Quattrini A, Nemni R, Sferrazza B, Ricevuti G, Dell'Antonio G, Lazzerini A, Iannaccone S
Department of Neurology, San Raffaele Scientific Institute, University of Milan, Italy.
Neurology. 1998 Aug;51(2):600-2. doi: 10.1212/wnl.51.2.600.
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.
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。
