Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease
Unusual association of sporadic olivopontocerebellar atrophy and motor neuron disease
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DOI：
10.1007/s100720200049
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发表时间：
2002-12
影响因子：
3.3
通讯作者：
D. Testa;V. Tiranti;F. Girotti
中科院分区：
文献类型：
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作者：
D. Testa;V. Tiranti;F. Girotti
散发性橄榄脑桥小脑萎缩 (OPCA) 是一种临床表现广泛的神经退行性疾病。运动神经元疾病（MND）的特征是运动神经元的选择性变性。一名60岁男子出现言语不清、步态不稳的症状。他还注意到难以保持头部直立和肩膀无力。疾病进展迅速。 63 岁时，磁共振成像支持 OPCA 的诊断，临床和电生理学检查结果提示 MND 的诊断。他还患有向上凝视麻痹症。肌肉活检显示零星的参差不齐的红色纤维和 Cox 缺陷纤维。本病例可以定义一种独特的疾病，因为两种退行性疾病偶尔发生的可能性似乎不大。
Sporadic olivopontocerebellar atrophy (OPCA) is a neurodegenerative disorder that presents a wide clinical spectrum. Motor neuron disease (MND) is characterized by a selective degeneration of motor neurons. A 60-year-old man developed slurred speech and unsteadiness of gait. He had also noticed difficulty in holding his head upright and shoulder weakness. The disease had a rapid progression. At the age of 63 years, magnetic resonance imaging supported a diagnosis of OPCA, and a diagnosis of MND was suggested by clinical and electrophysiological findings. He also had upward gaze palsy. A muscular biopsy showed sporadic ragged red and Cox deficient fibers. The present case could define a unique disorder, as the occasional occurrence of two degenerative disorders appears unlikely.