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Munirah MP, Mohamad N, Norhayati MN, Nurul Azman A. Posterior reversible encephalopathy syndrome: A conundrum of nephrotic syndrome complication. ELECTRONIC JOURNAL OF GENERAL MEDICINE 2023. [DOI: 10.29333/ejgm/12835] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
Abstract
Nephrotic syndrome is a kidney disease with proteinuria, hypoalbuminemia, and edema. One rare, potentially life-threatening complication of nephrotic syndrome is posterior reversible encephalopathy syndrome (PRES). Sudden episodes of neurological symptoms such as headache, confusion, seizures, or focal neurological deficits with radiological findings of white matter abnormalities in the parietal and occipital lobes characterize it. Multiple factors predispose an individual with nephrotic syndrome to PRES, such as uncontrolled hypertension, reduced serum albumin levels, administration of drugs (cyclosporine, tacrolimus), anasarca, disturbed body fluid status and renal insufficiency. Here, we report a case of PRES in a seven-year-old girl with nephrotic syndrome who presented with high blood pressure while admitted to the ward. Her neurological symptom rapidly recovered after the control of hypertension. Recurrence of acute severe hypertension, nephrotic state (edema/ hypoalbuminemia), and renal insufficiency may lead to recurrent PRES. Thus, early treatment of trigger factors, especially of hypertension, is vital to reduce the episodes of PRES.
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Affiliation(s)
- Mohd Puad Munirah
- Department of Family Medicine, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, MALAYSIA
| | - Noraini Mohamad
- School of Dental Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, MALAYSIA
| | - Mohd Noor Norhayati
- Department of Family Medicine, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, MALAYSIA
| | - Azzahra Nurul Azman
- Department of Radiology, Hospital Raja Permaisuri Bainun, Ipoh, Perak, MALAYSIA
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Posterior reversible encephalopathy syndrome (PRES) in a 6-year-old child with nephrotic syndrome. Radiol Case Rep 2020; 16:140-144. [PMID: 33240457 PMCID: PMC7674159 DOI: 10.1016/j.radcr.2020.10.057] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2020] [Revised: 10/27/2020] [Accepted: 10/30/2020] [Indexed: 12/05/2022] Open
Abstract
Posterior reversible encephalopathy syndrome (PRES) is a variable etiology clinical syndrome with similar neuroimaging results and clinical symptoms. PRES can develop in both adults and children and is characterized by headaches, disorders of consciousness, seizures and especially focal visual disturbances, often associated with hypertensive state. In most cases, symptoms resolve without neurological consequences. The treatment strategy concerns early diagnosis and general measures to correct the underlying cause of PRES. Here, we report a case of PRES that occurs in a 6-year-old child with nephrotic syndrome.
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Posterior reversible encephalopathy syndrome in children with kidney disease. Int Urol Nephrol 2017; 49:1793-1800. [DOI: 10.1007/s11255-017-1684-x] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2017] [Accepted: 08/17/2017] [Indexed: 01/13/2023]
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Ahn CH, Han SA, Kong YH, Kim SJ. Clinical characteristics of hypertensive encephalopathy in pediatric patients. KOREAN JOURNAL OF PEDIATRICS 2017; 60:266-271. [PMID: 29042869 PMCID: PMC5638725 DOI: 10.3345/kjp.2017.60.8.266] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/22/2017] [Revised: 05/21/2017] [Accepted: 06/12/2017] [Indexed: 12/25/2022]
Abstract
Purpose The aim of this study was to assess the clinical characteristics of hypertensive encephalopathy according to the underlying etiologies in children. Methods We retrospectively evaluated 33 pediatric patients who were diagnosed as having hypertensive encephalopathy in Chonbuk National University Children's Hospital. Among the patients, 18 were excluded because of incomplete data or because brain magnetic resonance imaging (MRI) was not performed. Finally, 17 patients were enrolled and divided into a renal-origin hypertension group and a non-renal-origin hypertension group according to the underlying cause. We compared the clinical features and brain MRI findings between the 2 groups. Results The renal group included renal artery stenosis (4), acute poststreptococcal glomerulonephritis (2), lupus nephritis (2), and acute renal failure (1); the nonrenal group included essential hypertension (4), pheochromocytoma (2), thyrotoxicosis (1), and acute promyelocytic leukemia (1). The mean systolic blood pressure of the renal group (172.5±36.9 mmHg) was higher than that of the nonrenal group (137.1±11.1 mmHg, P<0.05). Seizure was the most common neurologic symptom, especially in the renal group (P<0.05). Posterior reversible encephalopathy syndrome (PRES), which is the most typical finding of hypertensive encephalopathy, was found predominantly in the renal group as compared with the nonrenal group (66.6% vs. 12.5%, P<0.05). Conclusion We conclude that the patients with renal-origin hypertension had a more severe clinical course than those with non-renal-origin hypertension. Furthermore, the renal-origin group was highly associated with PRES on brain MRI.
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Affiliation(s)
- Chang Hoon Ahn
- Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea
| | - Seung-A Han
- Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea
| | - Young Hwa Kong
- Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea
| | - Sun Jun Kim
- Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea.,Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea
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Mukherjee D, Sinha R, Akhtar MS, Saha AS. Hyponatremic hypertensive syndrome - a retrospective cohort study. World J Nephrol 2017; 6:41-44. [PMID: 28101450 PMCID: PMC5215207 DOI: 10.5527/wjn.v6.i1.41] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2016] [Revised: 08/02/2016] [Accepted: 10/27/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To ascertain the frequency of hyponatremic hypertensive syndrome (HHS) in a cohort of children with hypertensive emergency in a tertiary pediatric hospital.
METHODS A retrospective review was undertaken among children with hypertensive emergency admitted in our tertiary children hospital between June 2014 and December 2015 with an aim to identify any children with HHS. Three children with HHS were identified during this period.
RESULTS The 3 patients with HHS presented with hypertensive emergency. They were initially managed with Labetalol infusion and thereafter switched to oral anti-hypertensives (combination of Nifedipine sustained release, Hydralazine and Beta Blocker). All 3 were diagnosed to have unilateral renal artery stenosis. One child was lost to follow up, whereas the other 2 underwent renal angioplasty which was followed with normalization of blood pressure.
CONCLUSION Despite activation of renin angiotensin axis secondary to renal artery stenosis, these groups of children have significant hyponatremia. Renal re-vascularisation produces excellent results in most of them.
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Chen TH, Lin WC, Tseng YH, Tseng CM, Chang TT, Lin TJ. Posterior Reversible Encephalopathy Syndrome in Children: Case Series and Systematic Review. J Child Neurol 2013; 28:1378-1386. [PMID: 24065581 DOI: 10.1177/0883073813500714] [Citation(s) in RCA: 47] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
To study presentations and outcome of posterior reversible encephalopathy syndrome in children, we retrospectively analyzed 14 patients admitted to our pediatric intensive care unit. We further assessed 94 additional pediatric cases from a systematic review. Our patients had a mean age of 11.6 years. Their precipitating factors were hypertension (100%), immunosuppressants (71%), antineoplastic agents (21%), and hemodialysis (14%). Initial neurologic manifestations included seizures (100%), mental change (100%), headache (79%), and visual disturbance (57%). After prompt diagnosis by magnetic resonance imaging (MRI) with intensive management, all patients had complete clinical recovery with subsequent radiologic resolution. Systemic literature review indicated that seizures (90%), hypertension (85%), and atypical neuroimaging findings (80%) are common presentations in childhood posterior reversible encephalopathy syndrome. We conclude that in children presenting with seizures and hypertension, a pediatric neurologist should consider posterior reversible encephalopathy syndrome within a comprehensive differential diagnosis of acute encephalopathy. Early recognition and intensive care are essential to ensure complete neurologic recovery in children with posterior reversible encephalopathy syndrome.
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Affiliation(s)
- Tai-Heng Chen
- 1Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
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Emergency management of hypertension in children. Int J Nephrol 2012; 2012:420247. [PMID: 22577545 PMCID: PMC3345222 DOI: 10.1155/2012/420247] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2011] [Revised: 12/13/2011] [Accepted: 01/12/2012] [Indexed: 12/18/2022] Open
Abstract
Systemic arterial hypertension in children has traditionally been thought to be secondary in origin. Increased incidence of risk factors like obesity, sedentary life-styles, and faulty dietary habits has led to increased prevalence of the primary arterial hypertension (PAH), particularly in adolescent age children. PAH has become a global epidemic worldwide imposing huge economic constraint on health care. Sudden acute increase in systolic and diastolic blood pressure can lead to hypertensive crisis. While it generally pertains to secondary hypertension, occurrence of hypertensive crisis in PAH is however rare in children. Hypertensive crisis has been further subclassified depending on presence or absence of end-organ damage into hypertensive emergency or urgency. Both hypertensive emergencies and urgencies are known to cause significant morbidity and mortality. Increasing awareness among the physicians, targeted at investigation of the pathophysiology of hypertension and its complications, better screening methods, generation, and implementation of novel treatment modalities will impact overall outcomes. In this paper, we discuss the etiology, pathogenesis, and management of hypertensive crisis in children. An extensive database search using keywords was done to obtain the information.
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Posterior reversible encephalopathy syndrome in children with kidney diseases. Pediatr Nephrol 2012; 27:375-84. [PMID: 21556718 DOI: 10.1007/s00467-011-1873-2] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2011] [Revised: 03/16/2011] [Accepted: 03/16/2011] [Indexed: 10/18/2022]
Abstract
Posterior reversible encephalopathy syndrome (PRES) was originally used to describe a reversible, predominantly posterior leukoencephalopathy in patients who had renal insufficiency, hypertension, or who received immunosuppressive therapy. Since PRES is prevalent in children with kidney diseases, awareness and understanding of it is important for practicing pediatric nephrologists. A comprehensive approach to the diagnosis of PRES includes thorough determination of predisposing factors, clinical symptoms, and mandatory appropriate imaging. Unfortunately, the pathophysiology of PRES is still obscure and specificity of radiological examination has not yet been established. Two major predisposing factors, namely hypertension and calcineurin inhibitors, are well recognized. In addition, nephrotic syndrome is a common underlying condition for development of PRES. Frequent symptoms include altered consciousness (coma, stupor, lethargy, confusion), seizure, headache, and visual disturbance. Most of these symptoms usually develop abruptly and resolve within a few weeks after proper management. Cranial magnetic resonance (MR) imaging is the first-line modality of imaging studies for detecting PRES. Diffusion-weighted imaging with quantification of apparent diffusion coefficient (ADC) values by ADC mapping may provide more accurate and specific images in the future.
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Day E, Stephens S, Rigden SPA, Reidy JF, Sinha MD. Malignant hypertension secondary to renovascular disease during infancy--an unusual cause of failure to thrive. Nephrol Dial Transplant 2011; 26:3816-9. [PMID: 21965587 DOI: 10.1093/ndt/gfr523] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Abstract
An 11-month-old girl presented with a history of failure to thrive, vomiting, polydipsia, polyuria and visual inattention. She was found to have malignant hypertension due to unilateral renal artery stenosis. This was successfully treated with percutaneous transluminal balloon angioplasty. Nearly 10 years following this initial presentation, she remains normotensive on no anti-hypertensive medications.
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Affiliation(s)
- Elisabeth Day
- Department of Paediatric Nephrology, Evelina Children’s Hospital, Guys and St Thomas’ NHS Foundation Trust, London, UK
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Lim HR, Seo HE, Kwon SH. Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children. KOREAN JOURNAL OF PEDIATRICS 2007. [DOI: 10.3345/kjp.2007.50.9.901] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Affiliation(s)
- Hae-Ri Lim
- Department of Pediatrics, college of Medicine, Kyungpook National University, Daegu, Korea
| | - Hye-Eun Seo
- Department of Pediatrics, college of Medicine, Kyungpook National University, Daegu, Korea
| | - Sun-Hak Kwon
- Department of Pediatrics, college of Medicine, Kyungpook National University, Daegu, Korea
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Anzai Y, Hayashi M, Ohya T. Zonisamide eradicated paroxysmal headache with EEG abnormalities triggered by hypertensive encephalopathy due to purpura nephritic syndrome. Brain Dev 2006; 28:610-3. [PMID: 16806778 DOI: 10.1016/j.braindev.2006.04.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2005] [Revised: 12/16/2005] [Accepted: 04/10/2006] [Indexed: 11/25/2022]
Abstract
Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks of headache, vomiting and disturbed consciousness with left side-predominant EEG abnormalities. Although carbamazepine and sodium valproate failed to improve her condition, zonisamide eradicated both the symptoms and EEG abnormalities, and an attack has not reoccurred for 5 years since completion of her treatment. It is noteworthy that delayed-onset complications can occur in child hypertensive encephalopathy, cases of which should be followed up prudently. Zonisamide should be considered for treatment of attacks of headaches with an epileptic character.
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Affiliation(s)
- Yuki Anzai
- Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan.
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Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Shishido S, Asanuma H, Nishimura G, Hiramoto R, Honda M. Posterior Reversible Encephalopathy Syndrome in Children: Its High Prevalence and More Extensive Imaging Findings. Am J Kidney Dis 2006; 48:231-8. [PMID: 16860188 DOI: 10.1053/j.ajkd.2006.04.076] [Citation(s) in RCA: 64] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2005] [Accepted: 04/17/2006] [Indexed: 11/11/2022]
Abstract
BACKGROUND Posterior reversible encephalopathy syndrome is a distinctive clinicoradiological entity observed in a variety of clinical settings, including pediatric patients. A greater prevalence of this syndrome has been suggested in kidney transplant recipients and patients with kidney disease. Although usually considered benign and reversible, characteristics of this syndrome in pediatric patients remain obscure. The objective of the present study involved disclosing details of imaging findings, as well as the clinical course and prevalence of the syndrome in this field. METHODS We investigated kidney transplant recipients and pediatric patients with kidney disease in our institution from 1990 to 2004. For these patients, clinical course, imaging findings, blood pressure, concurrent medical illnesses, and administrative condition of calcineurin inhibitors were analyzed. RESULTS Twenty cases of posterior reversible encephalopathy syndrome were investigated in patients ranging in age from 1.9 to 18.3 years. In most patients, radiological abnormalities extended to the gray matter (17 of 20 patients), frontal and temporal lobes, and even the cerebellum (16 patients). Of 177 kidney transplant recipients (cyclosporine, 127 patients; tacrolimus, 50 patients), 6 patients administered cyclosporine (4.7%) and 4 patients administered tacrolimus (8.0%) developed the syndrome after transplantation. CONCLUSION Posterior reversible encephalopathy syndrome should be suspected in pediatric kidney transplant recipients and patients with kidney disease if they have a sudden episode of neurological symptoms, even if imaging findings are not restricted to the subcortical white matter of the occipital region.
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Affiliation(s)
- Kenji Ishikura
- Department of Pediatric Nephrology, Pediatric Urology and Kidney Transplant, and Pediatric Radiology, Tokyo Metropolitan Kiyose Children's Hospital, Japan.
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Adamson DC, Dimitrov DF, Bronec PR. Upward transtentorial herniation, hydrocephalus, and cerebellar edema in hypertensive encephalopathy. Neurologist 2005; 11:171-5. [PMID: 15860139 DOI: 10.1097/01.nrl.0000159982.63592.9f] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
BACKGROUND Edema of the cerebellum with secondary obstructive hydrocephalus is a rare presentation of hypertensive encephalopathy. The authors report an unusual case of isolated posterior fossa swelling with upward transtentorial herniation and hydrocephalus causing neurologic deterioration. These patients are often initially evaluated by a neurologist because of the acute neurologic symptoms. Prompt diagnosis with aggressive blood pressure control may obviate the need for emergent cerebrospinal fluid (CSF) diversion. REVIEW SUMMARY This is a case report of a 26-year-old man who presented to the emergency room with confusion and somnolence over a 2-day period. His initial blood pressure was 175/110 mmHg. On examination he was disoriented, with a Glasgow Coma Scale score of 12 points, opening his eyes only to loud verbal stimuli, verbalizing inappropriately, and he was only able to follow simple commands. Neuroimaging revealed edema of the cerebellar folia with noncommunicating hydrocephalus and upward transtentorial herniation. Differential diagnoses of posterior fossa tumor, rhombencephalitis, and hypertensive encephalopathy were entertained. A thorough literature review is included with the discussion of this case. The patient underwent emergent ventriculostomy for CSF drainage and prompt blood pressure control with nitroprusside. After 48 hours of CSF drainage and correction of his hypertension, his neurologic examination normalized. Repeat imaging revealed near resolution of the obstructive hydrocephalus and cerebellar edema. CONCLUSION Isolated edema of the cerebellum with upward transtentorial herniation and obstructive hydrocephalus is a rare presentation of hypertensive encephalopathy and should be considered in patients with an acute hypertensive crisis and mental status changes. This entity responds to prompt blood pressure control; however, emergent ventriculostomy by a neurosurgical team should be entertained for neurologic deterioration secondary to significant obstructive hydrocephalus, as illustrated in this case.
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Affiliation(s)
- David C Adamson
- Division of Neurosurgery and Durham County Regional Hospital, Duke University Medical Center, Durham, NC 27710, USA.
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Ohtomo Y, Takada M, Fujinaga SI, Murakami H, Yamashiro Y. Hypertensive encephalopathy in a boy with biopsy-proven acute post-streptococcal glomerulonephritis. Pediatr Int 2005; 47:323-5. [PMID: 15910459 DOI: 10.1111/j.1442-200x.2005.02071.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Affiliation(s)
- Yoshiyuki Ohtomo
- Division of Nephrology, Saitama Children's Medical Center, Saitama, Japan.
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Feske SK. HYPERPERFUSION SYNDROMES. Continuum (Minneap Minn) 2005. [DOI: 10.1212/01.con.0000293722.63368.90] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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Leonard MB, Kasner SE, Feldman HI, Schulman SL. Adverse neurologic events associated with rebound hypertension after using short-acting nifedipine in childhood hypertension. Pediatr Emerg Care 2001; 17:435-7. [PMID: 11753188 DOI: 10.1097/00006565-200112000-00008] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
INTRODUCTION Short-acting nifedipine (SA-NIF) is widely prescribed for acute hypertension (HTN) in children despite reports of ischemic complications in adults. We describe two children with neurologic events caused by rebound hypertension following SA-NIF use. CASES Patient 1 is a 7-year-old with acute nephritis and blood pressure (BP) of 185/130. She received SA-NIF which decreased BP to 114/79. When BP rebounded to 160/103, she developed severe cortical visual impairment. Head CT demonstrated edema and petechial hemorrhages in the watershed region. Patient 2 is a 10-year-old renal transplant recipient who received SA-NIF for a BP of 155/98, which resulted in a prompt decrease to 114/74. Two hours later he developed aphasia and right-sided neglect. His BP increased to 168/88 and he developed partial complex seizures. Brain MRI showed high signal intensity in the watershed areas with early gadolinium enhancement. DISCUSSION The temporal association of the neurologic events with the rebound increase in BP suggests a possible role for the SA-NIF, consistent with its pharmacokinetic profile. Although the adult literature has focused on the unpredictable decline in BP after SA-NIF treatment, these cases suggest that rapid increases in BP following the maximal SA-NIF effect may be associated with impaired cerebral autoregulation and encephalopathy in children. These cases underscore the need for frequent blood pressure determinations and therapy to prevent rebound hypertension.
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Affiliation(s)
- M B Leonard
- Department of Pediatrics, The Children's Hospital of Philadelphia, PA 19104, USA.
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Abstract
A retrospective review was carried out of patients under 16 years old with malignant hypertension, who had been referred to a teaching hospital ophthalmology department because of reduced visual acuity. Four patients (three girls, one boy) were seen between 1994 and 2000 with a mean age at presentation of 11.5 years (range 9-15). In the short term, visual acuity improved after control of blood pressure in all four patients. However, in the long term, two patients were registered blind one to two years after presentation, one because of a choroidal neovascular membrane developing at the macula, and the other because of progressive optic neuropathy. Both of these patients had a longer duration of symptoms before diagnosis, worse visual acuity, and higher blood pressure at presentation when compared with the patients who made a good visual recovery. These observations suggest that early diagnosis of malignant hypertension in children is essential in reducing the likelihood of permanent severe visual damage.
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Affiliation(s)
- A C Browning
- Department of Ophthalmology and Visual Sciences, University Hospital, Queen's Medical Centre, Nottingham NG7 2UH, UK.
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Affiliation(s)
- F J Kirkham
- Neurosciences Unit, Institute of Child Health (University College London), 30 Guilford Street, London WC1N 1EH.
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Abstract
Reversible posterior leukoencephalopathy syndrome is a recently recognized disorder with characteristic radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. This complex syndrome is associated with cyclosporine A therapy or a variety of other conditions in which blood pressure rises acutely. Twelve patients from a variety of conditions who met the diagnostic criteria for this syndrome were studied. Interestingly, three of these patients had intra-abdominal neurogenic tumors, which have rarely been reported. Initial cranial magnetic resonance imaging scans revealed fairly symmetric areas of increased T(2) signal involving both white and gray matter of parieto-occipital lobes in the majority of the patients. However, the lesions were often located outside the parieto-occipital regions. Four patients had occipital region magnetic resonance spectroscopy during the acute phase, which revealed high lactate peak and normal N-acetyl aspartate/creatine and choline peaks. With appropriate treatment, most patients recovered from this syndrome and experienced almost complete resolution of brain lesions on follow-up magnetic resonance imaging. One patient, however, continued to have small residual hemosiderin deposits on a follow-up magnetic resonance imaging with neurologic sequellae. In conclusion, a better understanding of this complex syndrome may obviate unnecessary investigations and allow management of the associated problems in prompt and appropriate ways.
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Affiliation(s)
- S Kwon
- Department of Pediatrics, Kyungpook National University Hospital, Samdeok 2-50, Joong-Gu, Taegu 7000-721, South Korea
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Cooney MJ, Bradley WG, Symko SC, Patel ST, Groncy PK. Hypertensive encephalopathy: complication in children treated for myeloproliferative disorders--report of three cases. Radiology 2000; 214:711-6. [PMID: 10715035 DOI: 10.1148/radiology.214.3.r00mr19711] [Citation(s) in RCA: 56] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
We routinely perform echo-planar diffusion-weighted sequences in all brain magnetic resonance (MR) imaging studies. When three children undergoing chemotherapy for acute leukemia presented with seizures, conventional MR images demonstrated what appeared to be acute, posterior, parasagittal infarcts. However, diffusion-weighted images were normal. These MR imaging findings were consistent with those of hypertensive encephalopathy. Early recognition and treatment of minimal hypertension in these patients allows reversal of encephalopathy.
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Affiliation(s)
- M J Cooney
- Department of Radiology, Long Beach Memorial Medical Center, CA 90806, USA
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Froehlich T, Sandifer S, Varma PK, Testa FM. Two cases of hypertension-induced reversible posterior leukoencephalopathy syndrome secondary to glomerulonephritis. Curr Opin Pediatr 1999; 11:512-8. [PMID: 10590909 DOI: 10.1097/00008480-199912000-00007] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Affiliation(s)
- T Froehlich
- Department of Pediatrics, Children's Hospital of Pennsylvania, Philadelphia 19104, USA
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Pavlakis SG, Frank Y, Chusid R. Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: three names for an old syndrome. J Child Neurol 1999; 14:277-81. [PMID: 10342593 DOI: 10.1177/088307389901400502] [Citation(s) in RCA: 117] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Children with hypertension, seizures, lethargy, encephalopathy, headache, and occipital blindness are reviewed. After undergoing antihypertensive therapy, most children improve. Some patients have a similar syndrome associated with chemotherapy, transplantation, transfusion, or human immunodeficiency virus-1 (HIV-1) infection. These latter children can develop symptoms with only minimal or no discernible elevations in blood pressure and improve, in the case of cancer-associated encephalopathy, after discontinuing chemotherapy. The reported children with this distinctive clinical condition are compared to adults with reversible posterior leukoencephalopathy syndrome. Since both gray and white matter are involved, we had suggested previously that the name be changed to (reversible) occipitoparietal encephalopathy syndrome. However, reversible posterior leukoencephalopathy has been used in the adult population and probably should be employed in children for the sake of uniformity, since both children and adults have the same clinical presentation and presumably a similar pathophysiology for the encephalopathy syndrome. The diagnosis is confirmed by reversible posterior abnormalities seen on T2-weighted brain magnetic resonance imaging, and by the presence of either headache, altered mental status, seizures, or visual disturbances.
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Affiliation(s)
- S G Pavlakis
- Department of Neurology, North Shore University Hospital, New York University, Manhasset 11030, USA.
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Abstract
A boy presented with hypertension, seizures, lethargy, headache, and occipital blindness. He improved with antihypertensive therapy. Other reported children with a similar distinctive clinical condition are compared with adults with a syndrome termed reversible posterior leukoencephalopathy. Because both gray and white matter are involved, we suggest that the name be changed to occipital-parietal encephalopathy syndrome.
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Affiliation(s)
- S G Pavlakis
- Department of Neurology, North Shore University Hospital, Manhasset, NY, 11030, USA
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