Acute sensory neuronopathy as the presenting symptom of Sjögren’s syndrome

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• 作者：Nizar Souayah ; Peter Siao Tick Chong ; Didier Cros
• 关键词：Neuronopathy ; Sjö ; gren’ ; s syndrome ; Ataxia ; Sensory neuropathy ; Acute ganglionitis
• 刊名：Journal of Clinical Neuroscience
• 出版年：2006
• 出版时间：October 2006
• 年：2006
• 卷：13
• 期：8
• 页码：862-865
• 全文大小：76 K

文摘

Sensory neuronopathy associated with Sjögren’s syndrome (SS) usually has a subacute or chronic onset. We report the case of a 37-year-old woman who presented with an unusual hyperacute form of SS ganglionopathy. She initially developed paresthesias of her fingertips and rapidly became severely ataxic. Nerve conduction studies revealed abnormal sensory but normal motor functions. Lip biopsy showed findings consistent with SS. Sural nerve biopsy showed severe axonal loss. The patient showed modest improvement with immunosuppressive therapies.