头颅下垂综合征和轴性肌病

Dropped Head Syndrome and Axial Myopathy

头颅下垂综合征指患者站立时颈脊部伸肌群无力抬头,因而头颅下垂,背部弯曲。平卧时可缓解。该综合征由原发性和继发性的病因造成。原发性的轴性肌病,病因不明,十分罕见。这种肌病发生于40￣60岁,主要累及颈脊旁肌群,出现头颅下垂。脊旁肌肌电图和组织活检证实为一种慢性肌病。本病是一种应引人注意的临床肌病症状群之一。

Dropped head syndrome refers to severe weakness of neck extension with or without weakness of neck flexion. Such syndrome may occur in primary reason and in association with other disorders. Axial myopathy is an unrecognised entity and a rare muscular disorder. Such myopathy appeared within an age range of 40 to 60 years. The First synptoms were difficulty in keeping the head and trunk in a upright position. Both bent spine and dropeped head were reduced in a supine position. EMG and muscle biopsy specimens obtained from spinal muscles showed chronic myopathic pattern.