Sisters with clinically mild encephalopathy with a reversible splenial lesion (MERS)-like features; Familial MERS?

Abstract We first report sisters presenting with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)-like features, i.e., mild and reversible neurological manifestations, and MRI finding of a reversible lesion with transiently reduced diffusion in the corpus callosum...

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Bibliographic Details
Published in:	Journal of the neurological sciences Vol. 290; no. 1; pp. 153 - 156
Main Authors:	Imamura, Takuji, Takanashi, Jun-ichi, Yasugi, Jun, Terada, Hitoshi, Nishimura, Akira
Format:	Journal Article
Language:	English
Published:	Amsterdam Elsevier B.V 15-03-2010 Elsevier
Subjects:	Adenoviridae Infections - complications Asian Continental Ancestry Group Biological and medical sciences Brain - pathology Brain - physiopathology Brain Diseases, Metabolic - genetics Brain Diseases, Metabolic - pathology Brain Diseases, Metabolic - virology Brain Edema - metabolism Brain Edema - physiopathology Child Child, Preschool Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) Corpus Callosum - pathology Corpus Callosum - physiopathology Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Diffusion Diffusion Magnetic Resonance Imaging Encephalitis - genetics Encephalitis - pathology Encephalitis - virology Encephalopathy Familial Female Genetic Predisposition to Disease - genetics Humans Inheritance Patterns - genetics Japan Medical sciences Nerve Fibers, Myelinated - metabolism Nerve Fibers, Myelinated - pathology Neurology Reversible Siblings Splenium Syndrome Water-Electrolyte Balance Japan Splenium Reversible Diffusion Clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) Familial Encephalopathy Encephalitis Nervous system diseases Central nervous system disease Cerebral disorder
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Summary:	Abstract We first report sisters presenting with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)-like features, i.e., mild and reversible neurological manifestations, and MRI finding of a reversible lesion with transiently reduced diffusion in the corpus callosum associated with symmetrical white matter. The distributions of the white matter lesions (more extensive) and Na levels (normal) were different from those reported for sporadic MERS patients (subcortical white matter close to the central sulci, and hyponatremia), which suggested that the pathophysiology of the sisters with MERS-like features might be different from that of sporadic MERS. Some genetic factors might be involved in MERS, especially in some patients with extensive white matter lesions.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 ObjectType-Article-2 ObjectType-Feature-1
ISSN:	0022-510X 1878-5883
DOI:	10.1016/j.jns.2009.12.004