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家族性对称性痉挛性麻痹。由Seeligmuller最早报告,以后有同样病例报告及伴有视网膜变性、白内障、精神迟钝、末梢神经障碍等,但伴有肌萎缩尤其是双上肢肌萎缩的报告极少。本文报告兄弟两人同患家族性对称性痉挛性麻痹伴双手肌萎缩。例一男性,18岁,足月平产,1岁走路,幼儿期发育无特殊,12岁时行走有轻度下肢强直感,以后未再加剧,至17岁右手大鱼际肌萎缩,手指精细动作有困难,约3个月后出现左手肌肉萎缩、但无感觉、排尿障碍、于18岁时入院。检查一般情况可、智能可、未见白内障及视网膜病变、有痉挛步态,下肢轻度对称性痉挛性麻痹,有明显的双手大小鱼际肌及骨间肌萎缩、四肢肌力可,腱反射上肢正常,下肢亢进、双侧巴氏征及查都克氏征阳性,无小脑受损症状。血、尿、粪常规、血清、血生化、心电、肌电、CT等均无特殊。脊髓造影术(MLG):颈髓及胸髓轻
Familial Symmetrical Spastic Paralysis. The earliest reports by Seeligmuller, followed by the same case reports and associated with retinal degeneration, cataracts, mental retardation, peripheral neuropathy, etc., but with atrophy, especially in the upper extremity muscular atrophy report very small. This article reports the two brothers with familial symmetrical spastic paralysis with hands muscle atrophy. Example A man, 18 years old, full-term pregnancy, 1 year old walking, early childhood development is no special, 12-year-old walking mild leg sensation, and no further aggravate, to 17 years old right a large intertransverse muscle atrophy, finger fine Movement difficulties, about 3 months after left muscle atrophy, but no feeling, voiding disorders, at 18 years of age admitted to hospital. Check the general situation can be, can be smart, no cataracts and retinopathy, spasmodic gait, lower extremity mild symmetrical spastic paralysis, there are obvious hands-sized muscle and intertrochanteric muscle atrophy, muscle strength of the limbs, tendon reflex Upper extremity normal, lower extremity hyperthyroidism, bilateral Pakistan’s sign and check Duke’s sign positive, no symptoms of cerebellar damage. Blood, urine, fecal routine, serum, blood biochemistry, ECG, EMG, CT and so no special. Myelography (MLG): cervical and thoracic light