一项对3例肌萎缩侧索硬化症及类似CIDP的多发性神经病变患者的研究  

A study of three patients with amyotrophic lateral sclerosis and a polyneuropathy resembling CIDP

作　　者：Echaniz-Laguna A. Degos B. MohrM. 牛亚利 

机构地区：[1]Départment de Neurologie,Hpital Civil de Strasbourg, 1 Place de l'Hpital, 67091 Strasbourg, France Dr.

出　　处：《世界核心医学期刊文摘（神经病学分册）》2006年第6期50-51,共2页Digest of the World Core Medical Journals:Clinical Neurology

摘　　要：We report three patients with a syndrome that fulfilled clinical and laboratory criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed immunosuppressive treatment and eventually developed progressive amyotrophic lateral sclerosis (ALS). Mean disease duration was 23 months (13-38) before death. Two patients had a family history of ALS without mutations of the SOD1 gene. Postmortem examination in one patient showed an endoneurial infiltration of mononuclear cells in lumbar roots and distal and proximal peripheral nerves, mainly around myelinated fibers, with demyelination and axonal loss, consistent with CIDP. The spinal cord revealed severe neuronal loss in the anterior horn, axonal loss in the corticospinal tract, and large numbers of phagocytes in the anterior and lateral tracts, indicative of ALS. Whether demyelinati ng polyneuropathy was coincident with ALS or was a cause or consequence of motor neuron degeneration in these patients remains to be elucidated. This unusual co mbination may provide an important clue in elucidating the pathogenesis of ALS i n some patients.We report three patients with a syndrome that fulfilled clinical and laboratory criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy （CIDP） who failed immunosuppressive treatment and eventually developed progressive amyotrophic lateral sclerosis （ALS） Mean disease duration was 23 months （13-38） before death. Two patients had a family history of ALS without mutations of the SOD1 gene. Postmortem examination in one patient showed an endoneurial infiltration of mononuclear cells in lumbar roots and distal and proximal peripheral nerves, mainly around myelinated fibers, with demyelination and axonal loss, consistent with CIDP. The spinal cord revealed severe neuronal loss in the anterior horn, axonal loss in the corticospinal tract, and large numbers of phagocytes in the anterior and lateral tracts, indicative of ALS. Whether demyelinating polyneuropathy was coincident with ALS or was a cause or consequence of motor neuron degeneration in these patients remains to be elucidated. This unusual combination may provide an important clue in elucidating the pathogenesis of ALS in some

关 键 词：多发性神经病变 肌萎缩侧索硬化症 CIDP 患者 运动神经元变性 脱髓鞘性 髓鞘神经纤维 皮质脊髓 同时发生 实验室诊断 

分 类 号：R59[医药卫生—内科学] R746.4[医药卫生—临床医学]