CIDP不同的临床亚型开始被认识，就如同过去对格林巴利综合征不同类型的认识过程。但这些类型的病理机制和特殊治疗方案并没有被确立。末端潜伏期指数（terminal latency index）和调整F率（modified F ratio）等神经生理技术有助于CIDP...CIDP不同的临床亚型开始被认识，就如同过去对格林巴利综合征不同类型的认识过程。但这些类型的病理机制和特殊治疗方案并没有被确立。末端潜伏期指数（terminal latency index）和调整F率（modified F ratio）等神经生理技术有助于CIDP与IgM副蛋白血症周围神经病鉴别诊断，磁共振显示神经根及神经丛变粗，明显强化，对诊断有帮助。研究发现一小部分患者有致病性抗体存在，这使对本病的机制有进一步认识。免疫组织化学研究检测神经活检中粘附因子、共同激活因子和抗原呈递分子，显示了神经中T细胞激活的启动和持续过程，雪旺细胞具有抗原呈递细胞必须的标记物。最近的临床研究肯定了静脉免疫球蛋白和强的松的短期治疗效果。展开更多
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 eventua...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.展开更多
文摘CIDP不同的临床亚型开始被认识，就如同过去对格林巴利综合征不同类型的认识过程。但这些类型的病理机制和特殊治疗方案并没有被确立。末端潜伏期指数（terminal latency index）和调整F率（modified F ratio）等神经生理技术有助于CIDP与IgM副蛋白血症周围神经病鉴别诊断，磁共振显示神经根及神经丛变粗，明显强化，对诊断有帮助。研究发现一小部分患者有致病性抗体存在，这使对本病的机制有进一步认识。免疫组织化学研究检测神经活检中粘附因子、共同激活因子和抗原呈递分子，显示了神经中T细胞激活的启动和持续过程，雪旺细胞具有抗原呈递细胞必须的标记物。最近的临床研究肯定了静脉免疫球蛋白和强的松的短期治疗效果。
文摘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.
