Souayah Nizar, Chong Peter Siao Tick, Cros Didier
Neurology Department, New Jersey Medical School, Newark, NJ 07101, USA.
J Clin Neurosci. 2006 Oct;13(8):862-5. doi: 10.1016/j.jocn.2005.12.029. Epub 2006 Aug 28.
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.
与干燥综合征（SS）相关的感觉神经元病通常呈亚急性或慢性起病。我们报告一例37岁女性患者，其表现为一种不寻常的超急性形式的SS神经节病。她最初出现指尖感觉异常，并迅速发展为严重共济失调。神经传导研究显示感觉功能异常但运动功能正常。唇腺活检结果符合SS。腓肠神经活检显示严重轴突丢失。该患者经免疫抑制治疗后有一定程度的改善。
