Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Atypical posterior reversible encephalopathy syndrome associated with Sjögren's syndrome: a case report

Shion Kachi, M.D.1), Taichi Nomura, M.D., Ph.D.1) *, Kazuki Yamada, M.D.1), Yuki Oshima, M.D.1) and Shigehisa Ura, M.D.1)

Corresponding author: Department of Neurology, Japanese Red Cross Asahikawa Hospital
[1-1-1-1 Akebono, Asahikawa City, Hokkaido 070-8530, Japan]
1) Department of Neurology, Japanese Red Cross Asahikawa Hospital

Sjögren's syndrome (SJS) is a common autoimmune disease. Generally, posterior reversible encephalopathy syndrome (PRES) is often concomitant with autoimmune disease; however, PRES rarely complicates SJS. Thus, the detailed clinical course of cases with SJS and PRES remains unknown. We present the case of a 71-year-old female patient with primary SJS, whose magnetic resonance (MR) images showed bilateral vasogenic edema in the basal ganglia, brainstem, and cerebellum. Cerebrospinal fluid (CSF) examination revealed increased IgG index and higher interleukin-6 and anti-SSA-autoantibody levels. Management of her blood pressure combined with corticosteroid therapy improved her neurological symptoms, including abnormal CSF and MR imaging findings.
Full Text of this Article in Japanese PDF (808K)

(CLINICA NEUROL, 63: 159|162, 2023)
key words: Sjögren's syndrome, posterior reversible encephalopathy syndrome, anti-SSA-autoantibody, interleukin-6

(Received: 13-Sep-22)