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Chronic encephalitis, epilepsy, and cerebrovascular immune complex deposits
Author(s) -
Andrews John M.,
Thompson Joel A.,
Pysher Theodore J.,
Walker Marian L.,
Hammond M. Elizabeth
Publication year - 1990
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410280116
Subject(s) - pathology , cerebrospinal fluid , medicine , atrophy , cerebral vasculitis , encephalitis , epilepsy , hemispherectomy , vasculitis , immunoglobulin g , antibody , immunology , virus , disease , psychiatry
Refractory epilepsy, electroencephalographic abnormalities, progressive hemiplegia, and contralateral hemicerebral atrophy developed in a previously healthy 3‐year, 9‐month‐old girl. Extensive laboratory testing showed elevated serum antinuclear antibody titers, cerebrospinal fluid oligoclonal bands, and elevated immunoglobulin G (IgG): albumin ratio, IgG index, and IgG synthesis rate: Pathological study of a subtotal hemispherectomy specimen revealed widespread cerebral vasculitis with immunofluorescence staining for IgG, IgM, IgA, C3, and C1q, and ultrastructural evidence of vascular injury in addition to severe cortical atrophy with marked neuronal loss. Cerebrospinal fluid abnormalities in other reported patients siuggest that immunological abnormalities may not be unique to this girl. These data suggest possible immunopathogenetic mechanisms in these patients.