Optic neuropathy and central nervous system disease secondary to Sjögren's syndrome in a child.
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.
Berman
J L
JL
Department of Ophthalmology, Albert Einstein College of Medicine, New York, NY.
Kashii
S
S
Trachtman
M S
MS
Burde
R M
RM
D001927:Brain Diseases / Q000188:drug therapy / Q000209:etiology*; D002544:Cerebral Infarction / Q000188:drug therapy / Q000209:etiology; D002648:Child; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009901:Optic Nerve Diseases / Q000188:drug therapy / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology; D014792:Visual Acuity / Q000187:drug effects
D007166:Immunosuppressive Agents; D011241:Prednisone