良性局限性下肢肌萎缩

良性局限性下肢肌萎缩是一隐匿起病,缓慢进展,继之以3年以上稳定期的局限性肌萎缩疾病。该病肌萎缩与肌无力不成比例,无感觉障碍,锥体束损害和延髓麻痹。病例都呈散发性,无脊髓灰质炎病史。患者血肌酸磷酸激酶正常,抗神经节苷脂抗体阴性,肌电图上运动和感觉传导速度正常。肌电图和肌活检检查除萎缩肌肉外,在其他肢体上也可见到广泛前角细胞损害。肌肉CT显示肌萎缩呈不对称性,主要或选择性损害下肢后组肌群和股二头肌长头。该病是脊肌萎缩症的一种变异类型,预后良好。

Benign Monomelic Amyotrophy of Lower Limb

Patients of benign monomelic amyotrophy of lower limb presented with amyotrophy confined to a single lower limb and characterized to be insidious onset,slow progression and later stabilization at least 3 years. Wasting was out of proportion with disability and there were no sensory,pyramidal tract or bulbar signs. All cases were sporadic,and there was no history of poliomyelitis. CK,anti-ganglioside antibodies,motor and sensory conductions were normal. Quantitative EMG and muscle biopsy revealed neurogenic features also in clinically unaffected limbs. Muscular CT showed selective or predominant,asymmetrical involvement of posterior leg muscles and caput longus of biceps femoris. Monomelic amyotrophy of lower limb is a clinically localized variant of spinal muscula,atrophy with a particularly benign course.