平山病的致病机制、诊断与治疗进展  

Advances in pathogenesis, diagnosis and treatment of Hirayama disease

作　　者：郑超君[1] 姜建元[1] 

机构地区：[1]复旦大学附属华山医院骨科,上海200040

出　　处：《上海医药》2014年第24期3-8,共6页Shanghai Medical & Pharmaceutical Journal

基　　金：上海市科委医学重点项目:腰椎退变性疾病诊疗技术的优化选择与临床验证(12411951201);上海卫生系统第二批重要疾病联合攻关重点项目:平山病的临床规范化诊断与治疗研究(2014ZYJB0008)

摘　　要：平山病是远端上肢肌萎缩性疾病。近年来,随着平山病研究的不断深入,相关报道不断增加,颈椎屈曲位的磁共振成像和各项神经电生理检测技术亦已用于平山病的诊断和预后评估,使该病的早期诊断及早期治疗的重要性和有效性再次得到肯定及强调。因此,对于起病年龄<25岁的上肢肌肉萎缩患者,应警惕平山病的可能性,积极予以颈椎屈曲位磁共振技术及神经电生理检测。本文将就平山病的致病机制、诊断、治疗及近年的相关研究进展进行综述,以供读者对平山病这一少见疾病有进一步的认识和了解。Hirayama disease is a distalupper limb muscle atrophy disease. In recent years, with the progress of the research of the Hirayama disease, the reported number of patients with Hirayama diseases has been increasing. In addition, the magnetic resonance imaging （MRI） and electrophysiological techniques used in cervical flexion examination have been applied in the diagnosis and evaluation of prognosis of Hirayama disease. Importance of the early diagnosis and early treatment of the Hirayama disease has been affirmed and emphasized again. Therefore, it should be considered that the patients with upper limb muscle atrophy and the onset age less than 25 years old may suffer from Hirayama disease and cervical flexion examination using MRI and electrophysiological techniques should be actively performed. This paper discusses the pathogenesis, diagnosis and treatment of the Hirayama disease, as well as the related research progress in recent years so that the readers will further understand this rare disease of Hirayama disease.

关 键 词：平山病 致病机制 诊断 治疗 

分 类 号：R746.4[医药卫生—神经病学与精神病学]