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模拟下运动神经元病的淀粉样变性神经病

Amyloid neuropathy simulating lower motor neuron disease.

作者信息

Quattrini A, Nemni R, Sferrazza B, Ricevuti G, Dell'Antonio G, Lazzerini A, Iannaccone S

机构信息

Department of Neurology, San Raffaele Scientific Institute, University of Milan, Italy.

出版信息

Neurology. 1998 Aug;51(2):600-2. doi: 10.1212/wnl.51.2.600.

Abstract

We report a 57-year-old man with progressive symmetric weakness and fasciculation affecting the legs. Electromyography revealed fibrillations and neurogenic motor unit potentials in the leg muscles. Biopsy of a motor branch of the obturator nerve revealed axonal degeneration, loss of myelinated nerve fibers, and amyloidosis with deposits of lambda light chains. At 6-month follow-up, the patient manifested sensory and autonomic symptoms, and lambda light chains were first detected in the serum. In this case, diagnosis of amyloidosis remained elusive until motor nerve biopsy.

摘要

我们报告了一名57岁男性,其双腿出现进行性对称性无力和肌束震颤。肌电图显示腿部肌肉存在纤颤和神经源性运动单位电位。闭孔神经运动支活检显示轴索变性、有髓神经纤维丢失以及伴有λ轻链沉积的淀粉样变性。在6个月的随访中,患者出现感觉和自主神经症状,血清中首次检测到λ轻链。在本病例中,直到进行运动神经活检才明确淀粉样变性的诊断。

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