Optic neuropathy and central nervous system disease secondary to Sjögren's syndrome in a child.

Publication/Presentation Date

12-1-1990

Abstract

The authors describe a 10-year-old girl in whom optic neuropathy and central nervous system (CNS) disease developed in association with primary Sjögren's syndrome. There was angiographic evidence of cerebral vasculitis and multiple infarcts present on neuroimaging. Results of parotid biopsy, cerebrospinal fluid, and serologic analyses showed abnormalities that were consistent with the diagnosis of Sjögren's syndrome. Although the patient had optic disc pallor on initial evaluation, her color vision and acuity improved with immunosuppressive therapy, as did her other neurologic symptoms. The authors believe this represents the first reported case of optic neuropathy and CNS disease associated with primary Sjögren's syndrome in the pediatric population. The possibility of improvement in visual function with early institution of immunosuppressive therapy makes prompt diagnosis essential.

Volume

97

Issue

12

First Page

1606

Last Page

1609

ISSN

0161-6420

Disciplines

Medicine and Health Sciences

PubMedID

2087291

Department(s)

Department of Medicine

Document Type

Article

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