一過性の神経症候を呈した多発脳動脈狭窄を伴ったシェーグレン症候群の1例

A Case of Sjögren's Syndrome with Multiple Stenoses of the Cerebral Arteries and Transient Neurological Symptoms and Signs

症例は31歳女性，一過性の手指のしびれ感，構音障害を数回生じた。血液検査でアミラーゼ高値，血沈亢進，免疫グロブリンG高値，抗SS-A抗体陽性，脳脊髄液検査でわずかな細胞数増多と蛋白増加を認めた。MRI拡散強調画像で左基底核に高信号病変がみられ，MRAで多発脳動脈狭窄が疑われた。唾液腺造影，口唇唾液腺生検の結果，シェーグレン症候群（SjS）と診断された。これらの結果から，一過性脳虚血発作を思わせる一過性の神経症候，脳動脈狭窄とSjSに関連があることが推測された。

Abstract

A 31-year-old woman was admitted to the hospital after several episodes of transient numbness in her fingers and dysarthria. No neurological symptoms and signs were observed in this patient on admission; however, blood tests revealed elevated amylase and immunoglobulin G levels, an increased erythrocyte sedimentation rate, and positive anti-Sjögren's-syndrome (SjS)-related antigen A antibodies. The cerebrospinal fluid examination revealed a slight increase in the cell count and protein content. Diffusion-weighted and fluid-attenuated inversion recovery images on magnetic resonance imaging showed high-signal-intensity lesions in the cerebral white matter and basal ganglia supplied by the left middle cerebral artery. A part of the lesions was enhanced by the administration of gadolinium contrast medium. Magnetic resonance angiography suggested stenosis in the left anterior, middle, and posterior cerebral arteries. The patient was thus diagnosed with acute cerebral infarction and was then treated with argatroban, edaravone, and clopidogrel. Furthermore, Schirmer's test, sialography, and salivary gland biopsy results led to the diagnosis of SjS. A nerve conduction study suggested concomitant mild polyneuritis or mononeuritis multiplex. Collectively, we speculate that cerebral arterial stenosis associated with SjS leads to transient ischemic attack-like episodes, transient neurological symptoms and signs, and cerebral infarction.

(Received June 13, 2018; Accepted September 11, 2018; Published December 1, 2018)