| Rasmussen encephalitis associated with segmental vitiligo of the scalp: clinicopathologic report. | |
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MedLine Citation:
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PMID: 11392525 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 3-year-old boy with segmental vitiligo, poliosis, and alopecia over the right side of the scalp developed epilepsia partialis continua involving the left side of the body and progressive atrophy of the right cerebral hemisphere. There was a right ear dysacusia and a perilimbal vitiligo associated with an area of iris depigmentation in the right eye. Pleocytosis and hypergammaglobulin were detected in cerebrospinal fluid. Because medical treatment that included phenobarbital, phenytoin, carbamazepine, oxcarbazepine, benzodiazepines, corticosteroids, gamma-globulin, and a ketogenic diet was ineffective, he underwent a right hemispherectomy. Neuropathologic examination showed a widespread scattered inflammatory process with numerous microglial nodules and perivascular lymphocytic cuffing associated with degenerative changes with severe neuronal loss, loosening of the neuropil, and microcystic changes with tissue collapse. The coexistence of vitiligo and possibly Vogt-Koyanagi-Harada syndrome in this child reinforces the autoimmune theory as the pathogenesis of Rasmussen syndrome. |
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Authors:
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E M Yacubian; S Rosemberg; T L Garrido Neto; S K Marie; R M Valério; C L Jorge |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of child neurology Volume: 16 ISSN: 0883-0738 ISO Abbreviation: J. Child Neurol. Publication Date: 2001 May |
Date Detail:
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Created Date: 2001-06-05 Completed Date: 2001-12-07 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8606714 Medline TA: J Child Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 374-7 Citation Subset: IM |
Affiliation:
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Department of Neurology, University of São Paulo School of Medicine, Brazil. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Brain
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pathology,
radiography Child, Preschool Electroencephalography Encephalitis / complications*, diagnosis Functional Laterality / physiology Hearing Disorders / complications, diagnosis Humans Magnetic Resonance Imaging Male Nerve Degeneration / physiopathology Scalp Tomography, X-Ray Computed Vitiligo / complications*, physiopathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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