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目的明确感觉性神经元神经病(SND)的临床表现、神经电生理、颈椎核磁共振的影像学特点以及皮肤神经活检、腓肠神经活检和脊髓后索病变的病理学特征。方法分析2例胃肠道感染后死于 SND 的患者的临床与辅助检查资料,后者包括神经电生理检查、周围神经和脊髓的尸检病理,结合相关文献进行复习。结果患者独特的临床表现为早期出现共济失调,广泛分布的感觉减退和腱反射减低。电生理检查见感觉神经动作电位广泛异常,与神经纤维长度无关。SND 患者脊髓后索髓鞘脱失,腓肠神经活检可见以大有髓纤维为主的神经纤维丢失,无再生神经丛。本组2例患者颈椎磁共振检查结果正常。结论 SND 独特的临床表现与神经电生理检查特点提示周围感觉神经纤维广泛受累。脊髓后索尸检病理证实感觉神经中枢传导纤维变性脱失,支持病变位于脊髓后根神经节。SND 早期颈椎磁共振可能正常。
Objective To investigate the clinical manifestations of sensory neuropathy (SND), neuroelectrophysiology, imaging features of cervical MRI and the pathological features of skin nerve biopsy, sural nerve biopsy and spinal cord posterior lesion. Methods Clinical and laboratory examinations were performed in 2 patients who died of SND after gastrointestinal infection. The latter included neuroelectrophysiological examination, autopsy of peripheral nerve and spinal cord, and review of related literature. Results The patient’s unique clinical manifestations were early occurrence of ataxia, widespread sensory loss, and reduced tendon reflexes. Electrophysiological examination showed a wide range of sensory nerve action potential abnormalities, and nerve fiber length has nothing to do. SND patients after spinal cord demyelination, sural nerve biopsy can be seen in large myelinated nerve fibers loss, no regenerative nerve plexus. The two patients in this group cervical magnetic resonance imaging findings were normal. Conclusion The unique clinical manifestations and electrophysiological findings of SND suggest that peripheral sensory nerve fibers are widely involved. After spinal cord autopsy pathology confirmed sensory nerve central conduction fiber degeneration loss, support lesions in the spinal cord posterior ganglion. Early SND cervical magnetic resonance may be normal.