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家族性痉挛性截瘫(Familial SpasticParap—legia FSP)又名遗传性痉挛性截瘫和 Strümpellc—Lorrain 氏病,临床上以缓慢进展的双下肢痉挛性无力为主要表现。病理上以双侧皮质脊髓束、后索脱髓鞘、变性改变为特征。从1880年 Strilmpellc 首次报告这种病例以来,国内外均陆续有人报道。遗传方式文献上报告的有常染色体显性、隐性及 X-伴性隐性遗传三种。本文报告一种新的遗传方式,三代中均为男性发病,连续两代出现。表现为常染色体显性—从性遗传。病例摘要例一,先证者,男性,4岁6个月。因
Familial spastic paraplegia (Familial Spastic Parap-legia FSP), also known as hereditary spastic paraplegia and Strümpellc-Lorrain’s disease, clinically slow progress in the lower limb spastic weakness as the main performance. Pathology to the bilateral corticospinal tract, after demyelination, degeneration change is characterized. Since Strilmpellc first reported this case in 1880, one after another reported at home and abroad. Genetic methods reported in the literature are autosomal dominant, recessive and X-associated recessive three. This article reports a new genetic pattern that occurs in both men and women for three generations, two generations in a row. The performance of autosomal dominant - from the genetic. Case Summary First, the proband, male, 4 years and 6 months. because