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遗传-家族性共济失调,又名Friedrich氏病,是因Friedrieh于1863年根据两家的6个病例详细描述了它的临床症状而得名。此病自儿童或青年时期开始发病,主要表现是行走时共济运动失调,肌张力减退,腱反射减弱或消失,出现病理反射和深部感觉障碍。临床症状的出现是以脑脊髓侧、后索的神经纤维变性为基础。在许多情况下,病变可扩展到脑干,因而出现自发性水平眼震。作者们对58家的67例遗传-家族性共济失调及其表现尚健康的亲属75人进行了神经学和耳神经学方面的检查。发病情况,在67例中散发性病例(46例)比家族性的(19例)多见。男女发病
Genetic-familial ataxia, also known as Friedrich’s disease, was named after Friedrieh’s detailed description of its clinical symptoms in 1863 on the basis of six cases in both. The disease since the onset of childhood or youth onset, the main performance is walking ataxia disorders, hypotonia, tendon reflexes diminish or disappear, pathological reflex and deep sensory disturbances. The appearance of clinical symptoms is based on the myeloproliferative and retroflexional neurofibrillary degeneration. In many cases, the lesions can spread to the brainstem, resulting in spontaneous nystagmus. The authors examined neurological and otological neurological aspects in 67 of 67 genetic-familial ataxia and their healthy relatives, 75. Incidence, in 67 cases of sporadic cases (46 cases) than the familial (19 cases) more common. Male and female incidence