Poglio Fabio, Mongini Tiziana, Cocito Dario
Dipartimento di Neuroscienze, Università di Torino, Via Cherasco 15, 10126 Torino, Italy.
Muscle Nerve. 2007 Apr;35(4):532-5. doi: 10.1002/mus.20716.
We describe a patient who developed an ataxic sensory syndrome associated with xerophthalmia and progressive dysphagia with regurgitation. Electrophysiological findings were consistent with an axonal sensory neuropathy, and superficial peroneal nerve biopsy showed a reduction in number of myelinated fibers with epineurial inflammation. Rheumatoid factor, anti-SSA/SSB and antinuclear antibodies were positive and a diagnosis of Sjogren's syndrome was made. An endoscopic investigation revealed esophageal achalasia. We suggest that there may be a common autoimmune mechanism directed to different targets on the basis of this rare association.
我们描述了一名患者,其出现了与干眼症、进行性吞咽困难伴反流相关的共济失调性感觉综合征。电生理检查结果与轴索性感觉神经病相符,腓浅神经活检显示有髓纤维数量减少并伴有神经外膜炎症。类风湿因子、抗SSA/SSB及抗核抗体均呈阳性,遂诊断为干燥综合征。内镜检查发现食管贲门失弛缓症。基于这种罕见的关联,我们认为可能存在针对不同靶点的共同自身免疫机制。