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

Arzu Çoban; Selen Özyurt; Kaan Meriç; Handan Mısırlı; Erdem Tüzün; Recai Türkoğlu

doi:10.2169/internalmedicine.55.6222

CASE REPORTS

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

Arzu Çoban, Selen Özyurt, Kaan Meriç, Handan Mısırlı, Erdem Tüzün, Recai Türkoğlu

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Keywords: limbic encephalitis, autoimmune encephalitis, Sjögren's syndrome, motor neuropathy, immunotherapy

JOURNAL OPEN ACCESS

2016 Volume 55 Issue 16 Pages 2285-2289

DOI https://doi.org/10.2169/internalmedicine.55.6222

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Abstract

Sjögren'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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