慢性近端型脊髓肌萎缩症又称脊髓性进行性肌萎缩一少年型;假性肌病性家族性脊髓肌萎缩;Kugeberg-welander综合征,Wohldart-Kuglberg-Welander综合征。我科于1982年5月26日收治一例,现报告如下: 患者,男,56岁。住院号26341,病理号822573。近两年来先后出现左下肢、两上肢及右下肢活动无力,伴肢体近端肌肉萎缩,常感肉跳。上述症状逐渐加重而入院诊治。家族中无同样疾病患者。检查:两侧肩胛带、腰带、臀肌、臂肌及四肢近端肌肉有不同程度萎缩。两下肢腓扬肌相对完好。四肢腱反射对称性迟钝,各种感觉均正常。肌肉压痛(-),病理反射未引出。24小时尿肌酸测定为710毫克。SGOT,SGPT Chronic proximal spinal muscular atrophy, also known as spinal muscular atrophy juvenile; pseudo myopathic familial spinal muscular atrophy; Kugeberg-welander syndrome, Wohldart-Kuglberg-Welander syndrome. My department in May 26, 1982 admitted a case, report as follows now: Patient, male, 56 years old. Hospital number 26341, pathology number 822573. In the past two years, there have been left lower extremity, upper extremity and right lower extremity activity weakness, with proximal muscle atrophy, often feeling fleshy. The above symptoms gradually increased and hospitalized. No patients with the same disease in the family. Check: on both sides of the shoulder girdle, belt, gluteal muscle, arm muscles and proximal limb muscles have varying degrees of atrophy. The lower limbs feather muscle relatively intact. Limb tendon reflex symmetry dull, all kinds of feelings are normal. Muscle tenderness (-), pathological reflex did not lead. Urine creatinine was measured at 710 mg for 24 hours. SGOT, SGPT