Familial Neuromyelitis Optica and Sjogren's Overlap Syndrome-A Rare Case Report

摘要

Familial cases of NMO are rare; and there are very few reports in association with Sjogren's syndrome. To report a familial case of NMO-Sjogren's Overlap syndrome. Mother and daughter presented with phenotypically different neurological episodes; mother had recurrent optic neuritis and myelitis with brainstem episode while the daughter had diencephalic presentation. Both were found to have AQP4 positivity with Ro-52 antibodies and positive Schirmer's test. They responded to steroids and plasma exchange. NMO associated with Sjogren's syndrome association suggests that apart from ethnicity both may have similar genetic predisposition and HLA-linkage.