遗传性近端脊肌萎缩症为遗传性运动神经元病的一种，在临床上较为罕见，故将我科收治的一病例报道如下。患者，女性，５５岁，已婚。１９９３年１０月出现全身肌肉跳动，夜间较重。１９９５年４月下蹲时起立困难，平地行走不受影响，３个月后发现双下肢大腿肌肉萎缩，坐抬... Hereditary proximal spinal muscular atrophy is a kind of hereditary motor neuron disease, is relatively rare in clinical practice, so a case of our department reported as follows. Patient, female, 55 years old, married. Whole body muscle beating appeared in October 1993 and was heavier at night. April 1995 Squat stand up difficulties, walking flat is not affected, three months after the discovery of lower extremity thigh muscle atrophy, sitting ...