4.2 Article

Limbic Encephalitis Associated with Sjogren's Syndrome: Report of Three Cases

Journal

INTERNAL MEDICINE
Volume 55, Issue 16, Pages 2285-2289

Publisher

JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.55.6222

Keywords

limbic encephalitis; autoimmune encephalitis; Sjogren's syndrome; motor neuropathy; immunotherapy

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Sjogren's syndrome (SS) may be complicated by neurological manifestations. We herein report three women (age range 26-60 years old) who all presented with limbic encephalitis (LE) as the predominant clinical feature 3 months to 15 years after the diagnosis of SS. The 26-year-old patient also developed acute motor axonal neuropathy one week after autoimmune encephalitis. All three patients showed contrast-enhanced MRI lesions and inflammatory cerebrospinal fluid findings, while not displaying any anti-neuronal antibodies and showing a remarkable response to immunotherapy. SS is often overlooked when the symptoms are mild. Therefore, in LE cases with no identifiable cause, serological screening for rheumatologic disorders is recommended.

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