多节段受累平山病的临床、电生理与影像学特点分析  

Clinical,electrophysiological and imaging features of Hirayama disease with involvement of multiple segments

作　　者：沈东超[1] 方佳[1] 崔丽英[1,2] 管宇宙 丁青云[1] 吴双[1] 杜华[1] 李本红[1] SHEN Dong-chao FANG Jia CUI Li-ying et al(Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Belting 100730, China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院神经科学中心,100730

出　　处：《临床神经病学杂志》2016年第6期405-407,共3页Journal of Clinical Neurology

摘　　要：目的总结EMG呈多节段受累平山病患者的临床、电生理与影像学特点,分析多节段受累的相关因素。方法 2006年4月至2015年11月于我院经临床和EMG检查诊断为平山病者共82例,其中15例EMG呈多节段受累,对其EMG表现和相关因素进行分析。结果 15例(18.3%)多节段受累的平山病患者均为男性,青少年起病,其中7例(8.5%)为广泛神经源性损害。除颈段外,最常累及的节段为延髓段(13例,86.7%),其次为腰骶段(7例,46.7%),累及胸段者最少(5例,33.3%)。多节段受累患者的临床、电生理及影像学资料与仅有上肢受累患者相比无显著性差异。未发现与多节段受累相关的因素。对部分患者随访后发现其预后良好。结论平山病患者可以出现EMG多节段受累,但其机制尚不明确。Objective To summarize the clinical , electrophysiological and imaging features of Hirayama disease patients with involvement of multiple segments on EMG and to explore the relevant factors of multiple segments . Methods Eighty-two patients were diagnosed with Hirayama disease based on clinical manifestation and EMG findings collected from April 2006 to October 2015, of which 15 patients were identified with multiple segments involvement.Their data and factors affecting EMG manifestation were analyzed .Results Fifteen patients （18.3%） were found to have multiple segments involvement , seven （ 8.5%） of which with diffuse neurogenic damages . Besides the cervical segment , the most frequently involved part was medullary segment （13 cases, 86.7%）, followed by lumbosacral （7 cases, 46.7%） and thoracic segment （5 cases, 33.3%）.The clinical, electrophysiological and imaging features showed no significant difference between patients with involvement of multiple segments and only upper limbs .No factor was found to be related with multiple segments involvement .Follow-up of part of these patients revealed that their outcome were favorable .Conclusion Patients with Hirayama disease could present multiple segments involvement on EMG , though the pathogenic mechanism remains unclear .

关 键 词：平山病 临床特点 多节段 

分 类 号：R744[医药卫生—神经病学与精神病学]