| Focal encephalopathy with recurrent episodes of epileptic status and cluster mimicking hemiconvulsion-hemiplegia-epilepsy syndrome. | |
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MedLine Citation:
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PMID: 21752561 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Hemiconvulsion-hemiplegia-epilepsy syndrome is characterized by unilateral convulsions during fever, transient hemiplegia, and subsequent partial epilepsy with atrophy in the cerebrum. A 9-year-old boy with a history of West syndrome and hypoglycemic attacks had three episodes of epileptic status and clusters mimicking HHE syndrome over a 2-year period. Magnetic resonance imaging revealed the involvement of the right and left cerebrums. Because no abnormalities were detected in an endocrine examination, screening tests for metabolic errors, or magnetic resonance spectroscopy, a diagnosis of metabolic errors was not supported. Immunohistochemistry using the patient's sera showed binding of the serum immunoglobulin with neurons in the temporal and occipital cerebral cortices, indicating the possible involvement of autoimmune mechanisms in this case. Focal encephalopathy should be considered in children showing convulsions, psychiatric disorders, and/or involuntary movements for several months in a row. In such cases, immunohistochemistry using the patient's sera may be useful for the investigation of the pathogenesis of the illness. |
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Authors:
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Rie Miyata; Naoyuki Tanuma; Masaharu Hayashi; Yukitoshi Takahashi |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-7-11 |
Journal Detail:
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Title: Brain & development Volume: - ISSN: 1872-7131 ISO Abbreviation: - Publication Date: 2011 Jul |
Date Detail:
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Created Date: 2011-7-14 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7909235 Medline TA: Brain Dev Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Pediatrics, Tokyo-Kita Social Insurance Hospital, Tokyo, Japan. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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