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家族性痉挛性截瘫(简称FSP),通常为常染色体显性遗传,但亦有常染色体隐性遗传和性连隐性遗传的报道。FSP 可分为“单纯”性和“复合”性二种,后者合并有其他如呐吃、眼震、共济失调等特点。病理改变为皮质脊髓束变性,轻度脊髓后索变性,但脑干很少受累,通常不累及大脑皮质和白质。本文作者研究了一个有慢性神经变性病的家族,三代人中至少有六个男性成员受累,显示性连隐性遗传的特征。本文详列同代三例临床资料,所有患者均于10岁前后发生痉挛性截瘫,且都有耳聋。三例均有视网膜和晶状体损害,其中仅一例有视力障碍。三例有姿势性震颤。病例Ⅲ-3和病例Ⅲ-15
Familial spastic paraplegia (FSP), usually autosomal dominant inheritance, but also autosomal recessive inheritance and sex associated with recessive genetic coverage. FSP can be divided into “simple” and “complex” two kinds, which combined with other such as na eat, nystagmus, ataxia and so on. Pathological changes of corticospinal tract degeneration, mild spinal cord degeneration, but little involvement of the brain stem, usually does not affect the cerebral cortex and white matter. The authors studied a family of patients with chronic neurodegenerative disease that affects at least six male members of three generations and shows the characteristics of a sex-linked recessive inheritance. This article details the same generation three cases of clinical data, all patients before and after 10 years of spastic paraplegia, and have deafness. All three cases had retinal and lens damage, of which only one had visual impairment. Three cases had postural tremor. Case III-3 and Case III-15