODS Moyamoya patients undergoing surgery from 2001 to 2022 were studied retrospectively. The outcomes of revascularization surgery in adult and pediatric patients, with ischemic and hemorrhagic disease were analyzed, including functional outcomes, postoperative complications, bypass patency, and long-term rates of ischemic and hemorrhagic events. RESULTS A total of 68 patients with 122 revascularized hemispheres and 8 posterior circulation revascularizations were included in this study. Eighteen patients were of Asian descent and 46 were of Caucasian origin. Presentation was with ischemia in 124 hemispheres and hemorrhage in six hemispheres. There were 92 direct, 34 indirect, and 4 combined revascularization surgeries performed. Early postoperative complications occurred in 3.1% (n = 4) of operations and delayed complications (infection, subdural hematoma) occurred after 4.6% (n = 6) of operations. Mean follow-up was 6.5 years (3-252 months). There was 100% patency of direct grafts at last follow-up. There were no hemorrhagic events following surgery and 1 new ischemic event 2 years after surgery. There was significant improvement in physical health functional outcomes at most recent follow-up (P < 0.05); mental health outcomes were not different between preoperative and postoperative assessments. CONCLUSIONS The majority of Australian moyamoya patients are Caucasian and the most common clinical presentation is ischemia. Revascularization surgery had excellent outcomes with very low rates of ischemia and hemorrhage, comparing favorably to the natural history of moyamoya vasculopathy.
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Affiliation(s)
- Rachael Mariadas
- Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia
| | - Shinuo Liu
- Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia
| | - Mugdha Sachdeva
- Department of Neurosurgery, Diakonie Klinikum Jung Stilling Hospital, Siegen, Germany
| | - Sunil Unnikrishnan
- Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia
| | - Hui Yuan Foong
- Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia
| | - Marcus Stoodley
- Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia.
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Jeon JP, Hong EP, Ha EJ, Kim BJ, Youn DH, Lee S, Lee HC, Kim KM, Lee SH, Cho WS, Kang HS, Kim JE. Genome-wide association study identifies novel susceptibilities to adult moyamoya disease. J Hum Genet 2023; 68:713-720. [PMID: 37365321 DOI: 10.1038/s10038-023-01167-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2023] [Revised: 05/22/2023] [Accepted: 05/23/2023] [Indexed: 06/28/2023]
Abstract
Genome-wide association study has limited to discover single-nucleotide polymorphisms (SNPs) in several ethnicities. Here, we investigated an initial GWAS to identify genetic modifiers predicting with adult moyamoya disease (MMD) in Koreans. GWAS was performed in 216 patients with MMD and 296 controls using the large-scale Asian-specific Axiom Precision Medicine Research Array. A subsequent fine-mapping analysis was conducted to assess the causal variants associated with adult MMD. A total of 489,966 out of 802,688 SNPs were subjected to quality control analysis. Twenty-one SNPs reached a genome-wide significance threshold (p = 5 × 10-8) after pruning linkage disequilibrium (r2 < 0.8) and mis-clustered SNPs. Among these variants, the 17q25.3 region including TBC1D16, CCDC40, GAA, RNF213, and ENDOV genes was broadly associated with MMD (p = 3.1 × 10-20 to 4.2 × 10-8). Mutations in RNF213 including rs8082521 (Q1133K), rs10782008 (V1195M), rs9913636 (E1272Q), rs8074015 (D1331G), and rs9674961 (S2334N) showed a genome-wide significance (1.9 × 10-8 < p < 4.3 × 10-12) and were also replicated in the East-Asian populations. In subsequent analysis, RNF213 mutations were validated in a fine-mapping outcome (log10BF > 7). Most of the loci associated with MMD including 17q25.3 regions were detected with a statistical power greater than 80%. This study identifies several novel and known variations predicting adult MMD in Koreans. These findings may good biomarkers to evaluate MMD susceptibility and its clinical outcomes.
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Affiliation(s)
- Jin Pyeong Jeon
- Department of Neurosurgery, Hallym University College of Medicine, Chuncheon, Republic of Korea
- Institute of New Frontier Research, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Eun Pyo Hong
- Institute of New Frontier Research, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Eun Jin Ha
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Bong Jun Kim
- Institute of New Frontier Research, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Dong Hyuk Youn
- Institute of New Frontier Research, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Sungyoung Lee
- Department of Genomic Medicine, Center for Precision Medicine, Seoul National University Hospital, Seoul, Republic of Korea
| | - Hee Chang Lee
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Kang Min Kim
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Sung Ho Lee
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Won-Sang Cho
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Hyun-Seung Kang
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Jeong Eun Kim
- Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea.
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21
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Vassilopoulou S, Tountopoulou A, Korompoki E, Papageorgiou G, Kasselimis D, Velonakis G, Chatziioannou A, Potagas C, Spengos K. Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients. J Clin Med 2023; 12:5951. [PMID: 37762892 PMCID: PMC10531977 DOI: 10.3390/jcm12185951] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2023] [Revised: 08/31/2023] [Accepted: 09/07/2023] [Indexed: 09/29/2023] Open
Abstract
BACKGROUND AND PURPOSE The aim of our study is to present, for the first time, the clinical, radiological, and neurocognitive characteristics of Greek adult patients with Moyamoya disease (MMD). METHODS We analyzed prospectively collected data of 12 patients referred to our department from 2004 to 2019. All patients underwent a thorough diagnostic work up, including extensive clinical, neuroradiological, and neurocognitive assessment. RESULTS Our study population consisted of 7 females and the median age at the time of the diagnosis was 43.5 years. No patient had a positive family history of the disease and roughly 50% were hypertensives. Ten patients presented with transient or permanent cerebrovascular ischemia and two patients suffered from hemorrhagic complications. The median NIHSS was 7.5 (0-23) and clinical status remained stable during follow-up with conservative treatment in most of the patients. The majority (83.3%) had bilateral disease confirmed by DSA. All lesions exclusively affected the anterior circulation, with 50% of patients presenting with stenoocclusive changes. No aneurysm or AVM were revealed. The most common neurocognitive deficits were in the executive and language domains. CONCLUSIONS Our MMD patients had a later onset of the disease and an absence of familial occurrence. The most common manifestation was ischemia, transient or permanent, and all lesions affected the anterior circulation, whereas no vascular malformations (AVM, aneurysms) were demonstrated in brain imaging. These findings in Greek patients imply a probable different, Mediterranean phenotype.
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Affiliation(s)
- Sofia Vassilopoulou
- Stroke Unit, 1st Department of Neurology, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece
| | - Argyro Tountopoulou
- Stroke Unit, 1st Department of Neurology, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece
| | - Eleni Korompoki
- Stroke Unit, 1st Department of Neurology, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece
- Department of Clinical Therapeutics, National and Kapodistrian University of Athens, 11528 Athens, Greece
| | - Georgios Papageorgiou
- Neuropsychology and Language Disorders Unit, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece (D.K.)
| | - Dimitrios Kasselimis
- Neuropsychology and Language Disorders Unit, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece (D.K.)
- Department of Psychology, Panteion University of Social and Political Sciences, 17671 Athens, Greece
| | - Georgios Velonakis
- 2nd Department of Radiology, National and Kapodistrian University of Athens, 12462 Athens, Greece
| | - Achilles Chatziioannou
- 1st Department of Radiology, National and Kapodistrian University of Athens, 11528 Athens, Greece
| | - Constantin Potagas
- Neuropsychology and Language Disorders Unit, Eginition Hospital, National and Kapodistrian University of Athens, 11528 Athens, Greece (D.K.)
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22
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Chen T, Wei W, Yu J, Xu S, Zhang J, Li X, Chen J. The Progression of Pathophysiology of Moyamoya Disease. Neurosurgery 2023; 93:502-509. [PMID: 36912514 DOI: 10.1227/neu.0000000000002455] [Citation(s) in RCA: 14] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2022] [Accepted: 01/17/2023] [Indexed: 03/14/2023] Open
Abstract
Moyamoya disease (MMD) is a chronic steno-occlusive cerebrovascular disease that often leads to hemorrhagic and ischemic strokes; however, its etiology remains elusive. Surgical revascularization by either direct or indirect bypass techniques to restore cerebral hypoperfusion is the treatment of choice to date. This review aims to provide an overview of the current advances in the pathophysiology of MMD, including the genetic, angiogenic, and inflammatory factors related to disease progression. These factors may cause MMD-related vascular stenosis and aberrant angiogenesis in complex manners. With a better understanding of the pathophysiology of MMD, nonsurgical approaches that target the pathogenesis of MMD may be able to halt or slow the progression of this disease.
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Affiliation(s)
- Tongyu Chen
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Wei Wei
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Jin Yu
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Shuangxiang Xu
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Jianjian Zhang
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Xiang Li
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
- Brain Research Center, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
- Sino-Italian Ascula Brain Science Joint Laboratory, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
| | - Jincao Chen
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan , Hubei Province , China
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23
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Zhang XH, He JH, Zhang XS, Zhang J, Wang CJ, Dong YP, Tao W. Comparison of revascularization and conservative treatment for hemorrhagic moyamoya disease in East Asian Countries: a single-center case series and a systematic review with meta-analysis. Front Neurol 2023; 14:1169440. [PMID: 37332987 PMCID: PMC10272728 DOI: 10.3389/fneur.2023.1169440] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2023] [Accepted: 04/28/2023] [Indexed: 06/20/2023] Open
Abstract
Objective The optimal treatment approach for hemorrhagic moyamoya disease (HMMD) remains a topic of debate, particularly regarding the comparative efficacy of revascularization versus conservative treatment. Our study, which included a single-center case series and a systematic review with meta-analysis, aimed to determine whether surgical revascularization is associated with a significant reduction in postoperative rebleeding, ischemic events, and mortality compared to conservative treatment among East Asian HMMD patients. Methods We conducted a systematic literature review by searching PubMed, Google Scholar, Wanfang Med Online (WMO), and the China National Knowledge Infrastructure (CNKI). The outcomes of surgical revascularization and conservative treatment, including rebleeding, ischemic events and mortality, were compared. The authors' institutional series of 24 patients were also included and reviewed in the analysis. Results A total of 19 East Asian studies involving 1,571 patients as well as our institution's retrospective study of 24 patients were included in the study. In the adult patients-only studies, those who underwent revascularization had significantly lower rates of rebleeding, ischemic events, and mortality compared to those who received conservative treatment (13.1% (46/352) vs. 32.4% (82/253), P < 0.00001; 4.0% (5/124) vs. 14.9% (18/121), P = 0.007; and 3.3% (5/153) vs. 12.6% (12/95), P = 0.01, respectively). In the adult/pediatric patients' studies, similar statistical results of rebleeding, ischemic events, and mortality have been obtained (70/588 (11.9%) vs. 103/402 (25.6%), P = 0.003 or <0.0001 in a random or fixed-effects model, respectively; 14/296 (4.7%) vs. 26/183 (14.2%), P = 0.001; and 4.6% (15/328) vs. 18.7% (23/123), P = 0.0001, respectively). Conclusion The current single-center case series and systematic review with meta-analysis of studies demonstrated that surgical revascularization, including direct, indirect, and a combination of both, significantly reduces rebleeding, ischemic events, and mortality in HMMD patients in the East Asia region. More well-designed studies are warranted to further confirm these findings.
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Affiliation(s)
- Xiang-Hua Zhang
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
| | - Jun-Hua He
- Department of Neurosurgery, Zhejiang Provincial Tongde Hospital, Hangzhou, China
| | - Xiang-Sheng Zhang
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
| | - Jing Zhang
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
| | - Cheng-jun Wang
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
| | - Yi-Peng Dong
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
| | - Wu Tao
- Department of Neurosurgery, Beijing Friendship Hospital Affiliated With Capital Medical University, Beijing, China
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24
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Luo Y, Cao Z, Ye H, Wu S, Sun X. Antiplatelet therapy may improve the prognosis of patients with moyamoya disease: a 12-year retrospective study. J Neurol 2023:10.1007/s00415-023-11702-5. [PMID: 37106259 DOI: 10.1007/s00415-023-11702-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2022] [Revised: 03/22/2023] [Accepted: 04/03/2023] [Indexed: 04/29/2023]
Abstract
OBJECTIVES The clinical value of antiplatelet therapy (APT) for moyamoya disease (MMD) remains controversial. Our study attempts to clarify the value of APT in this disease. METHODS We collected basic information, treatment strategies, and prognostic information on patients with MMD from 2010 to 2022 at our center. The data were divided into two groups, depending on whether APT was used or not, and compared by Pearson Chi-Square, Fisher's exact test, or Wilcoxon rank-sum test. We used propensity scores or inverse probability of treatment weighting to balance the covariates. Following this, we performed a meta-analysis of APT use in MMD. RESULTS 177 patients were enrolled, with a median follow-up of 41.1 months. APT did not affect the prognosis of patients with perioperative MMD, ischemic MMD, or asymptomatic MMD (P > 0.05), without increasing cerebral hemorrhagic risk. In contrast, APT was found to reduce mortality among patients with hemorrhagic MMD (P = 0.019), without affecting functional status, increasing stroke risk, or causing intracerebral hemorrhage (P > 0.05). But the small group cannot show the effect of APT. Our meta-analysis included nine articles involving 28,925 patients with MMD. It showed that APT could reduce stroke risk (odds ratio, OR = 0.57, 95% CI 0.49 to 0.65) and the Modified Rankin Scale (mRS) (weighted mean difference, WMD = - 0.07, 95% CI 0.14-0.00) during follow-up. The cohort study has limited weight (1.97% and 19.29%) in the meta-analysis. CONCLUSION Although the limited number of included documents, APT could be beneficial to the prognosis of MMD.
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Affiliation(s)
- Yuting Luo
- Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases; National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China
| | - Zhixin Cao
- Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases; National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China
| | - Heng Ye
- Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases; National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China
| | - Shaoqing Wu
- Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
| | - Xunsha Sun
- Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases; National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China.
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25
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Chen Y, Gong X, Yang Z, Chen F, Wang J. Risk factors and a novel cerebral infarction extent scoring system for postoperative cerebral ischemia in patients with ischemic Moyamoya disease. Sci Rep 2023; 13:5726. [PMID: 37029162 PMCID: PMC10082086 DOI: 10.1038/s41598-022-26985-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2022] [Accepted: 12/22/2022] [Indexed: 04/09/2023] Open
Abstract
Postoperative cerebral ischemic complication is the most common complication of revascularization surgery for patients with moyamoya disease (MMD). This retrospective study was conducted on 63 patients with ischemic MMD. Postoperative ischemia occurred in 15 of the 70 revascularization operations performed for patients after surgical revascularization, translating to an incidence of 21.4%. Univariate analysis revealed that onset infarction (p = 0.015), posterior cerebral artery involvement (p = 0.039), strict perioperative management (p = 0.001), interval time between transient ischemic attack (TIA) or infarction presentation and operation (p = 0.002) and preoperatively cerebral infarction extent score (CIES) (p = 0.002) were significantly associated with postoperative cerebral ischemia. Multivariate analysis revealed that strict perioperative management (OR = 0.163; p = 0.047), and preoperatively CIES (OR = 1.505; p = 0.006) were independently associated with postoperative cerebral ischemia-related complications. After comprehensive improvement of perioperative management protocol, the incidence of symptomatic infarction declined to 7.4% (4 out of 54). Analysis of the area under the receiver operating characteristic curve (AUROC) indicated CIES was a predictor for both postoperative ischemia and high follow-up modified Rankin Scale scores. In summary, strict perioperative management and CIES were identified as independent risk factors for postoperative ischemic complications in ischemic MMD, demonstrating that comprehensive and individualized perioperative management improve postoperative outcomes in patients with MMD. Furthermore, application of CIES to evaluate pre-existing cerebral infarction can improve the management of patients.
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Affiliation(s)
- Yuanbing Chen
- Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Xuan Gong
- Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Zeng Yang
- Department of Neurosurgery, The First People's Hospital of Changde, Changde, Hunan, China
| | - Fenghua Chen
- Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan, China.
| | - Junyu Wang
- Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan, China.
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26
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Oh WO, Shim KW, Yeom IS, Park IT, Heo YJ, Han J. Risk and protective factors for a salutogenic sense of health in adolescents with Moyamoya disease. Int J Nurs Pract 2023; 29:e13066. [PMID: 35644528 DOI: 10.1111/ijn.13066] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2021] [Revised: 03/03/2022] [Accepted: 05/09/2022] [Indexed: 02/04/2023]
Abstract
PURPOSE To identify the risk and protective factors affecting a salutogenic sense of health in adolescents with Moyamoya disease based on Antonovsky's salutogenesis theory. METHODS This study used a qualitative descriptive design. We interviewed adolescents diagnosed with Moyamoya disease and their parents and experts with experience in treating them from 4 July 2019 to 10 October 2019. Data analysis was performed according to the process of deductive content analysis based on Antonovsky's salutogenesis theory. RESULTS This study identified risk and protective factors affecting a salutogenic sense of health in adolescents with Moyamoya disease. Risk factors were closely related to problems with friends, parent relationships and academic work. Protective factors were identified as emotional support through interpersonal relationships, positive attitudes to the disease situation, ability to understand and predict patterns of symptoms, increasing strength to resolve the state of tension and developing available resources. CONCLUSION The factors affecting a salutogenic sense of health in adolescents were identified reflecting on the characteristics of the developmental stage of adolescents and the symptoms and situations that are unique to adolescents. It is important for adolescents with Moyamoya disease to recognize available resources and use them appropriately to successfully manage the state of tension caused by stress.
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Affiliation(s)
- Won-Oak Oh
- College of Nursing, Korea University, Seoul, South Korea
| | - Kyu Won Shim
- Department of Pediatric Neurosurgery, Severance Children's Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - In-Sun Yeom
- College of Nursing and Brain Korea 21 FOUR Project, Yonsei University, Seoul, South Korea
| | - Il Tae Park
- College of Nursing, Korea University, Seoul, South Korea.,Department of Nursing, Woosong University, Daejeon, South Korea
| | - Yoo Jin Heo
- College of Nursing, Korea University, Seoul, South Korea
| | - Jihee Han
- College of Nursing, Korea University, Seoul, South Korea
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27
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Xue Y, Zhang Q, Wang LJ, Tu WJ, Zhao J. Application of Induced Pluripotent Stem Cells in Moyamoya Disease: Progress and Promises. Curr Stem Cell Res Ther 2023; 18:733-739. [PMID: 35674309 DOI: 10.2174/1574888x17666220607121027] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2021] [Revised: 01/16/2022] [Accepted: 02/21/2022] [Indexed: 11/22/2022]
Abstract
Moyamoya disease (MMD) is a chronic steno-occlusion cerebrovascular disease accompanied by the formation of the abnormal vascular network at the base of the brain. The etiology of MMD is not fully clarified. Lack of pathological specimens hinders the research progress. Induced pluripotent stem cells (iPSC) derived from patients with outstanding differentiation potential and infinite proliferation ability could conquer the problem of insufficient samples. The technology of iPSC holds the promise of clarifying the underlying molecular mechanism in the development of MMD. In this review, we summarized the latest progress and difficulties in the research of mechanism and detailed the application of iPSC in MMD, aiming to provide an outlook of iPSC in molecular mechanism and novel therapies of MMD.
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Affiliation(s)
- Yimeng Xue
- Savaid Medical School, University of Chinese Academy of Sciences, Beijing China
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Qian Zhang
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
- Center of Stroke, Beijing Institute for Brain Disorders, Beijing, China
- Beijing Key Laboratory of Translational Medicine for Cerebrovascular Disease, Beijing, China
- China National Clinical Research Center for Neurological Diseases, Beijing, China
| | - Lin-Jian Wang
- Savaid Medical School, University of Chinese Academy of Sciences, Beijing China
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Wen-Jun Tu
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
- Institute of Radiation Medicine, Chinese Academy of Medical Science & Peking Union Medical College, Tianjin, China
| | - Jizong Zhao
- Savaid Medical School, University of Chinese Academy of Sciences, Beijing China
- Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
- Center of Stroke, Beijing Institute for Brain Disorders, Beijing, China
- Beijing Key Laboratory of Translational Medicine for Cerebrovascular Disease, Beijing, China
- China National Clinical Research Center for Neurological Diseases, Beijing, China
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28
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Cao J, Xing Z, Dai L, Wang T, Zhang Y, Feng Y, Chen Y. Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis. Front Neurol 2023; 14:1128338. [PMID: 36937514 PMCID: PMC10018164 DOI: 10.3389/fneur.2023.1128338] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2022] [Accepted: 02/13/2023] [Indexed: 03/06/2023] Open
Abstract
Background The progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients. Methods We searched PubMed, Web of Science, Cochrane Library, and Embase databases up to December 10th, 2022 for randomized controlled trials, case-control studies, or cohort studies reporting predictors of disease progression in MMD patients. The results of each predictor were pooled by meta-analysis and further analyzed by subgroup analysis for predictors of unilateral to bilateral progression of MMD. Results A total of 842 patients from 12 studies were included. The estimated pooled means indicated lower age (standard mean difference [SMD]: -0.29, 95% confidence interval [CI]: -0.55 to -0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P < 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. Subgroup analyses indicated that the same three factors were associated with the progression of unilateral to bilateral MMD. Conclusions This meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.
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Affiliation(s)
- Jun Cao
- Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China
- Department of Neurosurgery, The Affiliated Rizhao People's Hospital, Jining Medical University, Rizhao, China
| | - Zixuan Xing
- Health Science Center, Xi'an Jiaotong University, Xi'an, China
| | - Ling Dai
- Department of Neurosurgery, Jinshan Hospital, Fudan University, Shanghai, China
| | - Tao Wang
- Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Yuhai Zhang
- Department of Neurosurgery, The Affiliated Rizhao People's Hospital, Jining Medical University, Rizhao, China
| | - Yao Feng
- Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China
| | - Yanfei Chen
- Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China
- *Correspondence: Yanfei Chen
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Dong Y, Cao L, Sun K, Li D, Wang H, Zhang M, Li H, Yang B. Hemodynamic changes of donor artery after combined revascularization in adult Moyamoya disease. Heliyon 2022; 8:e12159. [PMID: 36544837 PMCID: PMC9761717 DOI: 10.1016/j.heliyon.2022.e12159] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2022] [Revised: 09/02/2022] [Accepted: 11/29/2022] [Indexed: 12/13/2022] Open
Abstract
To explore the hemodynamic changes of the superficial temporal artery in adult Moyamoya Disease (MMD) who underwent combined revascularization surgery. A number of 40 patients with MMD were enrolled, and all of them underwent a direct superficial temporal artery (STA)-middle cerebral artery (STA-MCA) bypass combined with an encephalo-duro-arterio-synangiosis (EDAS). Hemodynamic parameters were detected by Color Doppler Ultrasonography (CDUS) at the preoperative, perioperative and follow-up time, including peak systolic velocity (PSV), end-diastolic velocity (EDV) and resistance index (RI). The control group were selected randomly during the same period. Researchers applied the SPSS 21 to conduct the two-sample analysis, Chi-Squared test and one-way repeated measures ANOVA between groups. P < 0.05 was considered statistically significant. In this study, 21 males and 19 females with an average age of 44.9 years (Range 28 y-56 y) were enrolled in the MMD group. Among them, 21 patients (52.5%) had perioperative complications, and all symptoms were transient neurological dysfunctions. Intermittent speech disorder was the most common complication during the period of operation. The preoperative hemodynamic of STA showed no significant difference between MMD and the control group. The perioperative hemodynamics had significant carnages compared with preoperative, and there was a trend of fluctuation. The perioperative PSV in the group with complications was significantly higher than the group without complications, except for EDV and RI. In the follow-up ( X ¯ = 5 months), PSV (60.21 ± 22.24 cm/s, P = 0.712) showed no difference compared with baseline data, while EDV (25.12 ± 9.94 cm/s, P = 0.000) and RI (0.575 ± 0.092, P = 0.000) showed significant difference between MMD and control group. The blood flow spectrogram showed high resistance in preoperative, but most patients showed a low resistance pattern during the follow-up time. It was the first time to demonstrate that the hemodynamic changes of STA fluctuated significantly within one week and eventually remained stable after combined revascularization. The PSV may play a more important role in postoperative complications. In the follow-up, PSV had no significant difference, EDV increased significantly, and RI decreased significantly. The blood flow spectrogram mainly shows a low resistance pattern when the hemodynamic is stable.
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30
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Xu R, Kalluri AL, Sun LR, Lawrence CE, Lee JK, Kannan S, Cohen AR. The neurosurgical management of Severe Hemophilia A and Moyamoya (SHAM): challenges, strategies, and literature review. Childs Nerv Syst 2022; 38:1077-1084. [PMID: 35262754 DOI: 10.1007/s00381-022-05489-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2021] [Accepted: 03/03/2022] [Indexed: 11/29/2022]
Abstract
INTRODUCTION Severe Hemophilia A and Moyamoya arteriopathy (SHAM syndrome) is a rare genetic disorder caused by deletion of portions of the cytogenic band Xq28. A case of SHAM syndrome requiring bilateral cerebral revascularization is described with an emphasis on perioperative management. CASE REPORT A 5-year-old boy with severe hemophilia A complicated by factor VIII inhibition presented with right-sided weakness. Imaging revealed multiple strokes and vascular changes consistent with Moyamoya disease. The patient underwent two-staged indirect cerebral bypass revascularizations, first on the left side and several months later on the right. Perioperative management required balancing the administration of agents to prevent coagulopathy and perioperative hemorrhage while mitigating the risk of thromboembolic events associated with bypass surgery. Despite a multidisciplinary effort by the neurosurgery, hematology, critical care, and anesthesiology teams, the post-operative course after both surgeries was complicated by stroke. Fortunately, the patient recovered rapidly to his preoperative functional baseline. CONCLUSION We describe a rare case of SHAM syndrome in a pediatric patient who required bilateral revascularizations and discuss strategies for managing the perioperative risk of hemorrhage and stroke. We also review existing literature on SHAM syndrome.
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Affiliation(s)
- Risheng Xu
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Anita L Kalluri
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Lisa R Sun
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Courtney E Lawrence
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Jennifer K Lee
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Sujatha Kannan
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA
| | - Alan R Cohen
- Johns Hopkins University School of Medicine, Phipps Building 554, 600 N Wolfe St, Baltimore, MD, 21287, USA.
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Sutton CXY, Carrazana E, Mitchell C, Viereck J, Liow KK, Ghaffari-Rafi A. Identification of associations and distinguishing moyamoya disease from ischemic strokes of other etiologies: A retrospective case-control study. Ann Med Surg (Lond) 2022; 78:103771. [PMID: 35734698 PMCID: PMC9206914 DOI: 10.1016/j.amsu.2022.103771] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2022] [Revised: 05/07/2022] [Accepted: 05/08/2022] [Indexed: 12/03/2022] Open
Abstract
Introduction Better characterizing moyamoya disease (MMD) from ischemic strokes of other etiologies may facilitate earlier diagnosis by raising suspicion for a diagnostic work-up. Methods To identify associated variables, MMD cases (n = 12) were compared against three sets of controls: age-, sex-, and race-matched controls of patients with general neurological disorders (n = 48), unmatched general controls (n = 48), and unmatched non-MMD ischemic stroke controls (n = 48). Results MMD patients were 32 years (p < 0.0001) younger than ischemic stroke controls. Relative to non-MMD ischemic strokes, MMD patients had greater odds of presenting with visual field defects (OR: 9.13, p = 0.09) or dizziness (OR: 9.13, p = 0.09), as well as being female (OR: 8.04, p = 0.008), Asian (OR: 3.68, p = 0.087), employed (OR: 6.96, p = 0.02), having migraines (OR: 21.61, p = 0.005), epilepsy (OR: 6.69, p = 0.01), insomnia (OR: 8.90, p = 0.099), and a lower Charlson Comorbidity Index (CCI; p = 0.002). Patients with MMD, compared to non-MMD ischemic strokes, also had a 4.67 kg/ m2 greater body mass index (BMI) and larger odds (OR relative to normal BMI: 21.00, p = 0.03) of being from obesity class III (>40 kg/ m2), yet reduced odds of coronary artery disease (OR: 0.13, p = 0.02). Relative to general controls, MMD patients had greater odds of diabetes mellitus type 2 (OR: 10.07, p = 0.006) and hypertension (OR: 7.28, p = 0.004). Conclusion MMD not only has a unique clinical presentation from other ischemic strokes, but also unique comorbidities, which may facilitate earlier work-up and treatment.
Moyamoya patients are 32 years younger than ischemic strokes of other etiologies. Moyamoya patients are 4.67 kg/ m2 heavier than those with ischemic strokes. Moyamoya patients are at greater odds of type 2 diabetes mellitus and hypertension. Moyamoya patients are at reduced odds of coronary artery disease. Moyamoya patients present more often with visual field deficits or dizziness.
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Xu S, Wei W, Zhang F, Chen T, Dong L, Shi J, Wu X, Zhang T, Li Z, Zhang J, Li X, Chen J. Transcriptomic Profiling of Intracranial Arteries in Adult Patients With Moyamoya Disease Reveals Novel Insights Into Its Pathogenesis. Front Mol Neurosci 2022; 15:881954. [PMID: 35711733 PMCID: PMC9197469 DOI: 10.3389/fnmol.2022.881954] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2022] [Accepted: 04/01/2022] [Indexed: 11/24/2022] Open
Abstract
Moyamoya disease (MMD) is a rare, progressively steno-occlusive cerebrovascular disorder of unknown etiology. Here, we revealed the gene expression profile of the intracranial arteries in MMD via the RNA-sequencing (RNA-seq). We identified 556 differentially expressed genes (DEGs) for MMD, including 449 and 107 significantly upregulated or downregulated genes. Compared with atherosclerosis-associated intracranial artery stenosis/occlusion (AS-ICASO) controls, upregulated genes were mainly involved in extracellular matrix (ECM) organization, whereas downregulated genes were primarily associated with mitochondrial function and oxidative phosphorylation in MMD. Moreover, we found that a separate sex analysis uncovers more DEGs (n = 1.022) compared to an combined sex analysis in MMD. We identified 133 and 439 sex-specific DEGs for men and women in MMD, respectively. About 95.6% of sex-specific DEGs were protein-coding genes and 3% of the genes belonged to long non-coding RNAs (lncRNA). Sex-specific DEGs were observed on all chromosomes, of which 95.49 and 96.59% were autosomal genes in men and women, respectively. These sex-specific DEGs, such as aquaporin-4 (AQP4), superoxide dismutase 3 (SOD3), and nuclear receptor subfamily 4 group A member 1 (NR4A1), may contribute to sex differences in MMD. This transcriptomic study highlighted that ECM and mitochondrial function are the central molecular mechanisms underlying MMD, and revealed sex differences in the gene expression in the intracranial arteries, thereby providing new insights into the pathogenesis of MMD.
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Affiliation(s)
- Shuangxiang Xu
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Wei Wei
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Feiyang Zhang
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Tongyu Chen
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Lixin Dong
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Jichun Shi
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Xiaolin Wu
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Tingbao Zhang
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Zhengwei Li
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
| | - Jianjian Zhang
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
- Jianjian Zhang
| | - Xiang Li
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
- Xiang Li
| | - Jincao Chen
- Department of Neurosurgery, Zhongnan Hospital, Wuhan University, Wuhan, China
- Brain Research Center, Zhongnan Hospital, Wuhan University, Wuhan, China
- *Correspondence: Jincao Chen
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Jeong Y, Jung YJ, Noh E, Ha S, Hwang J, Cho GJ, Oh MJ, Kim YH. The risk for future cerebrovascular disease in pregnant women with Moyamoya disease: a nationwide population-based study in South Korea. BMC Pregnancy Childbirth 2022; 22:433. [PMID: 35610618 PMCID: PMC9128146 DOI: 10.1186/s12884-022-04718-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2021] [Accepted: 04/22/2022] [Indexed: 11/12/2022] Open
Abstract
BACKGROUND Physiologic changes during pregnancy affect the development of postpartum cerebrovascular disease (CVD) in women with Moyamoya disease. Due to the rare prevalence of Moyamoya disease and its large regional variations, large-scale based studies on the risk of CVD after delivery have not been conducted. This study aimed to evaluate whether women with Moyamoya disease have an increased risk of CVD after delivery. METHODS Research data was collected from the National Health Insurance Claims Database of the Health Insurance Review and Assessment Service. Patients who delivered in Korea from 2007 to 2014 were enrolled in this study. We classified women as having CVD if they were diagnosed with any of the following conditions between delivery and December 31, 2016; cerebral infarction (I63.X in the International Classification of Diseases-10th Revision [ICD-10]) and/or intracranial hemorrhage (I61.X, I62.X in ICD-10) and/or subarachnoid hemorrhage (I60.X in ICD-10). Women with Moyamoya disease were identified as having I67.5 in ICD-10. We matched the study cohort by the ratio of 1:10 to analyze the risk CVD occurrence. The matching technique applied in this study was based on the variables of age and parity. To evaluate the adjusted hazard ratio (HR) for CVD in women with Moyamoya disease, we used multivariate Cox proportional hazard regression. RESULTS Among a total of 3,611,216 Korean women who underwent delivered, we identified 412 women with Moyamoya disease diagnosis and 1420 age- and parity-matched women without Moyamoya disease (control). Compared to the control group, women with Moyamoya disease had a significantly higher rate of Cesarean section, overt DM, and essential hypertension (all p < 0.0001). Among women with Moyamoya disease, 55 (13.35%) women developed CVD within the follow-up postpartum period. The presence of Moyamoya disease was associated with an increased risk of CVD after delivery (adjusted HR 37.42; 95% confidence interval (CI) 17.50-80.02 within 2.3 years) after adjusting for pregnancy-induced hypertension, gestational diabetes mellitus, pregestational diabetes, chronic hypertension. CONCLUSION This population based study showed that the occurrence rate of CVD after delivery was higher in women with Moyamoya disease than in those without. Therefore, careful and long-term postpartum surveillance is required for women with Moyamoya disease.
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Affiliation(s)
- Yeonseong Jeong
- Department of Obstetrics and Gynecology, Institute of Women's Life Medical Science, Yonsei University College of Medicine, Yonsei University Health System, Seoul, Republic of Korea
| | - Yun Ji Jung
- Department of Obstetrics and Gynecology, Institute of Women's Life Medical Science, Yonsei University College of Medicine, Yonsei University Health System, Seoul, Republic of Korea
| | - Eunjin Noh
- Korea University Guro Hospital Smart Healthcare Center, Seoul, Republic of Korea
| | - Sungyeon Ha
- Graduate School of Statistics, Sungkyunkwan University, Seoul, Republic of Korea
| | - Jeongeun Hwang
- Department of Biomedical Research Center, Korea University Guro Hospital, Seoul, Republic of Korea
| | - Geum Joon Cho
- Department of Obstetrics and Gynecology, Korea University College of Medicine, Seoul, Republic of Korea.
| | - Min-Jeong Oh
- Department of Obstetrics and Gynecology, Korea University College of Medicine, Seoul, Republic of Korea
| | - Young-Han Kim
- Department of Obstetrics and Gynecology, Institute of Women's Life Medical Science, Yonsei University College of Medicine, Yonsei University Health System, Seoul, Republic of Korea.
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Zhang S, Zhao LM, Xue BQ, Liang H, Guo GC, Liu Y, Wu RY, Li CY. Acute recurrent cerebral infarction caused by moyamoya disease complicated with adenomyosis: A case report. World J Clin Cases 2022; 10:4617-4624. [PMID: 35663064 PMCID: PMC9125300 DOI: 10.12998/wjcc.v10.i14.4617] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/20/2021] [Revised: 02/23/2022] [Accepted: 03/16/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Moyamoya disease is essentially an ischemic cerebrovascular disease. Here, we describe a case of acute recurrent cerebral infarction caused by moyamoya disease with concurrent adenomyosis which, to our knowledge, is the first in the literature. A literature review is also presented. CASE SUMMARY A 38-year-old female presented to the Research and Treatment Center of Moyamoya Disease in our hospital with "left limb weakness" as the main symptom. She was diagnosed with acute cerebral infarction and moyamoya disease through magnetic resonance imaging and digital subtraction angiography. Prior to this, she had experienced a prolonged menstrual period of one-month duration. This was investigated and adenomyosis was diagnosed. After passing the acute cerebral infarction phase, the patient underwent surgery for adenomyosis followed by combined cerebral revascularization. During the postoperative follow-up, improvements of the perfusion imaging stage and modified Rankin Scale were observed. A review of the literature showed only 16 reported cases of gynecological diseases complicated with stroke. The clinical characteristics, pathogenesis, therapeutic effects, and long-term prognosis of these cases have been studied and discussed. CONCLUSION In patients with moyamoya disease, early management of gynecological-related bleeding disorders is essential to prevent the complications of cerebral events.
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Affiliation(s)
- Shao Zhang
- Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Li-Ming Zhao
- Department of Neurosurgery, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Bing-Qian Xue
- Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Hao Liang
- Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Gao-Chao Guo
- Department of Neurosurgery, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Yang Liu
- Department of Neurosurgery, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Rui-Yu Wu
- Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
| | - Chao-Yue Li
- Department of Neurosurgery, Henan University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450000, Henan Province, China
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Lai PMR, Gomez-Paz S, Patel NJ, Frerichs KU, Thomas AJ, Aziz-Sultan MA, Patel AB, Ogilvy CS, Du R. Asymptomatic Moyamoya Disease in a North American Adult Cohort. World Neurosurg 2022; 161:e146-e153. [PMID: 35092810 DOI: 10.1016/j.wneu.2022.01.076] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2021] [Revised: 01/18/2022] [Accepted: 01/19/2022] [Indexed: 11/16/2022]
Abstract
OBJECTIVE The natural history of asymptomatic adult moyamoya disease (MMD) is unclear, and the benefit of treatment remains controversial. This study aimed to investigate the natural history of asymptomatic MMD in a North American cohort and to evaluate risk factors associated with and the effects of treatment on disease progression. METHODS Medical records from 3 institutions of consecutive adult patients with MMD diagnosed between 1984 and 2018 were retrospectively reviewed. Patients with unilateral or bilateral asymptomatic MMD were evaluated for subsequent development of infarction or hemorrhage. Multivariate Cox proportional hazards regression assessed risk factors associated with infarction or hemorrhage, adjusting for age, sex, race, initial Suzuki grade, hypertension, hyperlipidemia, diabetes, obesity, presence of aneurysms, smoking status, aspirin, and statin use at diagnosis. RESULTS We identified 106 hemispheres with asymptomatic MMD in 97 patients with mean 5.1 years (interquartile range, 1.0-7.9 years) of follow-up. Of 106 hemispheres, 59 were treated medically, and 47 were treated with revascularization with direct or indirect bypasses. The medical and surgical cohorts had a 1.9% and 1.3% annual rate of radiographic infarction or hemorrhage per hemisphere, respectively. Cox regression for radiographic events, including early postoperative events, showed no significant difference between the treatment groups (adjusted hazard ratio 0.34 [95% confidence interval 0.05-2.5]). CONCLUSIONS We found an overall 1.7% annual rate of radiographic infarction or hemorrhage in asymptomatic MMD hemispheres. Although we did not find a benefit to surgical treatment within the study period, asymptomatic patients with expected long-term survival may benefit from surgery given the sustained long-term benefits after surgery despite an initial postoperative risk.
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Affiliation(s)
- Pui Man Rosalind Lai
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Santiago Gomez-Paz
- Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
| | - Nirav J Patel
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Kai U Frerichs
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Ajith J Thomas
- Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
| | - M Ali Aziz-Sultan
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Aman B Patel
- Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Christopher S Ogilvy
- Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA
| | - Rose Du
- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
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Luo Y, Cao Z, Wu S, Sun X. Ring Finger Protein 213 in Moyamoya Disease With Pulmonary Arterial Hypertension: A Mini-Review. Front Neurol 2022; 13:843927. [PMID: 35401401 PMCID: PMC8987108 DOI: 10.3389/fneur.2022.843927] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 02/09/2022] [Indexed: 11/28/2022] Open
Abstract
Moyamoya disease (MMD), most often diagnosed in children and adolescents, is a chronic cerebrovascular disease characterized by progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Recently, many investigators show a great interest in MMD with pulmonary arterial hypertension (PAH). Ring finger protein 213 (RNF213) is a major susceptibility gene for MMD and also has strong correlations with PAH. Therefore, this review encapsulates current cases of MMD with PAH and discusses MMD with PAH in the aspects of epidemiology, pathology, possible pathogenesis, clinical manifestations, diagnosis, and treatment.
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Affiliation(s)
- Yuting Luo
- Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Zhixin Cao
- Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
| | - Shaoqing Wu
- Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
- *Correspondence: Shaoqing Wu
| | - Xunsha Sun
- Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
- Xunsha Sun
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Das S, Dubey S, Das S, Hazra A, Pandit A, Ghosh R, Ray BK. Epidemiology of Moyamoya Angiopathy in Eastern India. Front Neurol 2022; 13:837704. [PMID: 35309562 PMCID: PMC8931392 DOI: 10.3389/fneur.2022.837704] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2021] [Accepted: 01/31/2022] [Indexed: 01/15/2023] Open
Abstract
Introduction Moyamoya angiopathy (MMA) is a chronic, progressive intracranial vasculopathy with variation in prevalence and clinical manifestations across different populations. This study was aimed to estimate the frequency of MMA as an etiology of stroke and its epidemiological features in the largest cohort of MMA patients in India. Method A single-centered cross-sectional observational study over a period of 5 years (2016–2021) was undertaken among consecutive stroke and transient ischemic attack (TIA) patients to look for the presence of MMA angiographically. Each patient with angiographically proven MMA was further evaluated for demographic, clinical, and radiological characteristics. Results Among 10,250 consecutive stroke and TIA patients (ischemic = 78%, hemorrhagic = 22%), frequency of MMA was 1.56% (n = 160); 15.3% among children. Female preponderance (Male:Female = 1:1.4) was noted among 160 MMA patients, with bimodal age distribution, first peak at 3–8 years, and a shorter second peak at 41–47 years. Childhood-onset MMA was seen in 75 (46.9%) with commonest initial neurological symptom of fixed-motor-weakness (44.0%), followed by TIA (26.7%); while 85 (53.1%) had adult-onset MMA with fixed-motor-weakness (50.6%) followed by headache (24.7%) as the predominant initial neurological symptom; seizure significantly higher in children (p < 0.001) and headache in adults (p = 0.012). Transient and fixed neurological manifestations constituted 87.5 and 69.4% respectively, of symptoms throughout the disease course. Cerebral infarction (45.0%) and TIA (21.9%) were the commonest types of MMA. On brain imaging, infarction was noted in 80.6%, hemorrhage in 11.3%, significantly higher among adults (p < 0.001). Cortical infarct and Gyral pattern were commoner in children (p = 0.004), subcortical infarcts in adults (p = 0.018). Frequent Suzuki staging observed was stage 4 (31.3%), followed by stage 3 (30.0%). Involvement of posterior circulation was detected in 55.6%, brain atrophy at the time of diagnosis was seen in 65.0%. Conclusion MMA is an important etiological consideration in patients with stroke, especially in children. It can present with a myriad of transient neurological symptoms, frequently overlooked, leading to delayed diagnosis, and contributing to socio-economic burden. Indian MMA showed aberrations in its gender predisposition, age distribution, frequency of familial cases, disease manifestation, and type of stroke, in comparison to its Japanese and Caucasian counterparts pointing to the inter- and intra-continent differences of MMA phenotype. Future development of the Indian MMA national registry is of essence.
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Affiliation(s)
- Shambaditya Das
- Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India
| | - Souvik Dubey
- Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India
| | - Suman Das
- Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India
| | - Avijit Hazra
- Department of Pharmacology, Institute of Post Graduate Medical Education & Research, Kolkata, India
| | - Alak Pandit
- Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India
| | - Ritwik Ghosh
- Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, India
| | - Biman Kanti Ray
- Department of Neurology, Institute of Post Graduate Medical Education & Research, Bangur Institute of Neurosciences, Kolkata, India
- *Correspondence: Biman Kanti Ray
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Yin HL, Jiang Y, Huang WJ, Li SH, Lin GW. A Magnetic Resonance Angiography-Based Study Comparing Machine Learning and Clinical Evaluation: Screening Intracranial Regions Associated with the Hemorrhagic Stroke of Adult Moyamoya Disease. J Stroke Cerebrovasc Dis 2022; 31:106382. [PMID: 35183983 DOI: 10.1016/j.jstrokecerebrovasdis.2022.106382] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2021] [Revised: 01/25/2022] [Accepted: 01/29/2022] [Indexed: 02/05/2023] Open
Abstract
OBJECTIVES Moyamoya disease patients with hemorrhagic stroke usually have a poor prognosis. This study aimed to determine whether hemorrhagic moyamoya disease could be distinguished from MRA images using transfer deep learning and to screen potential regions that contain rich distinguishing information from MRA images in moyamoya disease. MATERIALS AND METHODS A total of 116 adult patients with bilateral moyamoya diseases suffering from hemorrhagic or ischemia complications were retrospectively screened. Based on original MRA images at the level of the basal cistern, basal ganglia, and centrum semiovale, we adopted the pretrained ResNet18 to build three models for differentiating hemorrhagic moyamoya disease. Grad-CAM was applied to visualize the regions of interest. RESULTS For the test set, the accuracies of model differentiation in the basal cistern, basal ganglia, and centrum semiovale were 93.3%, 91.5%, and 86.4%, respectively. Visualization of the regions of interest demonstrated that the models focused on the deep and periventricular white matter and abnormal collateral vessels in hemorrhagic moyamoya disease. CONCLUSION A transfer learning model based on MRA images of the basal cistern and basal ganglia showed a good ability to differentiate between patients with hemorrhagic moyamoya disease and those with ischemic moyamoya disease. The deep and periventricular white matter and collateral vessels at the level of the basal cistern and basal ganglia may contain rich distinguishing information.
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Affiliation(s)
- Hao-Lin Yin
- Department of Radiology, Huadong Hospital Affiliated to Fudan University, No. 221 Yan'anxi Road, Jing'an District, Shanghai 200040, China
| | - Yu Jiang
- Department of Radiology, West China Hospital, Sichuan University, 37# Guo Xue Xiang, Chengdu, Sichuan 610041, China
| | - Wen-Jun Huang
- Department of Radiology, Huadong Hospital Affiliated to Fudan University, No. 221 Yan'anxi Road, Jing'an District, Shanghai 200040, China
| | - Shi-Hong Li
- Department of Radiology, Huadong Hospital Affiliated to Fudan University, No. 221 Yan'anxi Road, Jing'an District, Shanghai 200040, China
| | - Guang-Wu Lin
- Department of Radiology, Huadong Hospital Affiliated to Fudan University, No. 221 Yan'anxi Road, Jing'an District, Shanghai 200040, China.
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Spinal Versus General Anesthesia for Cesarean Delivery in Pregnant Women With Moyamoya Disease: A Retrospective Observational Study. Anesth Analg 2022; 135:617-624. [PMID: 35089266 DOI: 10.1213/ane.0000000000005919] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
BACKGROUND Moyamoya disease, a rare chronic cerebrovascular disease with a fragile vascular network at the base of the brain, can cause ischemic or hemorrhagic strokes or seizures. Precise blood pressure control and adequate analgesia are important for patients with moyamoya disease to prevent neurological events such as ischemia and hemorrhage. This study aimed to compare the intraoperative mean arterial pressure of pregnant women with moyamoya disease according to the mode of anesthesia (general anesthesia versus spinal anesthesia) used during cesarean delivery. METHODS We retrospectively reviewed the medical records of 87 cesarean deliveries in 74 patients who had been diagnosed with moyamoya disease before cesarean delivery. The primary outcome, intraoperative maximum mean arterial pressure during anesthesia, was compared according to the type of anesthesia administered (general versus spinal anesthesia). Other perioperative hemodynamic data (lowest mean arterial pressure, incidence of hypotension, vasopressor use, and antihypertensive agent use), maternal neurologic symptoms, neonatal outcomes (Apgar scores <7, ventilatory support, and intensive care unit admission), maternal and neonatal length of stay, postoperative pain scores, and rescue analgesic use were assessed as secondary outcomes. RESULTS While the lowest blood pressure during anesthesia and incidence of hypotension did not differ between the 2 groups, the maximum mean arterial pressure during anesthesia was lower in the spinal anesthesia group than that in the general anesthesia group (104.8 ± 2.5 vs 122.0 ± 4.6; P = .002). Study data did not support the claim that maternal neurologic symptoms differ according to the type of anesthesia used (5.6% vs 9.3%; P = .628); all patients recovered without any sequelae. The postoperative pain scores were lower, and fewer rescue analgesics were used in the spinal anesthesia group than in the general anesthesia group. Other maternal and neonatal outcomes were not different between the 2 groups. CONCLUSIONS Compared with general anesthesia, spinal anesthesia mitigated the maximum arterial blood pressure during cesarean delivery and improved postoperative pain in patients with moyamoya disease.
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Zhang D, Huang L, Huang Z, Zhou Q, Yang X, Gu H, Li Z, Shi Y, Gan L, Wang H, Ma X, Wang Y, Zhao J. Epidemiology of Moyamoya disease in China: A nationwide hospital-based study. THE LANCET REGIONAL HEALTH. WESTERN PACIFIC 2022; 18:100331. [PMID: 35024660 PMCID: PMC8669373 DOI: 10.1016/j.lanwpc.2021.100331] [Citation(s) in RCA: 21] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/02/2022]
Abstract
Background The national epidemiologic data in mainland China is still absent for moyamoya disease (MMD). Methods This study was a nationwide hospital-based observational retrospective study to estimate epidemiological characteristics of MMD. The data was based on the Hospital Quality Monitoring System (HQMS), a national database which covers all tertiary hospitals in mainland China. This system consistently collects medical records including demographic characteristics, diagnoses, procedures, and expenses etc. for all inpatients. MMD was identified by ICD-10 code (I67·5) in HQMS. Findings A total of 47,443 new-onset patients with total 69,680 hospitalization records from 1312 hospitals during 2016 to 2018 were included. The annual incidence rate was 1·14 per 100,000 inhabitants (95% CI, 1·12–1·16) and approximately a 2-fold increase from 2016 to 2018. The incidence in children (0·18 per 100,000 inhabitants per year; 95% CI, 0·17–0·20) was significantly lower than that in adults (1·40 per 100,000; 95% CI, 1·38–1·42) (P<0·001) and the peak incidence was 45–54 years. The distribution model of incidence rate was presented as a clustered regional pattern (Moran's I = 0·155, P = 0·018, Z = 2·375) by global spatial correlation analysis. Interpretation Our study reported the annual incidence of MMD was 1·14 per 100,000 inhabitants in mainland China during 2016 to 2018, and it was increasing year by year. The geographical distribution of MMD incidence presented as a clustered regional pattern, which may provide new view for future study on the etiology for MMD. Funding National Natural Science Foundation of China and “13th Five-Year Plan” National Science and Technology Supporting Plan.
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Affiliation(s)
- Dong Zhang
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Liangran Huang
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Zheng Huang
- Department of Neurosurgery, Xiangya Hospital Central South University, Changsha, Hunan, China
| | - Qi Zhou
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Xin Yang
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Hongqiu Gu
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Zixiao Li
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Ying Shi
- China Standard Medical Information Research Center, Shenzhen, Guangdong, China
| | - Lanxia Gan
- China Standard Medical Information Research Center, Shenzhen, Guangdong, China
| | - Haibo Wang
- Clinical Trial Unit, Sun Yat-Sen University First Affiliated Hospital, Guangzhou, Guangdong, China
| | - Xvdong Ma
- National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Yongjun Wang
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,National Center for Healthcare Quality Management in Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
| | - Jizong Zhao
- China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.,Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
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Ijner P, Tompkins G, Shiohama T, Takahashi E, Levman J. Structural Abnormalities in Pediatric Moyamoya Disease Revealed by Clinical Magnetic Resonance Imaging, Regionally Distributed Relative Signal Intensities and Volumes. Int J Dev Neurosci 2021; 82:146-158. [PMID: 34969179 DOI: 10.1002/jdn.10167] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2021] [Revised: 12/15/2021] [Accepted: 12/22/2021] [Indexed: 11/05/2022] Open
Abstract
Moyamoya disease (MMD) is a rare, progressive cerebrovascular disorder, with an unknown etiology and pathogenesis. It is characterized by steno-occlusive changes at the terminal portion of the internal carotid artery (ICA), which is accompanied by variable development of the basal collaterals called moyamoya vessels. In this study, we investigate the potential for structural T1 magnetic resonance imaging (MRI) to help characterize MMD clinically, with the help of regionally distributed relative signal intensities (RRSIs) and volumes (RRVs). These RRSIs and RRVs provide the ability to characterize aspects of regional brain development and represent an extension to existing automated biomarker extraction technologies. This study included 269 MRI examinations from MMD patients and 993 MRI examinations from neurotypical controls, with regional biomarkers compared between groups with the area under the receiver operating characteristic curve (AUC). Results demonstrate abnormal presentation of RRSIs and RRVs in the insula (15-20 year old cohort, left AUC: 0.74, right AUC: 0.71), and the lateral orbitofrontal region (5-10 year old cohort, left AUC: 0.67; 15-20 year cohort, left AUC: 0.62, right AUC: 0.65). Results indicate that RRSIs and RRVs may help in characterizing brain development, assist in the assessment of the presentation of the brains of children with MMD, and may help overcome standardization challenges in multi-protocol clinical MRI. Further investigation of the potential for RRSIs and RRVs in clinical imaging is warranted and supported through the release of open source software.
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Affiliation(s)
- Prahar Ijner
- Department of Computer Science, St. Francis Xavier University, Antigonish, NS, Canada
| | - Grace Tompkins
- Department of Mathematics and Statistics, St. Francis Xavier University, Antigonish, NS, Canada
| | - Tadashi Shiohama
- Department of Pediatrics, Graduate School of Medicine, Chiba University, Japan
| | - Emi Takahashi
- Division of Newborn Medicine, Department of Medicine, Boston Children's Hospital, Boston, MA, USA.,Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Charlestown, MA, USA.,Department of Radiology, Harvard Medical School, Boston, MA, USA
| | - Jacob Levman
- Department of Computer Science, St. Francis Xavier University, Antigonish, NS, Canada
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The association between the Moyamoya disease susceptible gene RNF213 variant and incident cardiovascular disease in a general population: the Nagahama study. J Hypertens 2021; 39:2521-2526. [PMID: 34738993 DOI: 10.1097/hjh.0000000000002964] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
OBJECTIVE An association between the Moyamoya disease susceptible gene ring finger protein 213 (RNF213) variant and ischemic stroke and coronary artery disease has been suggested in case-control studies. We aimed to investigate the possible association between the RNF213 variant and the incidence of cardiovascular disease in a general population. METHODS The study participants consisted of 9153 Japanese community residents without history of cardiovascular disease. The clinical parameters employed in this analysis were observed at baseline between 2008 and 2010. The RNF213 p.R4859K variant was determined by TaqMan probe assay and then confirmed by Sanger sequencing. RESULTS During 8.52 years follow-up period, we observed 214 incident cases of cardiovascular diseases (99 total stroke cases, 119 major adverse cardiac event cases, including 4 cases of both). The incidence rate was higher for the variant allele carriers (120 cases; incidence rate, 71.0 per 10 000 person-years) than for the homozygotes of the wild-type allele (26.9), and the group differences achieved statistical significance (P = 0.009). Although the RNF213 variant was also associated with systolic blood pressure (dominant model: coefficient of 8.19 mmHg; P < 0.001), the Cox regression analysis adjusted for major covariates including systolic blood pressure identified the RNF213 variant as an independent determinant for cardiovascular disease (hazard ratio of 3.41, P = 0.002) and major adverse cardiac event (hazard ratio of 3.80, P = 0.010) but not with total stroke (P = 0.102). CONCLUSION The Moyamoya disease susceptible RNF213 variant was associated with blood pressure and the incidence of cardiovascular disease in a Japanese general population.
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43
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Im SH, Jang DK, Kim H, Park SK, Han KD. Long-term mortality in patients with moyamoya angiopathy according to stroke presentation type in South Korea. Acta Neurochir (Wien) 2021; 163:3473-3481. [PMID: 34427768 DOI: 10.1007/s00701-021-04959-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2021] [Accepted: 07/29/2021] [Indexed: 12/01/2022]
Abstract
BACKGROUND Incidence, prevalence, and long-term survival outcomes in patients with moyamoya angiopathy (MMA) according to stroke presentation type and age group have not been clearly elucidated. METHODS We investigated mortality in patients with MMA (moyamoya disease, probable moyamoya disease, moyamoya syndrome) of whose International Classification Disease 10 code was I67.5 from 2006 to 2015 using the Korean National Health Insurance database. MMA at diagnosis was classified into 3 types (ischemic, hemorrhagic, and asymptomatic or else) according to stroke presentation. Survival analysis was performed according to stroke presentation type and age group (< 15 years and ≥ 15 years) using the Kaplan-Meier method. RESULTS There were 12,146 newly diagnosed moyamoya cases, with a female-to-male ratio of 1.81; the ischemic type was identified in 3671 (30.2%) patients, the hemorrhagic type in 2449 (20.2%) patients, and the asymptomatic or else type in 6026 (49.6%) patients. The mean age at diagnosis according to stroke presentation was 33.1 (± 14.8) years in asymptomatic or else type, 41.2 (± 17.3) years in ischemic type, and 45.4 (± 14.3) years in hemorrhagic type (P < 0.001). The 10-year survival rates in ischemic-, hemorrhagic-, and asymptomatic or else-type patients were 88.9%, 76.3%, and 94.3%, respectively (log-rank test; P < 0.001). Pediatric MMA (< 15 years) and adult MMA (≥ 15 years) showed different survival curves according to stroke presentation type (log-rank test; P = 0.017, P < 0.001, respectively). CONCLUSIONS Our study showed that moyamoya patients had different diagnosis ages and distinct survival courses according to stroke presentation type. Adult moyamoya patients with hemorrhagic presentation had the worst survival outcomes.
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Affiliation(s)
- Sang-Hyuk Im
- Department of Neurosurgery, Eunpyeong St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Dong-Kyu Jang
- Department of Neurosurgery, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
- Department of Neurosurgery, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 56, Dongsu-ro, Bupyeong-gu, Incheon, 21431, Republic of Korea.
| | - Hoon Kim
- Department of Neurosurgery, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Sang-Kyu Park
- Department of Neurosurgery, Gangnam Severance Hospital, Yonsei University College of Medicine, 211, Eonju-ro, Gangnam-gu, Seoul, 06273, Republic of Korea
| | - Kyung-Do Han
- Department of Biostatistics, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
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44
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Ma Z, Wang X, Li M, Zhou D, Chen J. An ecological comparison study on the causal association between leptospirosis and moyamoya disease in Hubei, China, 2017-2019. Clin Neurol Neurosurg 2021; 210:107007. [PMID: 34741974 DOI: 10.1016/j.clineuro.2021.107007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Revised: 09/17/2021] [Accepted: 10/21/2021] [Indexed: 11/30/2022]
Abstract
OBJECTIVE The importance of environmental factors (especially leptospirosis) of moyamoya disease (MMD) has not been clarified. Here we investigated the epidemiological characteristics of MMD in Hubei province, China. METHODS We conducted a population-based study to describe the epidemiologic characteristics of MMD in Hubei province between 2017 and 2019. The regional clusters of the hot spots (high incidence) and cold spots (low incidence) of MMD were identified using the spatial statistical method. To evaluate the role of leptospirosis in MMD, we performed an ecological comparison study to evaluate whether the socioeconomic and environmental variables of hot spots are more suitable for leptospirosis spread. RESULTS The average annual sex and age adjusted incidence of MMD was 3.07 per 100,000 person-years from 2017 to 2019. The middle-aged had apparently higher incidence of MMD than the children. There existed an obvious geographic distribution difference of MMD at county level. The hot spots were identified mainly in the low mountainous and hilly terrain, while cold spots were located in the Jianghan Plains. Compared to cold spots, the hot spots had larger cattle density (32.0 vs 3.1, p = 0.002), higher percentages of rice field (85.6% vs 47.0%, p = 0.007), and lower elevation (33.6 vs 157.4, p < 0.001) CONCLUSIONS: There exists geographic distribution difference of MMD in Hubei province and was likely to be caused by leptospirosis in the early years. The MMD in China is dominant by middle-aged adults, which might be driven from leptospirosis outbreaks in the last century.
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Affiliation(s)
- Zhiyang Ma
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China
| | - Xiong Wang
- Research Centre for Spatial Planning and Human-Environment System Simulation, School of Geography and Information Engineering, China University of Geosciences (Wuhan), Wuhan 430074, China
| | - Meifang Li
- School of Geography and Environment, Jiangxi Normal University, Nanchang 330022, China
| | - Da Zhou
- Information Statistical Bureau, Hubei Health Committee, Wuhan 430079, China
| | - Jincao Chen
- Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan 430071, China.
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Mertens R, Graupera M, Gerhardt H, Bersano A, Tournier-Lasserve E, Mensah MA, Mundlos S, Vajkoczy P. The Genetic Basis of Moyamoya Disease. Transl Stroke Res 2021; 13:25-45. [PMID: 34529262 PMCID: PMC8766392 DOI: 10.1007/s12975-021-00940-2] [Citation(s) in RCA: 73] [Impact Index Per Article: 18.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Revised: 08/18/2021] [Accepted: 08/20/2021] [Indexed: 12/19/2022]
Abstract
Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive spontaneous bilateral occlusion of the intracranial internal cerebral arteries (ICA) and their major branches with compensatory capillary collaterals resembling a “puff of smoke” (Japanese: Moyamoya) on cerebral angiography. These pathological alterations of the vessels are called Moyamoya arteriopathy or vasculopathy and a further distinction is made between primary and secondary MMD. Clinical presentation depends on age and population, with hemorrhage and ischemic infarcts in particular leading to severe neurological dysfunction or even death. Although the diagnostic suspicion can be posed by MRA or CTA, cerebral angiography is mandatory for diagnostic confirmation. Since no therapy to limit the stenotic lesions or the development of a collateral network is available, the only treatment established so far is surgical revascularization. The pathophysiology still remains unknown. Due to the early age of onset, familial cases and the variable incidence rate between different ethnic groups, the focus was put on genetic aspects early on. Several genetic risk loci as well as individual risk genes have been reported; however, few of them could be replicated in independent series. Linkage studies revealed linkage to the 17q25 locus. Multiple studies on the association of SNPs and MMD have been conducted, mainly focussing on the endothelium, smooth muscle cells, cytokines and growth factors. A variant of the RNF213 gene was shown to be strongly associated with MMD with a founder effect in the East Asian population. Although it is unknown how mutations in the RNF213 gene, encoding for a ubiquitously expressed 591 kDa cytosolic protein, lead to clinical features of MMD, RNF213 has been confirmed as a susceptibility gene in several studies with a gene dosage-dependent clinical phenotype, allowing preventive screening and possibly the development of new therapeutic approaches. This review focuses on the genetic basis of primary MMD only.
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Affiliation(s)
- R Mertens
- Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Neurosurgery, Berlin, Germany
| | - M Graupera
- Vascular Biology and Signalling Group, ProCURE, Oncobell Program, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Catalonia, Barcelona, Spain
| | - H Gerhardt
- Integrative Vascular Biology Laboratory, Max-Delbrück Center for Molecular Medicine in the Helmholtz Association (MDC), Berlin, Germany
| | - A Bersano
- Cerebrovascular Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - E Tournier-Lasserve
- Department of Genetics, NeuroDiderot, Lariboisière Hospital and INSERM UMR-1141, Paris-Diderot University, Paris, France
| | - M A Mensah
- Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Institute of Medical Genetics and Human Genetics, Berlin, Germany.,BIH Biomedical Innovation Academy, Digital Clinician Scientist Program, Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany
| | - S Mundlos
- Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Institute of Medical Genetics and Human Genetics, Berlin, Germany.,Max Planck Institute for Molecular Genetics, RG Development & Disease, Berlin, Germany
| | - P Vajkoczy
- Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Department of Neurosurgery, Berlin, Germany.
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Clinical Management of Moyamoya Patients. J Clin Med 2021; 10:jcm10163628. [PMID: 34441923 PMCID: PMC8397113 DOI: 10.3390/jcm10163628] [Citation(s) in RCA: 21] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2021] [Revised: 08/11/2021] [Accepted: 08/13/2021] [Indexed: 01/01/2023] Open
Abstract
Moyamoya angiopathy (MMA) is a peculiar cerebrovascular condition characterized by progressive steno-occlusion of the terminal part of the internal carotid arteries (ICAs) and their proximal branches, associated with the development of a network of fragile collateral vessels at the base of the brain. The diagnosis is essentially made by radiological angiographic techniques. MMA is often idiopathic (moyamoya disease-MMD); conversely, it can be associated with acquired or hereditary conditions (moyamoya Syndrome-MMS); however, the pathophysiology underlying either MMD or MMS has not been fully elucidated to date, and this poor knowledge reflects uncertainties and heterogeneity in patient management. MMD and MMS also have similar clinical expressions, including, above all, ischemic and hemorrhagic strokes, then headaches, seizures, cognitive impairment, and movement disorders. The available treatment strategies are currently shared between idiopathic MMD and MMS, including pharmacological and surgical stroke prevention treatments and symptomatic drugs. No pharmacological treatment able to reverse the progressive disappearance of the ICAs has been found to date in both idiopathic and syndromic cases. Antithrombotic agents are usually prescribed in ischemic MMA, although the coexisting hemorrhagic risk should be considered. Surgical revascularization techniques, which are currently the best available treatment in symptomatic MMA, are associated with good long-term outcomes and reduced ischemic and hemorrhagic risks. Given the lack of dedicated randomized clinical trials, current treatment is mainly based on observational studies and physicians’ and surgeons’ expertise.
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Zhang X, Xiao W, Zhang Q, Xia D, Gao P, Su J, Yang H, Gao X, Ni W, Lei Y, Gu Y. Progression in Moyamoya Disease: Clinical Feature, Neuroimaging Evaluation and Treatment. Curr Neuropharmacol 2021; 20:292-308. [PMID: 34279201 PMCID: PMC9413783 DOI: 10.2174/1570159x19666210716114016] [Citation(s) in RCA: 30] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2021] [Revised: 05/08/2021] [Accepted: 07/09/2021] [Indexed: 11/22/2022] Open
Abstract
Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by progressive stenosis of the arteries of the circle of Willis, with the formation of collateral vascular network at the base of the brain. Its clinical manifestations are complicated. Numerous studies have attempted to clarify the clinical features of MMD, including its epidemiology, genetic characteristics, and pathophysiology. With the development of neuroimaging techniques, various neuroimaging modalities with different advantages have deepened the understanding of MMD in terms of structural, functional, spatial, and temporal dimensions. At present, the main treatment for MMD focuses on neurological protection, cerebral blood flow reconstruction, and neurological rehabilitation, such as pharmacological treatment, surgical revascularization, and cognitive rehabilitation. In this review, we discuss recent progress in understanding the clinical features, in the neuroimaging evaluation and treatment of MMD.
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Affiliation(s)
- Xin Zhang
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Weiping Xiao
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Qing Zhang
- Department of Nursing, Huashan Hospital North, Fudan University, China
| | - Ding Xia
- Department of Radiology, Huashan Hospital North, Fudan University, China
| | - Peng Gao
- Department of Radiology, Huashan Hospital North, Fudan University, China
| | - Jiabin Su
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Heng Yang
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Xinjie Gao
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Wei Ni
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Yu Lei
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
| | - Yuxiang Gu
- Department of Neurosurgery, Huashan Hospital, Shanghai Medical College, Fudan University, China
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Kaseka ML, Slim M, Muthusami P, Dirks PB, Westmacott R, Kassner A, Bhathal I, Williams S, Shroff M, Logan W, Moharir M, MacGregor DL, Pulcine E, deVeber GA, Dlamini N. Distinct Clinical and Radiographic Phenotypes in Pediatric Patients With Moyamoya. Pediatr Neurol 2021; 120:18-26. [PMID: 33962345 DOI: 10.1016/j.pediatrneurol.2021.03.002] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/03/2020] [Revised: 03/10/2021] [Accepted: 03/13/2021] [Indexed: 11/19/2022]
Abstract
BACKGROUND Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. METHODS We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. RESULTS A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). CONCLUSIONS Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.
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Affiliation(s)
- Matsanga Leyila Kaseka
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
| | - Mahmoud Slim
- Department of Physiology, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Experimental Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Prakash Muthusami
- Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Peter B Dirks
- Department of Neurosurgery, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Robyn Westmacott
- Department of Psychology, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Andrea Kassner
- Department of Physiology, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Ishvinder Bhathal
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Suzan Williams
- Division of Haematology & Oncology, SickKids, University of Toronto, Toronto, Ontario, Canada
| | - Manohar Shroff
- Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada
| | - William Logan
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Mahendranath Moharir
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Daune L MacGregor
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Elizabeth Pulcine
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Gabrielle A deVeber
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Nomazulu Dlamini
- Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
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Srinivasan HL, Hausman-Kedem M, Smith ER, Constantini S, Roth J. Current trends in pediatric moyamoya: a survey of international practitioners. Childs Nerv Syst 2021; 37:2011-2023. [PMID: 33694129 DOI: 10.1007/s00381-021-05074-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2020] [Accepted: 02/04/2021] [Indexed: 11/28/2022]
Abstract
OBJECTIVE Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide. METHODS A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments. RESULTS One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers. CONCLUSION This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.
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Affiliation(s)
- Harishchandra Lalgudi Srinivasan
- Pediatric Neurosurgery Department, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel
| | - Moran Hausman-Kedem
- Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.,Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Edward R Smith
- Pediatric Cerebrovascular Surgery, Pediatric Neurosurgery, Children's Hospital Boston/Harvard Medical School, Boston, MA, USA
| | - Shlomi Constantini
- Pediatric Neurosurgery Department, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.,Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Jonathan Roth
- Pediatric Neurosurgery Department, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel. .,Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
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Choi S, Sung JE, Jo E, Jeong JH. Language treatment effects on communicative abilities and working memory in Korean-speaking agrammatic Broca's aphasia caused by moyamoya disease: Phase II evidence from a case study. Neurocase 2021; 27:297-307. [PMID: 34338151 DOI: 10.1080/13554794.2021.1950768] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
The present study reports on the language treatment outcomes from sentence- and story-level linguistic facilitation and its generalization effect on communicative abilities, working memory, and sentence processing in the case of an adult with Moyamoya Disease (MMD). After treatment,the patient's overall performance, including the Aphasia Quotient, and sentence processing ability as measured by language testing, were improved. Furthermore, the treatment effects were generalizable to working memory abilities. Our case study conveys clinically meaningful implications since it is the first report on the effects of language treatment on linguistic and cognitive domains for an individual with MMD-induced agrammatic Broca's aphasia.
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Affiliation(s)
- Sujin Choi
- Department of Communication Disorders, Ewha Womans University, Seoul, Korea
| | - Jee Eun Sung
- Department of Communication Disorders, Ewha Womans University, Seoul, Korea
| | - Eunha Jo
- Department of Communication Disorders, Ewha Womans University, Seoul, Korea
| | - Jee Hyang Jeong
- Department of Neurology, Ewha Womans University Seoul Hospital, Ewha Womans University School of Medicine, Seoul, Korea
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