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本文报告3例脊髓小脑变性。例1临床观察10年,主要表现为共济失调,伴眼肌麻痹和锥体外型肌张力过高,尸检证实中脑动眼神经核及黑质有病变,提示共济失调症与巴金森氏症有密切关系。例2临床上为典型的遗传性小脑共济失调,病理上脊髓后索有脱鞘病变。例3临床上为典型Friedreich型共济失调;病理上亦见小脑、腰骶神经病变,提示与Charcot-Marje-Tooth神经型肌萎缩有关,病变部位见到淋巴细胞浸润或可提示慢性感染和(或)免疫反应。
This article reports 3 cases of spinocerebellar degeneration. Example 1 clinical observation of 10 years, mainly as ataxia, with ophthalmoplegia and extrapyramidal muscle hypertrophy, autopsy confirmed midbrain nerve nucleus and substantia nigra have lesions, suggesting that ataxia and Parkinson’s disease Disease is closely related. Example 2 is clinically a typical hereditary cerebellar ataxia, pathological dehiscence after spinal cord sheath lesions. Case 3 is a typical Friedreich ataxia pathology; pathology also see the cerebellum, lumbosacral neuropathy, suggesting that Charcot-Marje-Tooth neuromuscular atrophy related to the lesion see lymphocyte infiltration or can prompt chronic infection and ( Or) immune response.