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Özdemir HN, Kavalcı D, Gökçay F, Çelebisoy N. Pursuing the impact of headaches in patients with idiopathic intracranial hypertension: a prospective cohort study. Neurol Res 2025:1-9. [PMID: 40401644 DOI: 10.1080/01616412.2025.2508864] [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: 03/05/2025] [Accepted: 05/13/2025] [Indexed: 05/23/2025]
Abstract
OBJECTIVE Persistent headaches have been reported in a significant percentage of patients with idiopathic intracranial hypertension (IIH). This study aimed to evaluate possible factors underlying post-IIH headache. METHODS IIH patients were evaluated for demographic and clinical features, along with the Headache Impact Test (HIT-6) and Migraine Disability Assessment Scale (MIDAS) questionnaires to assess headache-related impact and disability. Features noted at the initial visit were compared with those at follow-up after resolution of papilledema. RESULTS Of the 91 patients included, 92.3% reported headaches at the initial visit, with 73.8% having headaches consistent with migraine. After resolution of papilledema, 54.9% continued to report headaches. Median HIT-6 and MIDAS scores at follow-up were significantly lower than at the first visit (p < 0.001 for both). On univariate regression analysis, none of the demographic or clinical features investigated, including CSF opening pressure, were associated with post-resolution headaches (p > 0.05). Higher HIT-6 and MIDAS scores at the first visit were associated with higher scores at follow-up (p < 0.05). Cerebrospinal fluid opening pressure was not linked to the presence or impact of post-resolution headaches (p > 0.05). Prior to IIH, 73.6% of patients reported headaches, with 73.1% of these consistent with migraine. Prior migraine was associated with higher HIT-6 and MIDAS scores at the initial visit (p = 0.02 and p < 0.001, respectively). CONCLUSION Migraine headaches before IIH diagnosis are associated with more severe and disabling headaches initially. Higher initial HIT-6 or MIDAS scores predict increased disability post-resolution. Headache-specific treatments should be further explored in patients with IIH.
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Affiliation(s)
| | | | - Figen Gökçay
- Department of Neurology, Ege University Medical School, İzmir, Turkey
| | - Neşe Çelebisoy
- Department of Neurology, Ege University Medical School, İzmir, Turkey
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Huang-Link Y, Eriksson S, Schmiauke J, Schmiauke U, Fredrikson M, Borgström M, Yang G. Optical coherence tomography surpasses fundus imaging and intracranial pressure measurement in monitoring idiopathic intracranial hypertension. Sci Rep 2025; 15:14859. [PMID: 40295571 PMCID: PMC12037751 DOI: 10.1038/s41598-025-96831-9] [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: 12/23/2024] [Accepted: 04/01/2025] [Indexed: 04/30/2025] Open
Abstract
We aim to evaluate the retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT) in comparison with papilledema grade, and to assess the relationship between RNFL thickness, papilledema grade, and intracranial pressure (ICP) in idiopathic intracranial hypertension (IIH). Sixty-five patients with active IIH (AIIH) with papilledema, 39 with chronic IIH (CIIH) without papilledema and 80 healthy controls (HC) were examined with OCT and fundus imaging. RNFL thickness, papilledema grade and ICP level were assessed in 55 with AIIH and 26 with CIIH. RNFL thickness was significantly higher in AIIH compared to CIIH or HC. RNFL thickness correlated strongly with papilledema grade (coefficient 0.78, p < 0.01) and moderately with ICP (coefficient 0.569, p < 0.01). RNFL thickness was associated with papilledema progression (R2 = 0.656, p < 0.01): specifically, with increases of 9 µm from normal to mild grade (p > 0.05), 91 µm from normal to moderate (p < 0.01), and 214 µm from normal to severe (p < 0.01). ICP showed a weaker correlation with papilledema grades (R2 = 0.339, p < 0.05), with significant increase (8 cm H2O, p < 0.01) only from normal to severe papilledema. RNFL correlated strongly with papilledema grade and moderately with ICP levels. RNFL thickness increased proportionally per papilledema grade.
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Affiliation(s)
- Yumin Huang-Link
- Division of Neurology, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden.
| | - Sanna Eriksson
- Faculty of Medicine and Healthy Sciences, Linköping University, Linköping, Sweden
| | - Jan Schmiauke
- Division of Neurology, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
| | - Ursula Schmiauke
- Division of Neurology, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
| | - Mats Fredrikson
- Forum Östergötland and Department of Biomedical and Clinical Sciences, Linköping University Hospital, Linköping, Sweden
| | - Max Borgström
- Division of Psychiatry, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
| | - Ge Yang
- Huizhou Aier Eye Hospital, Huizhou, China
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Mollan SP. Papilledema. Continuum (Minneap Minn) 2025; 31:436-462. [PMID: 40179403 DOI: 10.1212/con.0000000000001556] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/05/2025]
Abstract
OBJECTIVE This article describes the recognition, investigation, and management of papilledema. LATEST DEVELOPMENTS Papilledema describes optic disc swelling (usually bilateral) arising from raised intracranial pressure. Due to the serious nature of papilledema, there is a fear of underdiagnosis and hence one of the major stumbling points is the correct identification of papilledema. While there has been a stepwise increase in the incidence and prevalence of idiopathic intracranial hypertension due to the worldwide epidemic of people living with obesity, secondary causes such as cerebral venous sinus thrombosis, intracranial masses, and infections must not be missed. Our ability to image the optic nerve has been greatly enhanced by the directed use of optical coherence tomography, with recent studies observing novel imaging findings that can help distinguish pseudopapilledema from papilledema. The structured investigation of papilledema is now routine and research into the management of CSF disorders is finding new avenues for targeted treatments. ESSENTIAL POINTS Confirmation of papilledema typically requires a thorough ocular examination including visual field testing. Ocular imaging such as color fundus photographs and optical coherence tomography are useful. Joint care between ophthalmologists and neurologists is key to treating the underlying condition and managing any visual dysfunction and headache.
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Karakaş B, Sarıtaş AŞ, Ak AK. Evaluation of predictive factors of idiopathic intracranial hypertension in different clinics. Clin Neurol Neurosurg 2025; 249:108738. [PMID: 39854877 DOI: 10.1016/j.clineuro.2025.108738] [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: 10/22/2024] [Revised: 12/28/2024] [Accepted: 01/10/2025] [Indexed: 01/27/2025]
Abstract
PURPOSE Idiopathic Intracranial Hypertension (IIH) is marked by elevated intracranial pressure without an identifiable cause. This study aimed to compare predictive factors between two IIH groups: those experiencing remission (single attack) and those developing migrainous headaches, in order to identify factors influencing the disease's progression. METHODS This retrospective study was conducted after obtaining ethics committee approval. It involved 118 patients diagnosed with IIH who were followed up in the Neuro-ophthalmology outpatient clinic between 2011 and 2023. Data on demographics, clinical history, comorbidities, habits, neurologic and neuro-ophthalmologic findings, cranial imaging features, lumbar puncture opening pressure, and current medical treatments were collected to assess the clinical course and predictive factors. The analyzed parameters were compared between two subgroups: one experiencing remission (single attack) and the other developing migrainous headaches. Statistically significant parameters were subjected to univariate and multivariate analysis. RESULTS Of the 118 patients, 87 (73.7 %) were in the single-attack group, while 31 (26.3 %) were in the migrainous headache group. Key predictive factors identified between the groups were the presence of allodynia (p < 0.001), neck and back pain (p = 0.042), BMI > 27.8 kg/m² (p = 0.002), and a history of migraine (p < 0.001). CONCLUSIONS Allodynia, neck and back pain, a history of migraine, and a BMI > 27.8 kg/m² were found to be predictive factors for the development of migrainous headaches in IIH patients. This is the first study in the literature to compare these two patient groups and identify the factors that contribute to the development of migrainous headaches.
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Affiliation(s)
- Burak Karakaş
- Doctor of Neurology, Celal Bayar University, Department of Neurology, Manisa 45000, Turkey.
| | - Ayşegül Şeyma Sarıtaş
- Doctor of Neurology, Celal Bayar University, Department of Neurology, Manisa 45000, Turkey.
| | - Ayşın Kısabay Ak
- Doctor of Neurology, Celal Bayar University, Department of Neurology, Manisa 45000, Turkey.
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Arkoudis NA, Davoutis E, Siderakis M, Papagiannopoulou G, Gouliopoulos N, Tsetsou I, Efthymiou E, Moschovaki-Zeiger O, Filippiadis D, Velonakis G. Idiopathic intracranial hypertension: Imaging and clinical fundamentals. World J Radiol 2024; 16:722-748. [PMID: 39801664 PMCID: PMC11718525 DOI: 10.4329/wjr.v16.i12.722] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2024] [Revised: 11/15/2024] [Accepted: 12/11/2024] [Indexed: 12/27/2024] Open
Abstract
Neuroimaging is a paramount element for the diagnosis of idiopathic intracranial hypertension, a condition characterized by signs and symptoms of raised intracranial pressure without the identification of a mass or hydrocephalus being recognized. The primary purpose of this review is to deliver an overview of the spectrum and the specific role of the various imaging findings associated with the condition while providing imaging examples and educational concepts. Clinical perspectives and insights into the disease, including treatment options, will also be discussed.
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Affiliation(s)
- Nikolaos-Achilleas Arkoudis
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Efstathia Davoutis
- School of Medicine, National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Manos Siderakis
- Department of Radiology, Agios Savas Anticancer Hospital, Athens 11522, Greece
| | - Georgia Papagiannopoulou
- 2nd Department of Neurology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Nikolaos Gouliopoulos
- 2nd Department of Ophthalmology, “Attikon” University Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Ilianna Tsetsou
- Department of Imaging and Interventional Radiology, “Sotiria” General and Chest Diseases Hospital of Athens, Athens 11527, Greece
| | - Evgenia Efthymiou
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Ornella Moschovaki-Zeiger
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Dimitrios Filippiadis
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
| | - Georgios Velonakis
- Research Unit of Radiology and Medical Imaging, School of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
- 2nd Department of Radiology, Attikon University General Hospital, School of Medicine, National and Kapodistrian University of Athens, Chaidari 12462, Greece
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Zaic S, Krajnc N, Macher S, Michl M, Müller N, Mitsch C, Marik W, Novak K, Wöber C, Pemp B, Bsteh G. Therapeutic effect of a single lumbar puncture in idiopathic intracranial hypertension. J Headache Pain 2024; 25:145. [PMID: 39237912 PMCID: PMC11378469 DOI: 10.1186/s10194-024-01851-x] [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: 08/02/2024] [Accepted: 08/28/2024] [Indexed: 09/07/2024] Open
Abstract
BACKGROUND Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by increased intracranial pressure. Whilst lumbar puncture (LP) is necessary for the diagnosis of IIH, its therapeutic effect remains unclear. Our aim was to evaluate the therapeutic effect of a single LP in people with IIH (pwIIH). METHODS In this prospective observational study, we analysed short-term neurological and ophthalmological outcomes in pwIIH before, one (D1) and seven days (D7) after the LP. The primary outcome was the change in papilledema degree from baseline. Secondary outcomes included visual outcomes, morphological changes in optical coherence tomography (peripapillary retinal nerve fibre layer [pRNFL] thickness and ganglion cell layer [GCL] volume) and transbulbar sonography (arachnoid optic nerve sheath diameter [AONSD]), and headache outcomes (peak and median headache severity and burden related to headache). RESULTS We included 30 pwIIH (mean age 32.8 years [SD 8.4], 93.3% female, median cerebrospinal fluid [CSF] opening pressure 33.0 cmCSF [IQR 26.9-35.3], median body mass index (BMI) 34.8 kg/m2 [IQR 30.9-40.9]). The median papilledema grading at baseline was 2 (Friedman DI (1999) Pseudotumor cerebri. Neurosurg Clin N Am 10(4):609-621 viii); (Mollan SP, Aguiar M, Evison F, Frew E, Sinclair AJ (2019) The expanding burden of idiopathic intracranial hypertension. Eye Lond Engl 33(3):478-485); (Ab D, Gt L, Nj V, Sl G, Ml M, Nj N et al. (2007) Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol [Internet]. Apr [cited 2024 Jun 2];143(4). https://pubmed.ncbi.nlm.nih.gov/17386271/ ) and was significantly reduced at D7 (2 [1-2], p = 0.011). Median pRNFL thickness had decreased significantly at D7 (-9 μm [-62.5, -1.3], p = 0.035), with pRNFL thickness at baseline being associated with the pRNFL change (F(1,11) = 18.79, p = 0.001). Mean AONSD had decreased significantly at both D1 (-0.74 mm [0.14], p < 0.001) and D7 (-0.65 mm [0.17], p = 0.01), with AONSD at baseline being associated with the change in AONSD at both time points (D1: β= -0.89, 95% CI -1.37, -0.42, p = 0.002; D7: β= -0.85, 95% CI -1.42, -0.28, p = 0.007). Peak headache severity was slightly lower at D7 (-1/10 [-3, 0], p = 0.026), whereas median headache severity and headache burden remained unchanged. CONCLUSIONS This short-term follow-up study in pwIIH undergoing a single LP suggests a moderate effect on ophthalmological but not headache outcomes. The usefulness of LP as a therapeutic measure in IIH remains controversial and should likely be reserved for patients with limited treatment options, e.g., in pregnancy or intolerability to medication.
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Affiliation(s)
- Sina Zaic
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
| | - Nik Krajnc
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
| | - Stefan Macher
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
| | - Martin Michl
- Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
| | - Nina Müller
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
| | - Christoph Mitsch
- Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
| | - Wolfgang Marik
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
- Department of Neuroradiology, Medical University of Vienna, Vienna, Austria
| | - Klaus Novak
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
- Department of Neurosurgery, Medical University of Vienna, Vienna, Austria
| | - Christian Wöber
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria
| | - Berthold Pemp
- Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
| | - Gabriel Bsteh
- Department of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.
- Comprehensive Center for Clinical Neurosciences and Mental Health, Medical University of Vienna, Vienna, Austria.
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Prem Senthil M, Anand S, Chakraborty R, Bordon JE, Constable PA, Brown S, Al-Dasooqi D, Simon S. Exploring the utility of retinal optical coherence tomography as a biomarker for idiopathic intracranial hypertension: a systematic review. J Neurol 2024; 271:4769-4793. [PMID: 38856724 PMCID: PMC11319609 DOI: 10.1007/s00415-024-12481-3] [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: 04/08/2024] [Revised: 05/24/2024] [Accepted: 05/26/2024] [Indexed: 06/11/2024]
Abstract
This study aimed to examine the existing literature that investigated the effectiveness of optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) as a biomarker for idiopathic intracranial hypertension (IIH). Our search was conducted on January 17th, 2024, and included the databases, Medline, Scopus, Embase, Cochrane, Latin American and Caribbean Health Sciences Literature (LILACS), International Standard Randomized Controlled Trial Number (ISRCTN) registry, and the International Clinical Trials Registry Platform (ICTRP). Our final review included 84 articles. In 74 studies, OCT was utilized as the primary ocular imaging method, while OCT-A was employed in two studies including eight studies that utilized both modalities. Overall, the results indicated that IIH patients exhibited significant increases in retinal nerve fiber layer (RNFL) thickness, total retinal and macular thickness, optic nerve head volume, and height, optic disc diameter and area, rim area, and thickness compared to controls. A significant correlation was observed between cerebrospinal fluid (CSF) pressure and OCT parameters including RNFL thickness, total retinal thickness, macular thickness, optic nerve head volume, and optic nerve head height. Interventions aimed at lowering CSF pressure were associated with a substantial improvement in these parameters. Nevertheless, studies comparing peripapillary vessel density using OCT-A between IIH patients and controls yielded conflicting results. Our systematic review supports OCT as a powerful tool to accurately monitor retinal axonal and optic nerve head changes in patients with IIH. Future research is required to determine the utility of OCT-A in IIH.
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Affiliation(s)
- Mallika Prem Senthil
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia.
| | - Saumya Anand
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia
| | - Ranjay Chakraborty
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia
| | - Jose Estevez Bordon
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia
| | - Paul A Constable
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia
| | - Shannon Brown
- Central Library, Flinders University, Bedford Park, Adelaide, South Australia, Australia
| | - Dalia Al-Dasooqi
- College of Nursing and Health Sciences, Caring Futures Institute, Flinders University, Bedford Park, Adelaide, South Australia, 5042, Australia
| | - Simu Simon
- University of Adelaide, Adelaide, South Australia, Australia
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Zhou C, Zhou Y, Liu L, Jiang H, Wei H, Zhou C, Ji X. Progress and recognition of idiopathic intracranial hypertension: A narrative review. CNS Neurosci Ther 2024; 30:e14895. [PMID: 39097911 PMCID: PMC11298205 DOI: 10.1111/cns.14895] [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: 02/11/2024] [Revised: 06/03/2024] [Accepted: 07/19/2024] [Indexed: 08/06/2024] Open
Abstract
BACKGROUND Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. AIMS This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. METHODS Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. RESULTS The review highlights potential pathomechanisms and therapeutic advances in IIH. CONCLUSION IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging.
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Affiliation(s)
- Chenxia Zhou
- Department of NeurologyXuanwu Hospital, Capital Medical UniversityBeijingChina
- Neurology and Intracranial Hypertension and Cerebral Venous Disease CenterNational Health Commission of China, Xuanwu Hospital, Capital Medical UniversityBeijingChina
| | - Yifan Zhou
- Laboratory of Brain Disorders, Ministry of Science and Technology, Collaborative Innovation Center for Brain DisordersBeijing Institute of Brain Disorders, Beijing Advanced Innovation Center for Big Data‐based Precision Medicine, Capital Medical UniversityBeijingChina
| | - Lu Liu
- Department of NeurologyXuanwu Hospital, Capital Medical UniversityBeijingChina
- Neurology and Intracranial Hypertension and Cerebral Venous Disease CenterNational Health Commission of China, Xuanwu Hospital, Capital Medical UniversityBeijingChina
- Zhongguancun Xirui Institute of Precision Medicine for Heart and Brain TumorsBeijingChina
| | - Huimin Jiang
- Laboratory of Brain Disorders, Ministry of Science and Technology, Collaborative Innovation Center for Brain DisordersBeijing Institute of Brain Disorders, Beijing Advanced Innovation Center for Big Data‐based Precision Medicine, Capital Medical UniversityBeijingChina
| | - Huimin Wei
- Beijing Advanced Innovation Center for Big Data‐Based Precision MedicineSchool of Biological Science and Medical Engineering, Beihang UniversityBeijingChina
| | - Chen Zhou
- Neurology and Intracranial Hypertension and Cerebral Venous Disease CenterNational Health Commission of China, Xuanwu Hospital, Capital Medical UniversityBeijingChina
- Laboratory of Brain Disorders, Ministry of Science and Technology, Collaborative Innovation Center for Brain DisordersBeijing Institute of Brain Disorders, Beijing Advanced Innovation Center for Big Data‐based Precision Medicine, Capital Medical UniversityBeijingChina
| | - Xunming Ji
- Neurology and Intracranial Hypertension and Cerebral Venous Disease CenterNational Health Commission of China, Xuanwu Hospital, Capital Medical UniversityBeijingChina
- Laboratory of Brain Disorders, Ministry of Science and Technology, Collaborative Innovation Center for Brain DisordersBeijing Institute of Brain Disorders, Beijing Advanced Innovation Center for Big Data‐based Precision Medicine, Capital Medical UniversityBeijingChina
- Department of NeurosurgeryXuanwu Hospital, Capital Medical UniversityBeijingChina
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Cho KH, Baek SH, Kim SH, Kim BS, Sohn JH, Chu MK, Kang MK, Mo HJ, Lee SH, Park HK, Cho S, Oh SY, Seo JG, Lee W, Lee JY, Lee MJ, Cho SJ. Clinical profile and treatment outcomes of idiopathic intracranial hypertension: a multicenter study from Korea. J Headache Pain 2024; 25:106. [PMID: 38918698 PMCID: PMC11197278 DOI: 10.1186/s10194-024-01794-3] [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: 04/10/2024] [Accepted: 05/20/2024] [Indexed: 06/27/2024] Open
Abstract
BACKGROUND Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension. METHODS We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1-2 and 3-6 months of treatment. RESULTS Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3-6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients. CONCLUSION Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.
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Affiliation(s)
- Kyung-Hee Cho
- Department of Neurology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea
| | - Seol-Hee Baek
- Department of Neurology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea
| | - Sung-Hee Kim
- Department of Neurology, Ewha Womans University Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Republic of Korea
| | - Byung-Su Kim
- Department of Neurology, Ewha Womans University Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Republic of Korea
| | - Jong-Hee Sohn
- Department of Neurology, Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Min Kyung Chu
- Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Mi-Kyoung Kang
- Department of Neurology, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, 7 Keunjaebong-Gil, Hwaseong, 18450, Republic of Korea
| | - Hee Jung Mo
- Department of Neurology, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, 7 Keunjaebong-Gil, Hwaseong, 18450, Republic of Korea
| | - Sang-Hwa Lee
- Department of Neurology, Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Republic of Korea
| | - Hong-Kyun Park
- Department of Neurology, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea
| | - Soohyun Cho
- Department of Neurology, Uijeongbu Eulji Medical Center, Eulji University School of Medicine, Uijeongbu, Republic of Korea
| | - Sun-Young Oh
- Department of Neurology, Jeonbuk National University Hospital, Jeonbuk National University School of Medicine, Jeonju, Republic of Korea
| | - Jong-Geun Seo
- Department of Neurology, School of Medicine, Kyungpook National University, Daegu, Republic of Korea
| | - Wonwoo Lee
- Department of Neurology, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Republic of Korea
| | - Ju-Young Lee
- Department of Neurology, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Republic of Korea
| | - Mi Ji Lee
- Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, 101, Daehak-Ro Jongno-Gu, Seoul, 03080, Republic of Korea.
| | - Soo-Jin Cho
- Department of Neurology, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, 7 Keunjaebong-Gil, Hwaseong, 18450, Republic of Korea.
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10
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Yiangou A, Mollan SP, Sinclair AJ. Idiopathic intracranial hypertension: a step change in understanding the disease mechanisms. Nat Rev Neurol 2023; 19:769-785. [PMID: 37957260 DOI: 10.1038/s41582-023-00893-0] [Citation(s) in RCA: 22] [Impact Index Per Article: 11.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/11/2023] [Indexed: 11/15/2023]
Abstract
The understanding of idiopathic intracranial hypertension (IIH) has evolved over the past few years. Previously, IIH was considered a disease exclusively affecting the neuro-ophthalmic axis, characterized by raised intracranial pressure, headache and papilloedema, and resulting in the risk of severe and permanent visual loss and life-changing disabling headaches. Recent advances have begun to redefine IIH as a probable metabolic disease involving a range of systemic manifestations. More than 95% of individuals affected by the disease are women of reproductive age with obesity. The incidence is rapidly rising and parallels the escalating worldwide obesity rates. Contemporary insights identify associations with insulin resistance, type 2 diabetes and a twofold increased risk of cardiovascular disease in excess of that driven by obesity alone. Adipose distribution in people with IIH, like that in other metabolic diseases, is preferentially centripetal and is associated with changes in intracranial pressure. Evidence now demonstrates adipose tissue dysfunction in people with IIH, involving transcriptional and metabolic priming for lipogenesis and weight gain. Hormonal perturbations are also observed, including a unique phenotype of androgen excess that promotes cerebrospinal fluid secretion. Knowledge of these additional disease features is driving research into novel therapeutic targets and altering the approach to multidisciplinary care.
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Affiliation(s)
- Andreas Yiangou
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
- Department of Neurology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Susan P Mollan
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
- Birmingham Neuro-Ophthalmology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Alexandra J Sinclair
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK.
- Department of Neurology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.
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11
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Mollan SP, Fraser CL, Digre KB, Diener HC, Lipton RB, Juhler M, Miller NR, Pozo-Rosich P, Togha M, Brock K, Dinkin MJ, Chan CKM, Tassorelli C, Sinclair AJ, Terwindt GM, Jensen RH. Guidelines of the International Headache Society for Controlled Clinical Trials in Idiopathic Intracranial Hypertension. Cephalalgia 2023; 43:3331024231197118. [PMID: 37661711 DOI: 10.1177/03331024231197118] [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] [Indexed: 09/05/2023]
Abstract
The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society Guidelines for Controlled Clinical Trials in Idiopathic Intracranial Hypertension aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension.
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Affiliation(s)
- Susan P Mollan
- Neuro-ophthalmology, Queen Elizabeth Hospital, University Hospital Birmingham NHS Foundation Trust, Birmingham, Birmingham, UK
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Clare L Fraser
- Save Sight Institute, University of Sydney, Sydney, NSW, Australia
| | - Kathleen B Digre
- Neuro-ophthalmology Division, John A. Moran Eye Center, University of Utah, Salt Lake City, USA
| | - Hans-Christoph Diener
- Institut für Medizinische Informatik, Biometrie undEpidemiologie (IMIBE), Medizinische Fakultät der Universität Duisburg-Essen, Essen, Germany
| | - Richard B Lipton
- The Saul R. Korey Department of Neurology, and the Montefiore Headache Center, Albert Einstein College of Medicine, Bronx, New York, USA
| | - Marianne Juhler
- Department of Neurosurgery, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark
- Institute of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
| | - Neil R Miller
- Wilmer Eye Institute and Departments of Ophthalmology, Neurology, and Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | - Patricia Pozo-Rosich
- Headache Unit, Neurology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain
- Headache and Neurological Pain Research Group, Vall d'Hebron Research Institute, Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain
| | - Mansoureh Togha
- Headache Department, Iranian Centre of Neurological Research, Neuroscience Institute, Tehran University of Medical Sciences, Tehran, Iran
- Headache Department, Neurology Ward, Sina Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
| | - Kristian Brock
- Cancer Research Clinical Trials Unit, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Marc J Dinkin
- Department of Ophthalmology, Weill Cornell Medical College, NY Presbyterian Hospital, New York, New York, USA
- Department of Neurology, Weill Cornell Medical College, NY Presbyterian Hospital, New York, New York, USA
| | - Carmen K M Chan
- Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong SAR, China
- Hong Kong Eye Hospital, Hong Kong SAR, China
| | - Cristina Tassorelli
- Unit of Translational Neurovascular Research, IRCCS Mondino Foundation, 27100, Pavia, Italy
- Department of Brain and Behavioural Sciences, University of Pavia, 27100, Pavia, Italy
| | - Alex J Sinclair
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
- Department of Neurology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
| | - Gisela M Terwindt
- Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands
| | - Rigmor H Jensen
- Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark
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12
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Hagen SM, Wibroe EA, Korsbæk JJ, Andersen MS, Nielsen AB, Nortvig MJ, Beier D, Poulsen FR, Jensen RH, Hamann S. Retinal vessel dynamics analysis as a surrogate marker for raised intracranial pressure in patients with suspected idiopathic intracranial hypertension. Cephalalgia 2023; 43:3331024221147494. [PMID: 36786365 DOI: 10.1177/03331024221147494] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/15/2023]
Abstract
INTRODUCTION Retinal vessel dynamics analysis has proven to be a viable, non-invasive surrogate marker for increased intracranial pressure. We aimed to test this method in patients with suspected idiopathic intracranial hypertension. METHODS Patients with suspected idiopathic intracranial hypertension were prospectively enrolled for hand-held fundus-videography during diagnostic lumbar puncture. After extracting optic disc images, peripapillary arteriole-to-venule-ratios were measured using machine-learning algorithms with manual identification control. A general linear model was applied to arteriole-to-venule-ratios and corresponding lumbar opening pressures to estimate cerebrospinal fluid pressure. RESULTS Twenty-five patients were included with a significant difference in arteriole-to-venule-ratio between patients with (n = 17) and without (n = 8) idiopathic intracranial hypertension (0.78 ± 0.10 vs 0.90 ± 0.08, p = 0.006). Arteriole-to-venule-ratio correlated inversely with lumbar opening pressure (slope regression estimate -0.0043 (95% CI -0.0073 to -0.0023), p = 0.002) and the association was stronger when lumbar opening pressure exceeded 15 mm Hg (20 cm H2O) (slope regression estimate -0.0080 (95% CI -0.0123 to -0.0039), p < 0.001). Estimated cerebrospinal fluid pressure predicted increased lumbar opening pressure >20 mm Hg (27 cm H2O) with 78% sensitivity and 92% specificity (AUC 0.81, p = 0.02). A stand-alone arteriole-to-venule-ratio measurement predicting lumbar opening pressure >20 mm Hg (27 cm H2O) was inferior with a 48% sensitivity and 92% specificity (AUC 0.73, p = 0.002). CONCLUSION Retinal vessel dynamics analysis with the described model for estimating cerebrospinal fluid pressure is a promising non-invasive method with a high sensitivity and specificity for detecting elevated intracranial pressure at follow-up assessments of patients with confirmed idiopathic intracranial hypertension if initial lumbar opening pressure and arteriole-to-venule-ratio data are available.
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Affiliation(s)
- Snorre Malm Hagen
- Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark
| | | | - Johanne Juhl Korsbæk
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Denmark
| | - Mikkel Schou Andersen
- Department of Neurosurgery, Odense University Hospital, Denmark.,Department of Clinical Research and BRIDGE (Brain Research Interdisciplinary Guided Excellence), University of Southern Denmark, Odense, Denmark
| | - Asger Bjørnær Nielsen
- Department of Neurosurgery, Odense University Hospital, Denmark.,Department of Clinical Research and BRIDGE (Brain Research Interdisciplinary Guided Excellence), University of Southern Denmark, Odense, Denmark
| | - Mathias Just Nortvig
- Department of Neurosurgery, Odense University Hospital, Denmark.,Department of Clinical Research and BRIDGE (Brain Research Interdisciplinary Guided Excellence), University of Southern Denmark, Odense, Denmark
| | - Dagmar Beier
- Department of Clinical Research and BRIDGE (Brain Research Interdisciplinary Guided Excellence), University of Southern Denmark, Odense, Denmark.,Department of Neurology, Odense University Hospital, Denmark
| | - Frantz Rom Poulsen
- Department of Neurosurgery, Odense University Hospital, Denmark.,Department of Clinical Research and BRIDGE (Brain Research Interdisciplinary Guided Excellence), University of Southern Denmark, Odense, Denmark
| | - Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Denmark
| | - Steffen Hamann
- Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Denmark
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13
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Mollan SP, Sinclair AJ, Tsermoulas G. Letter: Cerebrospinal Fluid Shunting for Idiopathic Intracranial Hypertension: A Systematic Review, Meta-Analysis, and Implications for a Modern Management Protocol. Neurosurgery 2023; 92:e59-e60. [PMID: 36700758 DOI: 10.1227/neu.0000000000002316] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2022] [Accepted: 10/25/2022] [Indexed: 01/27/2023] Open
Affiliation(s)
- Susan P Mollan
- Translational Brain Science, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston, UK
- Birmingham Neuro-Ophthalmology, University Hospitals Birmingham NHS Foundation Trust, UK
| | - Alexandra J Sinclair
- Translational Brain Science, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston, UK
- Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, UK
| | - Georgios Tsermoulas
- Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston, UK
- Department of Neurosurgery, University Hospitals Birmingham NHS Foundation Trust, UK
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14
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Lyons HS, Ramalingam S, Mitchell JL, Yiangou A, Thaller M, Sinclair AJ, Mollan SP. Multiple lumbar punctures aiming to relieve headache results in iatrogenic spinal hematoma: a case report. J Med Case Rep 2022; 16:464. [PMID: 36514112 DOI: 10.1186/s13256-022-03687-y] [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: 04/27/2022] [Accepted: 11/10/2022] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Multiple lumbar punctures have historically been a strategy to relieve headaches associated with idiopathic intracranial hypertension despite limited clinical evidence of long-term efficacy. Lumbar puncture is typically a straightforward procedure with minimal complications reported, however, serious complications can occur. Lumbar-puncture-related spinal hematomas are rare but can lead to irreversible paralysis. CASE PRESENTATION We report a case of a 28-year-old Caucasian woman who was treated with multiple lumbar punctures to manage headache, thought to be attributed to idiopathic intracranial hypertension. The patient developed a lumbosacral epidural hematoma following a lumbar puncture, which led to incomplete cauda equina syndrome. Multiple lumbar punctures had been the long-term management for the patient's chronic headaches associated with her diagnosis of idiopathic intracranial hypertension. She had no risks of an underlying coagulopathy. Following a lumbar puncture, she re-presented with lower back pain and bilateral paresthesia. Over the subsequent 48 hours, this progressed to urinary incontinence and saddle paresthesia. Imaging revealed an epidural hematoma, which was conservatively managed. She continued to report saddle paresthesia and urinary incontinence 7 months following the lumbar puncture. Between 1974 to 2022, our literature search found 41 case reports detailing lumbar-puncture-related spinal hematomas. It is an established but rare complication of lumbar puncture and there are limited studies looking at the incidence of its occurrence. Whilst coagulopathy has been found to be a risk factor, it is unclear if the gauge of the needle is relevant. Case evidence suggests there may be no significant difference in outcomes between surgical and conservative management of spinal hematomas. This case highlights that lumbar punctures can be invasive, with potentially serious complications. A lumbar puncture should therefore only be performed when clinically justified. CONCLUSIONS This case highlights a rare complication of lumbar puncture and emphasizes the importance of a risk-benefit discussion for each procedure. Spinal hematoma following lumbar puncture is a rare complication but with potentially devastating consequences. Within the setting of idiopathic intracranial hypertension, the evidence base for the long-term benefit of headache relief by repeat lumbar puncture is low.
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Affiliation(s)
- Hannah S Lyons
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK
| | - Satheesh Ramalingam
- Department of Interventional Radiology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK
| | - James L Mitchell
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, B15 2TH, UK
| | - Andreas Yiangou
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, B15 2TH, UK
| | - Mark Thaller
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, B15 2TH, UK
| | - Alexandra J Sinclair
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2WB, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, B15 2TH, UK
| | - Susan P Mollan
- Translational Brain Science, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, B15 2TT, UK. .,Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
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15
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Adderley NJ, Subramanian A, Perrins M, Nirantharakumar K, Mollan SP, Sinclair AJ. Headache, Opiate Use, and Prescribing Trends in Women With Idiopathic Intracranial Hypertension: A Population-Based Matched Cohort Study. Neurology 2022; 99:e1968-e1978. [PMID: 35985824 PMCID: PMC9651462 DOI: 10.1212/wnl.0000000000201064] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2022] [Accepted: 06/21/2022] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND AND OBJECTIVES Physician prescribing habits for opiates and headache therapies have not been previously evaluated in a large, matched cohort study in idiopathic intracranial hypertension (IIH). Our objective was to evaluate opiate and headache medication prescribing habits in women with IIH compared with matched women with migraine and population controls. We also investigated the occurrence of new onset headache in IIH compared with population controls. METHODS We performed a population-based matched, retrospective cohort study to explore headache outcomes. Cross-sectional analyses were used to describe medication prescribing patterns. We used data from IQVIA Medical Research Data, an anonymized, nationally representative primary care electronic medical record database in the United Kingdom, from January 1, 1995, to September 25, 2019. Women aged 16 years and older were eligible for inclusion. Women with IIH (exposure) were matched by age and body mass index with up to 10 control women without IIH but with migraine (migraine controls), and without IIH or migraine (population controls). RESULTS A total of 3,411 women with IIH, 13,966 migraine controls, and 33,495 population controls were included. The adjusted hazard ratio for new onset headache in IIH compared with population controls was 3.09 (95% CI 2.78-3.43). In the first year after diagnosis, 58% of women with IIH were prescribed acetazolamide and 20% topiramate. In total, 20% of women with IIH were prescribed opiates within the first year of their diagnosis, reducing to 17% after 6 years, compared with 8% and 11% among those with migraine, respectively. Twice as many women with IIH were prescribed opiates compared with migraine controls, and 3 times as many women with IIH were prescribed opiates compared with population controls. Women with IIH were also prescribed more headache preventative medications compared with migraine controls. DISCUSSION Women with IIH were more likely to be prescribed opiate and simple analgesics compared with both migraine and population controls. Women with IIH trialed more preventative medications over their disease course suggesting that headaches in IIH may be more refractory to treatment.
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Affiliation(s)
- Nicola Jaime Adderley
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom
| | - Anuradhaa Subramanian
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom
| | - Mary Perrins
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom
| | - Krishnarajah Nirantharakumar
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom
| | - Susan P Mollan
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom
| | - Alexandra Jean Sinclair
- From the Institute of Applied Health Research (N.J.A., A.S., M.P., K.N.), University of Birmingham; Centre for Endocrinology, Diabetes, and Metabolism (K.N.), Birmingham Health Partners; Health Data Research UK (K.N.), Birmingham; Birmingham Neuro-Ophthalmology (S.P.M.), Queen Elizabeth Hospital; Metabolic Neurology (A.J.S.), Institute of Metabolism and Systems Research, University of Birmingham; and Department of Neurology (A.J.S.), University Hospitals Birmingham, Queen Elizabeth Hospital, United Kingdom.
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16
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Korsbæk JJ, Beier D, Hagen SM, Molander LD, Jensen RH. Psychiatric Comorbidities in Patients With Idiopathic Intracranial Hypertension: A Prospective Cohort Study. Neurology 2022; 99:e199-e208. [PMID: 35473759 DOI: 10.1212/wnl.0000000000200548] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2021] [Accepted: 03/02/2022] [Indexed: 11/15/2022] Open
Abstract
BACKGROUND AND OBJECTIVES Psychiatric disease is a frequent comorbidity in idiopathic intracranial hypertension (IIH) and has been associated with a poor subjective outcome. No prospective studies have investigated psychiatric comorbidities in new-onset IIH. Our primary aim was to investigate disease severity in IIH depending on comorbid psychiatric disease. Important secondary outcomes were disease severity after 6 months and prevalence of psychiatric diseases in IIH. METHODS This prospective, observational cohort study consecutively included patients with clinically suspected, new-onset IIH from 2 headache centers. A standardized diagnostic workup was completed to confirm or exclude IIH according to the revised Friedman criteria: semi-structured interview, neurologic and neuro-ophthalmologic examination, lumbar puncture with opening pressure (OP), and neuroimaging. Exclusion criteria were known IIH, secondary intracranial hypertension, missing data, and pregnancy. Disease severity was evaluated based on papilledema, visual fields, OP, and headache in patients with IIH without psychiatric disease (IIH-P) compared with patients with IIH with psychiatric disease (IIH+P). A blinded neuro-ophthalmologist assessed visual outcomes. Age- and sex-matched prevalence rates of psychiatric diseases in the general population were found in national reports. RESULTS A total of 258 patients were screened; 69 were excluded. A total of 189 patients with clinically suspected IIH were then evaluated with the diagnostic workup and IIH was confirmed in 111 patients (58.7%). A total of 45% of patients with IIH had a psychiatric comorbidity. Visual fields were worse in patients with IIH+P at baseline (-8.0 vs -6.0 dB; p = 0.04) and after 6 months (-5.5 vs -4.0 dB; p < 0.01) compared with IIH-P. Highly prevalent psychiatric disorders were major depressive disorder (24.3%; n = 27), anxiety or stress-related disorders (24.3%; n = 27), and emotionally unstable personality disorder (6.3%; n = 7). Substance abuse (1.8%), schizophrenia (0.9%), and bipolar disorder (0.9%) were rare. In the general population, prevalence estimates of major depressive disorder and emotionally unstable personality disorder were 1.8%-3.3% and 2%, respectively. DISCUSSION Visual fields were significantly worse at baseline and 6 months in IIH+P compared with IIH-P. Psychiatric comorbidities, particularly depression and emotionally unstable personality disorder, were highly prevalent in IIH. Increased awareness of psychiatric disease as a marker for worse visual status may improve patient care.
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Affiliation(s)
- Johanne Juhl Korsbæk
- From the Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup (J.J.K., R.H.J.), and Department of Ophthalmology, Rigshospitalet (S.M.H.), University of Copenhagen; Departments of Neurology (D.B.) and Ophthalmology (L.D.M.) and OPEN, Odense Patient Data Explorative Network (D.B.), Odense University Hospital; and Department of Clinical Research (D.B.), University of Southern Denmark
| | - Dagmar Beier
- From the Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup (J.J.K., R.H.J.), and Department of Ophthalmology, Rigshospitalet (S.M.H.), University of Copenhagen; Departments of Neurology (D.B.) and Ophthalmology (L.D.M.) and OPEN, Odense Patient Data Explorative Network (D.B.), Odense University Hospital; and Department of Clinical Research (D.B.), University of Southern Denmark
| | - Snorre Malm Hagen
- From the Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup (J.J.K., R.H.J.), and Department of Ophthalmology, Rigshospitalet (S.M.H.), University of Copenhagen; Departments of Neurology (D.B.) and Ophthalmology (L.D.M.) and OPEN, Odense Patient Data Explorative Network (D.B.), Odense University Hospital; and Department of Clinical Research (D.B.), University of Southern Denmark
| | - Laleh Dehghani Molander
- From the Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup (J.J.K., R.H.J.), and Department of Ophthalmology, Rigshospitalet (S.M.H.), University of Copenhagen; Departments of Neurology (D.B.) and Ophthalmology (L.D.M.) and OPEN, Odense Patient Data Explorative Network (D.B.), Odense University Hospital; and Department of Clinical Research (D.B.), University of Southern Denmark
| | - Rigmor Højland Jensen
- From the Department of Neurology, Danish Headache Center, Rigshospitalet-Glostrup (J.J.K., R.H.J.), and Department of Ophthalmology, Rigshospitalet (S.M.H.), University of Copenhagen; Departments of Neurology (D.B.) and Ophthalmology (L.D.M.) and OPEN, Odense Patient Data Explorative Network (D.B.), Odense University Hospital; and Department of Clinical Research (D.B.), University of Southern Denmark.
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17
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Elmaaty AAA, Zarad CA, Belal TI, Elserafy TS. Diagnostic value of brain MR imaging and its correlation with clinical presentation and cognitive functions in idiopathic intracranial hypertension patients. THE EGYPTIAN JOURNAL OF NEUROLOGY, PSYCHIATRY AND NEUROSURGERY 2021. [DOI: 10.1186/s41983-021-00338-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Idiopathic intracranial hypertension (IIH) is a neurological disorder of unknown etiology and ambiguous pathophysiology due to cerebrospinal fluid dysregulation. This study is designed to evaluate the role of brain magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) in diagnosis of IIH, to clarify the nature and extent of cognitive deficits, and to detect if there is a correlation between radiology, clinical findings, and cognitive dysfunctions in those patients.
Results
The study included 34 patients and 34 age-, sex-, body mass index (BMI)-, and education-matched healthy control subjects. MR brain imaging and Montreal cognitive assessment (MoCA) test were used for both groups. MRI and MRV sensitivity for IIH diagnosis were 85.2% and 85.3% with 100% and 94.1% specificity respectively. 44.1% had cognitive impairment, memory was the most affected domain, followed by attention, abstraction, and orientation with statistically significantly lower total MoCA score (p < 0.005). Domain comparisons reveal a statistically significantly lower memory/delayed recall (p < 0.001) and abstract scores (p < 0.007) in IIH cases versus control subjects. In comparing patients with cognitive impairment (CI) versus those without CI, there were statistically significantly higher CI in low education level, presence of diplopia, hormonal contraceptive use, abnormal MRI brain, papilledema grades, BMI, and opening pressure.
Conclusions
Presence of more than or equal 3 MR imaging findings, bilateral transverse sinus stenosis, and less than or equal 4 combined conduit score increase the specificity and sensitivity of MRI and MRV for IIH diagnosis. IIH had detrimental effect on different cognitive domains especially when patient have low education level, diplopia, papilledema ≥ grade III, high OP ≥ 61.5 cm H2O, and BMI ≥ 34 Kg/m2 with abnormal MRI and MRV findings.
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18
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Mollan SP, Wakerley BR, Alimajstorovic Z, Mitchell J, Ottridge R, Yiangou A, Thaller M, Gupta A, Grech O, Lavery G, Brock K, Sinclair AJ. Intracranial pressure directly predicts headache morbidity in idiopathic intracranial hypertension. J Headache Pain 2021; 22:118. [PMID: 34620087 PMCID: PMC8499560 DOI: 10.1186/s10194-021-01321-8] [Citation(s) in RCA: 34] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2021] [Accepted: 09/01/2021] [Indexed: 01/03/2023] Open
Abstract
Objective Headache is the predominant disabler in idiopathic intracranial hypertension (IIH). The aim was to characterise headache and investigate the association with intracranial pressure. Methods IIH:WT was a randomised controlled parallel group multicentre trial in the United Kingdom investigating weight management methods in IIH. Participants with active IIH (evidenced by papilloedema) and a body mass index (BMI) ≥35 kg/m2 were recruited. At baseline, 12 months and 24 months headache characteristics and quality of life outcome measures were collected and lumbar puncture measurements were performed. Results Sixty-six women with active IIH were included with a mean age of 32.0 years (SD ± 7.8), and mean body mass index of 43.9 ± 7.0 kg/m2. The headache phenotype was migraine-like in 90%. Headache severity correlated with ICP at baseline (r = 0.285; p = 0.024); change in headache severity and monthly headache days correlated with change in ICP at 12 months (r = 0.454, p = 0.001 and r = 0.419, p = 0.002 respectively). Cutaneous allodynia was significantly correlated with ICP at 12 months. (r = 0.479, p < 0.001). Boot strap analysis noted a positive association between ICP at 12 and 24 months and enabled prediction of both change in headache severity and monthly headache days. ICP was associated with significant improvements in quality of life (SF-36). Conclusions We demonstrate a positive relationship between ICP and headache and cutaneous allodynia, which has not been previously reported in IIH. Those with the greatest reduction in ICP over 12 months had the greatest reduction in headache frequency and severity; this was associated with improvement of quality of life measures. Trial registration This work provides Class IIa evidence of the association of raised intracranial pressure and headache. ClinicalTrials.gov number, NCT02124486. Supplementary Information The online version contains supplementary material available at 10.1186/s10194-021-01321-8.
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Affiliation(s)
- S P Mollan
- Birmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham, Birmingham, UK
| | - B R Wakerley
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham, Birmingham, UK
| | - Z Alimajstorovic
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK
| | - J Mitchell
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham, Birmingham, UK
| | - R Ottridge
- University of Birmingham College of Medical and Dental Sciences, Birmingham, UK
| | - A Yiangou
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham, Birmingham, UK
| | - M Thaller
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham, Birmingham, UK
| | - A Gupta
- University of Birmingham College of Medical and Dental Sciences, Birmingham, UK
| | - O Grech
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK
| | - G Lavery
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK
| | - K Brock
- Birmingham Clinical Trials Unit, University of Birmingham, Birmingham, UK
| | - A J Sinclair
- University of Birmingham, Metabolic Neurology, Institute of Metabolism and Systems Research, Birmingham, UK. .,Department of Neurology, University Hospitals Birmingham, Birmingham, UK.
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19
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Bandatmakur M, Bench C, Ngwa N, Osman H, Dave P, Farooqi A, Sivaswamy L. Factors Predisposing to Post Dural Puncture Headache in Children. J Child Neurol 2021; 36:831-840. [PMID: 33882752 DOI: 10.1177/08830738211007699] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
BACKGROUND Knowledge of the factors that predispose to postdural puncture headache in children may help reduce the occurrence of this complication. MATERIALS AND METHODS A retrospective cohort study of children who presented to the study institution between 2010 and 2018 was conducted. Children were divided into 2 groups: those who experienced postdural puncture headache and those who did not. The 2 groups were compared with respect to certain demographic, technical, and personnel-related factors. Only children who had opening pressure documented during the procedure were included in the core study group. RESULTS In univariate analysis, children aged ≥10 years, female gender, children with higher body mass index, standard blinded lumbar puncture procedure, use of sedation, higher opening pressure, and presence of pseudotumor cerebri increased the probability of postdural puncture headache. In multivariable logistic regression analysis, presence of pseudotumor cerebri was the only factor that attained statistical significance when the opening pressure was measured and documented. CONCLUSIONS The risk factors for postdural puncture headache in a pediatric cohort varied from risk factors that are classically implicated in adults.
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Affiliation(s)
- Madhav Bandatmakur
- Department of Pediatric Neurology, 2969Children's Hospital of MI, Detroit, MI, USA
| | - Carter Bench
- 12267Wayne State University School of Medicine, Detroit, MI, USA
| | - Ngum Ngwa
- Department of Pediatrics, 2969Children's Hospital of MI, Detroit, MI, USA
| | - Heba Osman
- Department of Anesthesia, Detroit Medical Center, Detroit, MI, USA
| | - Pooja Dave
- Department of Pediatrics, 2969Children's Hospital of MI, Detroit, MI, USA
| | - Ahmad Farooqi
- Department of Biostatistics, Children's Hospital of MI, Detroit, MI, USA
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20
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Jensen RH, Vukovic-Cvetkovic V, Korsbaek JJ, Wegener M, Hamann S, Beier D. Awareness, Diagnosis and Management of Idiopathic Intracranial Hypertension. Life (Basel) 2021; 11:718. [PMID: 34357090 PMCID: PMC8303648 DOI: 10.3390/life11070718] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Revised: 07/06/2021] [Accepted: 07/14/2021] [Indexed: 11/16/2022] Open
Abstract
The diagnosis and management of idiopathic intracranial hypertension (IIH) can be difficult and multiple medical subspecialities are often involved. Several national and international guidelines regarding the investigations and management of IIH have been published in recent years but still there is no consensus about the optimal organization of IIH-care. The objective of this review was to propose and describe a referral pathway and an organization scheme for diagnosis and management of IIH. An extensive search of existing literature was conducted and summarized. In total, 237 IIH-articles were identified and hereof 43 included. The clinical practice in our specialized IIH-clinic is characterized and described. We conclude that an educational campaign involving medical care providers and patients with chronic headaches is necessary. A detailed organizational proposal for a referral pathway and management of IIH patients based on the literature search and our clinical experience from a highly specialized IIH outpatient clinic is suggested and discussed.
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Affiliation(s)
- Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet, University of Copenhagen, 1165 København, Denmark; (V.V.-C.); (J.J.K.)
| | - Vlasta Vukovic-Cvetkovic
- Danish Headache Center, Department of Neurology, Rigshospitalet, University of Copenhagen, 1165 København, Denmark; (V.V.-C.); (J.J.K.)
| | - Johanne Juhl Korsbaek
- Danish Headache Center, Department of Neurology, Rigshospitalet, University of Copenhagen, 1165 København, Denmark; (V.V.-C.); (J.J.K.)
| | - Marianne Wegener
- Department of Ophthalmology, Rigshospitalet, University of Copenhagen, 1165 København, Denmark; (M.W.); (S.H.)
| | - Steffen Hamann
- Department of Ophthalmology, Rigshospitalet, University of Copenhagen, 1165 København, Denmark; (M.W.); (S.H.)
| | - Dagmar Beier
- Department of Neurology, Odense University Hospital, 5000 Odense, Denmark;
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21
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Mollan SP, Grech O, Sinclair AJ. Headache attributed to idiopathic intracranial hypertension and persistent post-idiopathic intracranial hypertension headache: A narrative review. Headache 2021; 61:808-816. [PMID: 34106464 DOI: 10.1111/head.14125] [Citation(s) in RCA: 47] [Impact Index Per Article: 11.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Accepted: 04/12/2021] [Indexed: 01/01/2023]
Abstract
OBJECTIVE Headache is a near-universal sequela of idiopathic intracranial hypertension (IIH). The aim of this paper is to report current knowledge of headache in IIH and to identify therapeutic options. BACKGROUND Disability in IIH is predominantly driven by headache; thus, headache management is an urgent and unmet clinical need. At present, there is currently no scientific evidence for the directed use of abortive or preventative headache therapy. METHODS A detailed search of the scientific literature and narrative review was performed. RESULTS Headache in IIH is driven by raised intracranial pressure (ICP) and reduction of ICP has been reported in some studies to reduce headache. Despite resolution of papilledema and normalization of raised ICP, a majority suffer persistent post-IIH headache. The lack of evidence-based management approaches leaves many untreated. Where clinicians attempt to manage IIH headache, they use off-label therapies to target the prevailing headache phenotype. A recent prospective open-label study demonstrated the effective use of a calcitonin gene-related peptide monoclonal antibody therapy in IIH for persistent post-IIH headache. CONCLUSIONS There is overwhelming evidence of the headache burden in IIH. Studies are required to investigate the biological foundations of headache related to ICP and to develop treatments specifically directed to manage headache in IIH.
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Affiliation(s)
- Susan P Mollan
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK
| | - Olivia Grech
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - Alexandra J Sinclair
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK.,Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, UK
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22
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Mollan SP, Chong YJ, Grech O, Sinclair AJ, Wakerley BR. Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema. Life (Basel) 2021; 11:472. [PMID: 34073844 PMCID: PMC8225003 DOI: 10.3390/life11060472] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2021] [Revised: 05/16/2021] [Accepted: 05/18/2021] [Indexed: 02/07/2023] Open
Abstract
The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients with IIHWOP are phenotypically similar to those with idiopathic intracranial hypertension (IIH), it remains uncertain as to whether IIHWOP is nosologically distinct from IIH. The incidence, prevalence, and the degree of association with the world-wide obesity epidemic is unknown. Establishing a diagnosis of IIHWOP can be challenging, as often lumbar puncture is not routinely part of the work-up for refractory headaches. There are published diagnostic criteria for IIHWOP; however, some report uncertainty regarding a pathologically acceptable cut off for a raised lumbar puncture opening pressure, which is a key criterion. The literature provides little information to help guide clinicians in managing patients with IIHWOP. Further research is therefore needed to better understand the mechanisms that drive the development of chronic daily headaches and a relationship to intracranial pressure; and indeed, whether such patients would benefit from therapies to lower intracranial pressure. The aim of this narrative review was to perform a detailed search of the scientific literature and provide a summary of historic and current opinion regarding IIHWOP.
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Affiliation(s)
- Susan P. Mollan
- Birmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK;
| | - Yu Jeat Chong
- Birmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK;
| | - Olivia Grech
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UK; (O.G.); (A.J.S.); (B.R.W.)
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK
| | - Alex J. Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UK; (O.G.); (A.J.S.); (B.R.W.)
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK
- Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK
| | - Benjamin R. Wakerley
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UK; (O.G.); (A.J.S.); (B.R.W.)
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK
- Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UK
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23
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Abstract
Objective To review and discuss the clinical presentation and treatment of
idiopathic intracranial hypertension. Discussion Visual alterations and headache are the two main symptoms of
idiopathic intracranial hypertension, although additional
features including cranial nerve palsies, cognitive deficits,
olfactory deficits and tinnitus are not uncommon. The headache
associated with idiopathic intracranial hypertension frequently
has a migrainous phenotype. The underlying cause of the disorder
has not yet been elucidated. Several hypotheses have been
postulated but none of them can explain the full clinical
picture. Therapeutic options remain limited, focusing mainly on
reduction in body weight and the reduction of CSF production
with carbonic anhydrase inhibitors. Conclusion The accurate diagnosis of idiopathic intracranial hypertension is
essential as visual deterioration due to papilledema may be
irreversible. Given its phenotypic similarity and frequent
overlap with chronic migraine it is essential to consider
idiopathic intracranial hypertension in the diagnostic workup of
chronic headache; in particular, when considering its increasing
prevalence. Understanding in detail the pathophysiological
mechanisms behind the associated headache would also allow study
of current and future therapeutic options in a structured
way.
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Affiliation(s)
- Naz Raoof
- Department of Paediatrics, Strabismus and Neuro-ophthalmology, Moorfields Eye Hospital, London, UK.,Department of Ophthalmology, Royal London Hospital, Barts Health NHS Trust, London, UK
| | - Jan Hoffmann
- Wolfson Centre for Age-Related Diseases, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK.,NIHR-Wellcome Trust King's Clinical Research Facility/SLaM Biomedical Research Centre, King's College Hospital, London, UK
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24
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Mollan SP, Grech O, Alimajstorovic Z, Wakerley BR, Sinclair AJ. New horizons for idiopathic intracranial hypertension: advances and challenges. Br Med Bull 2020; 136:118-126. [PMID: 33200788 DOI: 10.1093/bmb/ldaa034] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/28/2020] [Indexed: 02/05/2023]
Abstract
INTRODUCTION Idiopathic intracranial hypertension (IIH) is becoming a recognized condition due to the increasing incidence linked to a global obesity epidemic. SOURCES OF DATA All English papers on PubMed, Cochrane and Scholar between inception until 1 March 2020 were considered. AREAS OF AGREEMENT Studies suggest central adiposity has a pathogenic role. Recent weight gain is a risk factor and weight loss has a key role in management. AREAS OF CONTROVERSY Interpretation of abnormal lumbar puncture opening pressure is debated. There is an increasing recognition of obesity stigma and how this should be approached. GROWING POINTS Further evidence is required for the choice of surgical intervention for fulminant IIH. Education regarding IIH should be evidence based. AREAS TIMELY FOR DEVELOPING RESEARCH Novel research of the pathology of IIH is influencing development of therapies such as glucagon-like peptide-1 receptor agonists and targeting unique androgen signatures. The newly discovered cardiovascular risk requires further attention.
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Affiliation(s)
- Susan P Mollan
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK
| | - Olivia Grech
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK
| | - Zerin Alimajstorovic
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK
| | | | - Alexandra J Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham B15 2WB, UK
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25
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Yiangou A, Mitchell JL, Fisher C, Edwards J, Vijay V, Alimajstorovic Z, Grech O, Lavery GG, Mollan SP, Sinclair AJ. Erenumab for headaches in idiopathic intracranial hypertension: A prospective open-label evaluation. Headache 2020; 61:157-169. [PMID: 33316102 PMCID: PMC7898289 DOI: 10.1111/head.14026] [Citation(s) in RCA: 32] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2020] [Revised: 10/14/2020] [Accepted: 10/15/2020] [Indexed: 12/21/2022]
Abstract
Objective To determine the effectiveness of erenumab in treating headaches in idiopathic intracranial hypertension (IIH) in whom papilledema had resolved. Background Disability in IIH is predominantly driven by debilitating headaches with no evidence for the use of preventative therapies. Headache therapy in IIH is an urgent unmet need. Methods A prospective, open‐label study in the United Kingdom was conducted. Adult females with confirmed diagnosis of IIH now in ocular remission (papilledema resolved) with chronic headaches (≥15 days a month) and failure of ≥3 preventative medications received erenumab 4‐weekly (assessments were 3‐monthly). The primary end point was change in monthly moderate/severe headache days (MmsHD) from baseline (30‐day pretreatment period) compared to 12 months. Results Fifty‐five patients, mean (SD) age 35.3 (9) years and mean duration of headaches 10.4 (8.4) years with 3.7 (0.9) preventative treatment failures, were enrolled. Mean baseline MmsHD was 16.1 (4.7) and total monthly headache days (MHD) was (29) 2.3. MmsHD reduced substantially at 12 months by mean (SD) [95% CI] 10.8 (4.0) [9.5, 11.9], p < 0.001 and MHD reduced by 13.0 (9.5) [10.2, 15.7], p < 0.001. Crystal clear days (days without any head pain) increased by 13.1 (9.5) [9.6, 15.3], p < 0.001, headache severity (scale 0–10) fell by 1.3 (1.7) [0.9, 1.9], p < 0.001, and monthly analgesic days reduced by 4.3 (9.2) [1.6, 6.9], p = 0.002. All these measures had improved significantly by 3 months, with a consistent significant response to 12 months. Headache impact test‐6 score and quality of life Short Form‐36 Health Survey significantly improved at 12 months. Sensitivity analysis revealed similar results for patients with and without a prior migraine diagnosis (28/55 (52%) patients) or those with or without medication overuse (27/55 (48%) patients). Conclusions This study provides evidence for the effectiveness of erenumab to treat headaches in IIH patients with resolution of papilledema. It provides mechanistic insights suggesting that calcitonin gene‐related peptide is likely a modulator driving headache and a useful therapeutic target.
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Affiliation(s)
- Andreas Yiangou
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - James L Mitchell
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Claire Fisher
- Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Julie Edwards
- Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Vivek Vijay
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Zerin Alimajstorovic
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - Olivia Grech
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - Gareth G Lavery
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - Susan P Mollan
- Birmingham Neuro-Ophthalmology Unit, Ophthalmology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Alexandra J Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
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26
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A clinical and radiological study in patients with idiopathic intracranial hypertension. THE EGYPTIAN JOURNAL OF NEUROLOGY, PSYCHIATRY AND NEUROSURGERY 2020. [DOI: 10.1186/s41983-020-00189-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Idiopathic intracranial hypertension (IIH) is one of the chronic causes of elevated intracranial pressure with no evident cause in neuroimaging and normal CSF analysis. It primarily affects overweight women of childbearing age.
Aim of work
To describe the clinical picture of IIH, neuroimaging and response to treatment after 3 months follow up in a sample of Egyptian patients.
Patients and methods
This was a prospective study carried out on 25 patients presented at Alexandria University Hospital with symptomatology of IIH. All the patients were subjected to complete history taking, neurological examination, fundus examination, lumbar puncture, and MRI brain and MR cerebral venography. The patients were followed-up for 3 months for assessment of treatment response.
Results
Patients’ ages ranged from 1 0to 50 years, with a mean BMI 32.32 ± 5.28 kg m2. Headache was the presenting manifestation in 100% of the patients. About 60% of the sample were either misdiagnosed or had a delayed diagnosis. There was a significant positive correlation between CSF opening pressure (r = 0.504, p = 0.010), severity of headache (r = 0.472, p = 0.017) and grade of papilledema. Optic hydropes and empty sellaturcica were the most common MRI abnormalities occurring in 95.8% and 70.8%, respectively. Only 30% of the cases had normal MRV. Stenosis at genu junction and focal stenosis at transverse sinus occurred in 24% and 20% of the cases, respectively. Combined medical and serial lumbar puncture were the most effective line of therapy in the recruited patients.
Conclusion
Headache is the most common presentation of IIH, and its severity is positively correlated with papilledema grade. CSF pressure is also positively correlated with papilledema grade.
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27
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How and when to refer patients diagnosed with secondary headache and other craniofacial pain in the emergency department and primary care: Recommendations of the Spanish Society of Neurology's Headache Study Group. NEUROLOGÍA (ENGLISH EDITION) 2020. [DOI: 10.1016/j.nrleng.2017.08.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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28
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An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management. J Neurol 2020; 268:3249-3268. [PMID: 32462350 DOI: 10.1007/s00415-020-09943-9] [Citation(s) in RCA: 36] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2020] [Revised: 05/18/2020] [Accepted: 05/20/2020] [Indexed: 02/06/2023]
Abstract
Idiopathic intracranial hypertension is a neurological syndrome determined by a rise in intracranial pressure without a detectable cause. Course and prognosis may be changeable, requiring a multidisciplinary approach for its diagnosis and management. Although its precise pathogenesis is still unknown, many studies have been carried out to define the possible causal and associated factors, such as retinoids, steroid hormones, body mass index and recent weight gains, cytokines and adipokines levels. The clinical presentation can be variable including chronic headache, disturbance of vision, diplopia and tinnitus. Even if papilloedema is considered the most specific sign, it could not be observed in more than 5% of patients during the evaluation of the fundus oculi. Neuroradiological signs acquire greater importance in patients who do not present papilloedema and may suggest the diagnosis of idiopathic intracranial hypertension. Other assessments can be useful in the diagnostic process, such as optical coherence tomography, visual evoked potentials, ocular ultrasonography and fundus fluorescein angiography and autofluorescence. Nonetheless, cerebrospinal fluid pressure measurement is required to establish a definite diagnosis. Management may be different, since surgical procedures or lumbar punctures are often required when symptoms develop rapidly leading to a loss of visual function. Apart from these cases, patients can be treated with a pharmacological approach and low-calorie diet, but they also need to be monitored over time since relapses years later are not uncommon.
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29
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Griffin AS, Lu L, Peacock S, Gray L, Kranz PG, Amrhein TJ. CSF volume provocation maneuvers during lumbar puncture as a possible predictive tool for diagnosing spontaneous intracranial hypotension. Clin Neurol Neurosurg 2019; 186:105552. [DOI: 10.1016/j.clineuro.2019.105552] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2019] [Revised: 09/11/2019] [Accepted: 10/04/2019] [Indexed: 01/03/2023]
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30
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Abstract
Idiopathic intracranial hypertension (IIH) is a condition characterized by increased intracranial pressure without a detectable cause. The most common symptom of IIH is a headache, which occurs in almost all cases at the time of diagnosis with various headache phenotypes. In clinical practice, diagnosis of headache attributed to IIH is given referring to the International Headache Society (IHS) criteria of the International Classification of Headache Disorders. In the present publication, we consider how the IHS diagnostic criteria for headache attributed to intracranial idiopathic hypertension have evolved through the years.
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31
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Mitchell JL, Mollan SP, Vijay V, Sinclair AJ. Novel advances in monitoring and therapeutic approaches in idiopathic intracranial hypertension. Curr Opin Neurol 2019; 32:422-431. [PMID: 30865008 PMCID: PMC6522204 DOI: 10.1097/wco.0000000000000690] [Citation(s) in RCA: 27] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
PURPOSE OF REVIEW The current article appraises the recent developments in idiopathic intracranial hypertension (IIH), with particular attention to novel therapeutic avenues and advanced clinical assessment and monitoring with optical coherence tomography and telemetric intracranial pressure devices. RECENT FINDINGS The incidence of IIH is increasing. The first consensus guidelines for IIH have been published detailing investigation and management algorithms for adult IIH. Improved understanding, clinical assessment and monitoring are emerging with the use of optical coherence tomography. Intracranial pressure telemetry is providing unique insights into the physiology of raised intracranial pressure in IIH. There are now an increasing number of ongoing clinical trials evaluating weight loss methods and novel targeted therapies, such as 11ß-HSD1 inhibition and Glucagon-like peptide 1 (GLP-1) receptor agonists. SUMMARY Several studies are evaluating new therapies for IIH. Monitoring techniques are advancing, aiding diagnosis and allowing the clinician to accurately evaluate changes in papilloedema and intracranial pressure.
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Affiliation(s)
- James L. Mitchell
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham
- Department of Neurology, University Hospitals Birmingham
| | | | - Vivek Vijay
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham
- Department of Neurology, University Hospitals Birmingham
| | - Alexandra J. Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
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Hoffmann J, Kreutz KM, Csapó-Schmidt C, Becker N, Kunte H, Fekonja LS, Jadan A, Wiener E. The effect of CSF drain on the optic nerve in idiopathic intracranial hypertension. J Headache Pain 2019; 20:59. [PMID: 31122204 PMCID: PMC6734439 DOI: 10.1186/s10194-019-1004-1] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2019] [Accepted: 04/22/2019] [Indexed: 01/03/2023] Open
Abstract
Background Elevation of intracranial pressure in idiopathic intracranial hypertension induces an edema of the prelaminar section of the optic nerve (papilledema). Beside the commonly observed optic nerve sheath distention, information on a potential pathology of the retrolaminar section of the optic nerve and the short-term effect of normalization of intracranial pressure on these abnormalities remains scarce. Methods In this exploratory study 8 patients diagnosed with idiopathic intracranial hypertension underwent a MRI scan (T2 mapping) as well as a diffusion tensor imaging analysis (fractional anisotropy and mean diffusivity). In addition, the clinical presentation of headache and its accompanying symptoms were assessed. Intracranial pressure was then normalized by lumbar puncture and the initial parameters (MRI and clinical features) were re-assessed within 26 h. Results After normalization of CSF pressure, the morphometric MRI scans of the optic nerve and optic nerve sheath remained unchanged. In the diffusion tensor imaging, the fractional anisotropy value was reduced suggesting a tissue decompression of the optic nerve after lumbar puncture. In line with these finding, headache and most of the accompanying symptoms also improved or remitted within that short time frame. Conclusion The findings support the hypothesis that the elevation of intracranial pressure induces a microstructural compression of the optic nerve impairing axoplasmic flow and thereby causing the prelaminar papilledema. The microstructural compression of the optic nerve as well as the clinical symptoms improve within hours of normalization of intracranial pressure.
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Affiliation(s)
- Jan Hoffmann
- Department of Neurology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany. .,Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, King's College London, Wellcome Foundation Building, Denmark Hill Campus, London, SE5 9PJ, UK.
| | - Katharina Maria Kreutz
- Department of Neurology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany.,Department of Neuroradiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany
| | - Christoph Csapó-Schmidt
- Department of Neuroradiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany
| | - Nils Becker
- Department of Neurology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany.,Department of Neuroradiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany
| | - Hagen Kunte
- Department of Neurology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany.,Faculty of Natural Sciences, Medical School Berlin, Berlin, Germany
| | - Lucius Samo Fekonja
- Department of Neurosurgery, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Berlin, Germany
| | - Anas Jadan
- Department of Neuroradiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany
| | - Edzard Wiener
- Department of Neuroradiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Germany.
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Radojicic A, Vukovic-Cvetkovic V, Pekmezovic T, Trajkovic G, Zidverc-Trajkovic J, Jensen RH. Predictive role of presenting symptoms and clinical findings in idiopathic intracranial hypertension. J Neurol Sci 2019; 399:89-93. [PMID: 30782528 DOI: 10.1016/j.jns.2019.02.006] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2018] [Revised: 02/04/2019] [Accepted: 02/05/2019] [Indexed: 10/27/2022]
Abstract
BACKGROUND The aim of the study was to evaluate the presenting symptoms and signs of idiopathic intracranial hypertension (IIH) in a large cohort of patients and to estimate their possible role in establishing the diagnosis of IIH. METHODS This prospective cohort study in two tertiary centers, the Danish Headache Center in Rigshospitalet-Glostrup and the Neurology Clinic of the Clinical Center of Serbia, included 286 patients referred by attending specialists for possible IIH evaluation. Patients were divided into two groups: one with confirmed IIH diagnosis and one with rejected IIH diagnosis. RESULTS The diagnosis of IIH was confirmed in 219 (76.6%) patients. It was more often confirmed if the patient was referred by an ophthalmologist than if the referral was from a neurologist (83.6% vs. 69.8%, p = .029) and in patients with higher body mass index (BMI) (p = .032). Transient visual obscurations (p = .006), double vision (p = .033), neck pain (p = .025), and tinnitus (p = .013) were presenting symptoms more frequently reported by patients with IIH diagnosis. In the same group of patients, papilledema (p < .001) and sixth nerve palsy (p = .010) were noted significantly more often. Papilledema was extracted by multivariate analysis as an independent predictor of IIH diagnosis (p < .001). CONCLUSION Although studies investigating IIH report an abundance of presenting symptoms, our results indicate that these symptoms are not diagnostic for IIH. Papilledema is the most reliable clinical sign predicting the correct IIH diagnosis in patients with suspected IIH.
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Affiliation(s)
- Aleksandra Radojicic
- Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, Serbia.
| | - Vlasta Vukovic-Cvetkovic
- Danish Headache Center, Department of Neurology, University of Copenhagen, Rigshospitalet-Glostrup, Denmark
| | - Tatjana Pekmezovic
- Institute of Epidemiology, Faculty of Medicine, University of Belgrade, Serbia
| | - Goran Trajkovic
- Department of Medical Statistics and Informatics, Faculty of Medicine, University of Belgrade, Serbia
| | - Jasna Zidverc-Trajkovic
- Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, Serbia
| | - Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, University of Copenhagen, Rigshospitalet-Glostrup, Denmark
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Affiliation(s)
- Jan Hoffmann
- Department of Systems Neuroscience, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
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Hoffmann J, Mollan SP, Paemeleire K, Lampl C, Jensen RH, Sinclair AJ. European headache federation guideline on idiopathic intracranial hypertension. J Headache Pain 2018; 19:93. [PMID: 30298346 PMCID: PMC6755569 DOI: 10.1186/s10194-018-0919-2] [Citation(s) in RCA: 119] [Impact Index Per Article: 17.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2018] [Accepted: 09/14/2018] [Indexed: 02/08/2023] Open
Abstract
Background Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of intracranial pressure (ICP no identifiable cause. The aetiology remains largely unknown, however observations made in a number of recent clinical studies are increasing the understanding of the disease and now provide the basis for evidence-based treatment strategies. Methods The Embase, CDSR, CENTRAL, DARE and MEDLINE databases were searched up to 1st June 2018. We analyzed randomized controlled trials and systematic reviews that investigate IIH. Results Diagnostic uncertainty, headache morbidity and visual loss are among the highest concerns of clinicians and patients in this disease area. Research in this field is infrequent due to the rarity of the disease and the lack of understanding of the underlying pathology. Conclusions This European Headache Federation consensus paper provides evidence-based recommendations and practical advice on the investigation and management of IIH. Electronic supplementary material The online version of this article (10.1186/s10194-018-0919-2) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Jan Hoffmann
- Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology and Neuroscience, Wellcome Foundation Building, Denmark Hill Campus, King's College London, London, SE5 9PJ, UK.
| | - Susan P Mollan
- Birmingham Neuro-Ophthalmology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, UK
| | - Koen Paemeleire
- Department of Neurology, Ghent University Hospital, Ghent, Belgium
| | - Christian Lampl
- Headache Medical Centre, Seilerstaette Linz, Ordensklinikum Linz, Barmherzige Schwestern, Linz, Austria
| | - Rigmor H Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Glostrup, Denmark
| | - Alexandra J Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston, UK
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Affiliation(s)
- Susan P Mollan
- Birmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham, UK
- Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
- Institute of Translational Medicine, Birmingham Health Partners, University of Birmingham, UK
| | - David Spitzer
- PHGH Doctors, Temple Fortune Medical Centre, London, UK
| | - David J Nicholl
- Department of Neurology, City Hospital, Birmingham, UK
- Department of Neurology, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham, UK
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Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR, Krishnan A, Chavda SV, Ramalingam S, Edwards J, Hemmings K, Williamson M, Burdon MA, Hassan-Smith G, Digre K, Liu GT, Jensen RH, Sinclair AJ. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry 2018; 89:1088-1100. [PMID: 29903905 PMCID: PMC6166610 DOI: 10.1136/jnnp-2017-317440] [Citation(s) in RCA: 333] [Impact Index Per Article: 47.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2017] [Revised: 02/06/2018] [Accepted: 02/07/2018] [Indexed: 01/03/2023]
Abstract
The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). METHODS Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements. RESULTS Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. CONCLUSIONS In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.
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Affiliation(s)
- Susan P Mollan
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK
| | - Brendan Davies
- Department of Neurology, University Hospital North Midlands NHS Trust, Stoke-on-Trent, UK
| | - Nick C Silver
- Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK
| | - Simon Shaw
- Department of Neurosurgery, University Hospital North Midlands NHS Trust, Royal Stoke University Hospital, Stoke-on-Trent, UK
| | - Conor L Mallucci
- Department of Neurosurgery, The Walton Centre NHS Foundation Trust, Liverpool, UK
- Department of Paediatric Neurosurgery, Alder Hey Children's NHS Foundation Trust, Liverpool, UK
| | - Benjamin R Wakerley
- Department of Neurology, Gloucestershire Hospitals NHS Foundation Trust, Cheltenham, UK
- Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, UK
| | - Anita Krishnan
- Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK
| | - Swarupsinh V Chavda
- Department of Neuroradiology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
| | - Satheesh Ramalingam
- Department of Neuroradiology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
| | - Julie Edwards
- Department of Neurology, Sandwell and West Birmingham NHS Trust, Birmingham, UK
- Department of Neurology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
| | | | | | - Michael A Burdon
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK
| | - Ghaniah Hassan-Smith
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
- Department of Neurology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
| | - Kathleen Digre
- Departments of Ophthalmology and Neurology, Moran Eye Center, University of Utah, Salt Lake City, Utah, USA
| | - Grant T Liu
- Neuro-ophthalmology Services, Children's Hospital of Philadelphia and Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark
| | - Alexandra J Sinclair
- Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
- Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK
- Department of Neurology, University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
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Yiangou A, Mitchell J, Markey KA, Scotton W, Nightingale P, Botfield H, Ottridge R, Mollan SP, Sinclair AJ. Therapeutic lumbar puncture for headache in idiopathic intracranial hypertension: Minimal gain, is it worth the pain? Cephalalgia 2018; 39:245-253. [PMID: 29911422 PMCID: PMC6376596 DOI: 10.1177/0333102418782192] [Citation(s) in RCA: 46] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Background Headache is disabling and prevalent in idiopathic intracranial hypertension. Therapeutic lumbar punctures may be considered to manage headache. This study evaluated the acute effect of lumbar punctures on headache severity. Additionally, the effect of lumbar puncture pressure on post-lumbar puncture headache was evaluated. Methods Active idiopathic intracranial hypertension patients were prospectively recruited to a cohort study, lumbar puncture pressure and papilloedema grade were noted. Headache severity was recorded using a numeric rating scale (NRS) 0–10, pre-lumbar puncture and following lumbar puncture at 1, 4 and 6 hours and daily for 7 days. Results Fifty two patients were recruited (mean lumbar puncture opening pressure 32 (28–37 cmCSF). At any point in the week post-lumbar puncture, headache severity improved in 71% (but a small reduction of −1.1 ± 2.6 numeric rating scale) and exacerbated in 64%, with 30% experiencing a severe exacerbation ≥ 4 numeric rating scale. Therapeutic lumbar punctures are typically considered in idiopathic intracranial hypertension patients with severe headaches (numeric rating scale ≥ 7). In this cohort, the likelihood of improvement was 92% (a modest reduction of headache pain by −3.0 ± 2.8 numeric rating scale, p = 0.012, day 7), while 33% deteriorated. Idiopathic intracranial hypertension patients with mild (numeric rating scale 1–3) or no headache (on the day of lumbar puncture, prior to lumbar puncture) had a high risk of post- lumbar puncture headache exacerbation (81% and 67% respectively). Importantly, there was no relationship between lumbar puncture opening pressure and headache response after lumbar puncture. Conclusion Following lumbar puncture, the majority of idiopathic intracranial hypertension patients experience some improvement, but the benefit is small and post-lumbar puncture headache exacerbation is common, and in some prolonged and severe. Lumbar puncture pressure does not influence the post-lumbar puncture headache.
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Affiliation(s)
- Andreas Yiangou
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - James Mitchell
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,3 Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Keira Annie Markey
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - William Scotton
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,3 Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Peter Nightingale
- 4 NIHR/Wellcome Trust Clinical Research Facility, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Hannah Botfield
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
| | - Ryan Ottridge
- 5 Birmingham Clinical Trials Unit, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Susan P Mollan
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,6 Birmingham Neuro-Ophthalmology Unit, Ophthalmology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Alexandra J Sinclair
- 1 Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.,2 Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.,3 Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
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Abstract
Abnormalities of cerebrospinal fluid (CSF) pressure are relatively common and may lead to a variety of symptoms, with headache usually being the most prominent one. The clinical presentation of alterations in CSF pressure may vary significantly and show a striking similitude to several primary headache syndromes. While an increase in CSF pressure may be of primary or secondary origin, a pathologic decrease of CSF pressure is usually the result of a meningeal rupture with a resulting leakage of CSF. The pathophysiologic mechanisms of idiopathic intracranial hypertension (IIH) remain largely unknown. However recent evidence indicates that an abnormality in CSF outflow and absorption is likely to play a significant role. Treatment usually consists of a combination of weight loss and a pharmacologic approach using carbonic anhydrase inhibitors. Recent results of the first randomized, double-blind, placebo-controlled trial (RCT) with acetazolamide proved its efficacy in reducing headache and visual disturbances. Clinical evidence suggests efficacy for topiramate and furosemide but no RCT has been conducted to date to confirm these results. In contrast to IIH, spontaneous intracranial hypotension frequently remits spontaneously without specific treatment. If necessary, treatment options range from conservative methods to epidural blood or fibrin sealant patches and surgical interventions.
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Affiliation(s)
- Jan Hoffmann
- Department of Systems Neuroscience, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
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Gago-Veiga AB, Díaz de Terán J, González-García N, González-Oria C, González-Quintanilla V, Minguez-Olaondo A, Santos-Lasaosa S, Viguera Romero J, Pozo-Rosich P. How and when to refer patients diagnosed with secondary headache and other craniofacial pain in the Emergency Department and Primary Care: Recommendations of the Spanish Society of Neurology's Headache Study Group. Neurologia 2017; 35:323-331. [PMID: 28870392 DOI: 10.1016/j.nrl.2017.08.002] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2017] [Accepted: 08/01/2017] [Indexed: 01/03/2023] Open
Abstract
INTRODUCTION When secondary headache is suspected and the patient is referred to the emergency department or to the outpatient neurology clinic, it is important to know which are the appropriate complementary examinations to perform and the suitable referral pathway for patients to follow. In order to establish recommendations on this matter, the Spanish Society of Neurology's Headache Study Group (GECSEN) has decided to issue a series of agreed recommendations constituting a referral protocol for patients with headache and/or craniofacial neuralgias. DEVELOPMENT Young neurologists with an interest and experience in headache were invited to draft a series of practical guidelines in collaboration with GECSEN's Executive Committee. For practical reasons, the document was divided into 2 articles: the first focuses on primary headaches and craniofacial neuralgias and this second article on secondary headaches and other craniofacial pain. In order for the recommendations to be helpful for daily practice, they follow a practical approach, with tables summarising referral criteria, examinations to be performed, and referral to other specialists. CONCLUSIONS We hope to offer a guide and tools to improve decision-making regarding patients with headache, identifying complementary tests to prioritise and referral pathways to be followed, in order to avoid duplicated consultations and delayed diagnosis and treatment.
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Affiliation(s)
- A B Gago-Veiga
- Unidad de Cefaleas, Servicio de Neurología, Instituto de Investigación Sanitaria Princesa, Hospital Universitario de la Princesa, Madrid, España.
| | - J Díaz de Terán
- Unidad de Cefaleas, Servicio de Neurología, Hospital Universitario La Paz, IdiPAZ, Instituto de Investigación Sanitaria, Madrid, España
| | - N González-García
- Unidad de Cefaleas, Servicio de Neurología, Hospital Clínico San Carlos, Madrid, España
| | - C González-Oria
- Unidad de Cefaleas, Servicio de Neurología, Hospital Universitario Virgen del Rocío, Sevilla, España
| | - V González-Quintanilla
- Unidad de Cefaleas, Servicio de Neurología, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, España
| | - A Minguez-Olaondo
- Departamento de Neurología, Clínica Universitaria de Navarra, Pamplona, Navarra, España; Unidad de Cefaleas, Servicio de Neurología, Hospital Clínico Universitario de Valencia, Valencia, España
| | - S Santos-Lasaosa
- Unidad de Cefaleas, Servicio de Neurología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España
| | - J Viguera Romero
- Unidad Gestión Clínica de Neurología, Hospital Virgen Macarena, Sevilla, España
| | - P Pozo-Rosich
- Unidad de Cefalea, Servicio de Neurología, Hospital Universitari Vall d'Hebron, Barcelona, España; Grupo de Investigación en Cefalea, VHIR, Universitat Autònoma de Barcelona, Barcelona, España
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Friedman DI, Quiros PA, Subramanian PS, Mejico LJ, Gao S, McDermott M, Wall M. Headache in Idiopathic Intracranial Hypertension: Findings From the Idiopathic Intracranial Hypertension Treatment Trial. Headache 2017; 57:1195-1205. [PMID: 28752894 DOI: 10.1111/head.13153] [Citation(s) in RCA: 116] [Impact Index Per Article: 14.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2017] [Revised: 05/03/2017] [Accepted: 05/04/2017] [Indexed: 12/24/2022]
Abstract
OBJECTIVE To characterize the phenotype, headache-related disability, medical co-morbidities, use of symptomatic headache medications, and headache response to study interventions in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). METHODS Patients with untreated IIH and mild vision loss enrolled in the IIHTT and randomized to acetazolamide (ACZ) and weight loss or placebo (PLB) and weight loss had prospective assessment of headache disability using the Headache Impact Test-6 (HIT-6) questionnaire. Subjects with headache at the baseline visit were assigned a headache phenotype using the International Classification for Headache Disorders version 3 beta (ICHD-3b). Medication overuse was determined using the participants' reported medication use for the preceding month and ICHD-3b thresholds for diagnosing medication overuse headache. We investigated relationships between headache disability and various other clinical characteristics at baseline and at 6 months. RESULTS Headache was present in 139 (84%) of the 165 enrollees at baseline. The most common headache phenotypes were migraine (52%), tension-type headache (22%), probable migraine (16%), and probable tension-type headache (4%). Fifty-one (37%) participants overused symptomatic medications at baseline, most frequently simple analgesics. A similar amount of improvement in the adjusted mean (± standard error) HIT-6 score occurred in the ACZ (-9.56 ± 1.05) and PLB groups (-9.11 ± 1.14) at 6 months (group difference -0.45, 95% CI -3.50 to 2.60, P = .77). Headache disability did not correlate with any of the studies, variables of interest, which included: the lumbar puncture opening pressure at baseline or at 6 months, body mass index, the amount of weight lost, papilledema grade, perimetric mean deviation, or the use of hormonal contraception. Headache disability was significantly associated with patient-reported quality of life in the physical, mental, and visual domains. CONCLUSIONS Headache was common, of varied character, disabling, and associated with poorer quality of life in our cohort of patients with mild visual impairment. The lack of correlation between headache disability and cerebrospinal fluid (CSF) pressure at baseline and at the end of the randomized phase of the study implies that headache in IIH may be related to factors other than intracranial hypertension, and that specific headache treatment is needed in addition to therapies directed at lowering CSF pressure.
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Affiliation(s)
- Deborah I Friedman
- Department of Ophthalmology, University of California Los Angeles, Los Angeles, CA, USA
| | - Peter A Quiros
- Departments of Neurology & Neurotherapeutics and Ophthalmology, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | - Prem S Subramanian
- Departments of Ophthalmology, Neurology and Neurosurgery, University of Colorado Denver School of Medicine, Aurora, CO, USA.,Departments of Neurology and Ophthalmology, SUNY UMU, Syracuse, NY, USA
| | - Luis J Mejico
- Departments of Neurology and Ophthalmology, SUNY UMU, Syracuse, NY, USA
| | - Shan Gao
- Department of Biostatistics and Computational Biology, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA
| | - Michael McDermott
- Departments of Biostatistics and Computational Biology, Neurology; Center for Neurotherapeutics, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA
| | - Michael Wall
- Departments of Neurology and Ophthalmology & Visual Sciences, University of Iowa, Iowa City, IA, USA
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Current concepts and strategies in the diagnosis and management of idiopathic intracranial hypertension in adults. J Neurol 2017; 264:1622-1633. [DOI: 10.1007/s00415-017-8401-7] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2016] [Revised: 01/17/2017] [Accepted: 01/18/2017] [Indexed: 10/20/2022]
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Mollan SP, Ali F, Hassan-Smith G, Botfield H, Friedman DI, Sinclair AJ. Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management. J Neurol Neurosurg Psychiatry 2016; 87:982-92. [PMID: 26888960 PMCID: PMC5013119 DOI: 10.1136/jnnp-2015-311302] [Citation(s) in RCA: 159] [Impact Index Per Article: 17.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/01/2015] [Accepted: 01/19/2016] [Indexed: 01/12/2023]
Abstract
Idiopathic intracranial hypertension (IIH) is a rare but important disease associated with significant morbidity. There is an expected rise in prevalence in line with the escalating global burden of obesity. Modern revisions in the terminology and diagnostic criteria for IIH help guide clinicians in investigations and researchers in standardising recruitment criteria for clinical trials. The pathophysiology of IIH is incompletely characterised; suggested underpinning mechanisms include the role of cerebrospinal fluid regulation as well as metabolic and endocrinological perspectives. Recent treatment trials are providing insights into the management but debate still surrounds key areas in treatment. This review will provide an up-to-date discussion on the potential pathogenic mechanisms and management of IIH.
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Affiliation(s)
- Susan P Mollan
- Birmingham Neuro-Ophthalmology Unit, Ophthalmology Department, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Birmingham, UK
| | - Fizzah Ali
- Neurometabolism, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Ghaniah Hassan-Smith
- Neurometabolism, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK Neurology Department, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Birmingham, UK
| | - Hannah Botfield
- Neurometabolism, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
| | - Deborah I Friedman
- Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas, USA Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Alexandra J Sinclair
- Neurometabolism, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK Neurology Department, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Birmingham, UK
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Jensen RH, Radojicic A, Yri H. The diagnosis and management of idiopathic intracranial hypertension and the associated headache. Ther Adv Neurol Disord 2016; 9:317-26. [PMID: 27366239 PMCID: PMC4916517 DOI: 10.1177/1756285616635987] [Citation(s) in RCA: 48] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023] Open
Abstract
Idiopathic intracranial hypertension (IIH) is a challenging disorder with a rapid increasing incidence due to a close relation to obesity. The onset of symptoms is often insidious and patients may see many different specialists before the IIH diagnosis is settled. A summary of diagnosis, symptoms, headache characteristics and course, as well as existing evidence of treatment strategies is presented and strategies for investigations and management are proposed.
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Affiliation(s)
- Rigmor Højland Jensen
- Danish Headache Center, Department of Neurology, Rigshospitalet-Glostrup, University of Copenhagen, Denmark
| | | | - Hanne Yri
- Danish Headache Center, Department of Neurology, University of Copenhagen, Rigshospitalet-Glostrup, 2600 Glostrup, Denmark
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Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 2016; 15:78-91. [PMID: 26700907 DOI: 10.1016/s1474-4422(15)00298-7] [Citation(s) in RCA: 315] [Impact Index Per Article: 35.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2015] [Revised: 09/07/2015] [Accepted: 10/13/2015] [Indexed: 01/10/2023]
Abstract
Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.
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Affiliation(s)
- Keira A Markey
- Neurometabolism Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Edgbaston, Birmingham, UK
| | - Susan P Mollan
- Neurometabolism Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Edgbaston, Birmingham, UK; Birmingham Neuro-Ophthalmology Unit, Ophthalmology Department, University Hospitals Birmingham NHS Trust, Queen Elizabeth Hospital Birmingham, Birmingham, UK
| | - Rigmor H Jensen
- Danish Headache Centre, Department of Neurology, Glostrup Hospital, University of Copenhagen, Copenhagen, Denmark
| | - Alexandra J Sinclair
- Neurometabolism Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Edgbaston, Birmingham, UK.
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