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近年随着神经病学各领域的迅速发展,神经系变性疾病的病因已逐渐明瞭;免疫机能异常所致的神经肌病,在治疗方法上有了新的进展;正电子放射断层摄影(PET)及核磁共振已有可能用于临床,对此,本文略作简述。一、脊髓小脑变性病的代谢异常1.丙酮酸代谢及线粒体1974年Kark等在共济失调症患者的肌活检中证实丙酮酸脱氢酶复合物(PDHC)活性降低,继之Blass等用培养本病患者皮肤纤维母细胞的方法也发现了PDHC降低。1980年Kark等又发现PDHC的一部分即脂酰胺脱氢酶(LAD)的活性,在杂合子Friedreich氏病患者双亲上介于患者和正常人的中间值,由此可诊断本病之遗传病因携带者。但并不是全部Fri-
In recent years, with the rapid development in various fields of neurology, the etiology of degenerative diseases of the nervous system has become clear. Neonomyopathy caused by abnormal immune function has made new progress in the treatment. Positron emission tomography (PET) and Nuclear magnetic resonance has been possible for clinical use, for which we briefly describe this article. First, the metabolic abnormalities of spinocerebellar degeneration 1. Pyruvate metabolism and mitochondria Kark et al in 1974, ataxic muscle biopsy confirmed pyruvate dehydrogenase complex (PDHC) activity decreased, followed by Blass and other culture Methods of this patient’s skin fibroblasts have also been found to reduce PDHC. In 1980, Kark et al. Found that the activity of fatty acid amide dehydrogenase (LAD), a part of PDHC, was found to be intermediate between patient and normal in the parents of heterozygous Friedreich’s disease, thus diagnosing the genetic cause of the disease By. But not all Fri-