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模拟面肩肱型和肢带型肌营养不良的慢性脊髓性肌萎缩。两例报告。

Chronic spinal muscular atrophy simulating facioscapulohumeral type and limb-girdle type of muscular dystrophy. Report of two cases.

作者信息

Kazakov V M, Kovalenko T M, Skorometz A A, Mikhailov E P

出版信息

Eur Neurol. 1977;16(1-6):90-8. doi: 10.1159/000114885.

Abstract

Two cases of chronic spinal muscular atrophy simulating the clinical picture of the facioscapulohumeral type and limb-girdle type of muscular dystrophy are reported. Both patients had a waddling gait, Gowers' maneuver in arising, terminal atrophies and pseudohypertrophies of some muscles, marked fasciculations, and fascicular tremor. The electromyogram revealed signs of anterior horn cell disease. Calf muscle biopsy (case 2) revealed 'myopathic' changes.

摘要

报告了两例慢性脊髓性肌萎缩症病例,其临床表现类似面肩肱型和肢带型肌营养不良症。两名患者均有蹒跚步态,起身时出现Gowers征,部分肌肉有终末性萎缩和假性肥大,明显的肌束震颤和肌束性震颤。肌电图显示前角细胞疾病的迹象。腓肠肌活检(病例2)显示“肌病性”改变。

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