舞蹈-肌萎缩-棘红细胞增多综合征(ACCS)以成人发病、面部和声带抽搐、舌咬伤、弓形足、反射降低、神经病或伴血清CK增高的肌病为其临床表现。作者研究了患有ACCS的两同胞兄弟,并有球形细胞(SC)溶血性贫血。病例1,男,20岁起病,曾有黄疸及接受输血治疗,腿无痛性肌无力及萎缩,面部、头、颈不随意运动、皮肤黄染及桔色大便。53岁时因腿无力再次入院。体检示近端肩、腿肌无力及萎缩、双侧弓形足,无踝反射,左Babinski征、呐吃、口面抽搐、爆发性语言、不规则呼吸及头、颈、肩、手的舞蹈运动,现有声带抽搐及偶发猥亵语言,三角肌自发性收缩和手套-袜套样感觉丧失、黄疸及脾肿大,神经传导速度正常,EMG可见弥散性肌纤维自发性收缩电位,运动单位 Dance - Muscular atrophy - Acanthosis syndrome (ACCS) is characterized by adult onset, facial and vocal cord convulsions, tongue bites, arched feet, reduced reflexes, neuropathy or myopathy with elevated serum CK. The authors studied two siblings with ACCS and had spherical cell (SC) hemolytic anemia. Case 1, male, 20 years old, had jaundice and blood transfusions, painless leg weakness and atrophy, facial, head and neck involuntary movement, skin yellow dye and orange stool. 53 years old due to leg weakness again hospitalization. Physical examination showed proximal shoulder, weakness and atrophy of leg muscles, bilateral arched legs, ankle-free reflex, left Babinski sign, napping, spasmodic tingling, explosive language, irregular breathing and dance movements of the head, neck, shoulders, hands , Existing vocal convulsions and occasional obscenity, spontaneous detrusor of the deltoid muscle and loss of sensation in the glove - sock, jaundice and splenomegaly, normal nerve conduction velocity, EMG visible spontaneous contraction potential of diffuse myofibers, motor units