CAJ | 학술논문

通过分析总结青少年上肢远端肌萎缩症(平山病)的临床、神经电生理、影像学和病理学特点并结合文献复习,提高其诊断水平.分析2005年至2008年本院诊治的2例平山病患者临床资料,并进行文献复习.平山病男性多见,常于青春期发病,单或双侧上肢远端无力伴肌萎缩,无感觉障碍和锥体束征.肌电图以第4颈椎至第1胸椎支配区为中心呈神经源性异常.颈椎MRI示下颈髓轻度萎缩,屈颈位下颈髓程度不等受压扁平、前移,硬膜囊后间隙增宽,硬膜外新月形或条状高信号,部分可见血管流空影;增强扫描新月形信号影有强化.肌肉活检可呈轻度神经源性异常,部分病例正常.平山病少见,结合症状、电生理和影像学检查一般可诊断.
통과분석총결청소년상지원단기위축증(평산병)적림상、신경전생리、영상학화병이학특점병결합문헌복습,제고기진단수평.분석2005년지2008년본원진치적2례평산병환자림상자료,병진행문헌복습.평산병남성다견,상우청춘기발병,단혹쌍측상지원단무력반기위축,무감각장애화추체속정.기전도이제4경추지제1흉추지배구위중심정신경원성이상.경추MRI시하경수경도위축,굴경위하경수정도불등수압편평、전이,경막낭후간극증관,경막외신월형혹조상고신호,부분가견혈관류공영;증강소묘신월형신호영유강화.기육활검가정경도신경원성이상,부분병례정상.평산병소견,결합증상、전생리화영상학검사일반가진단.
To analyze clinical, neuro-electrophysiologocal, imaging and pathological characteristics of Hirayama disease (HD) and review its relevant literatures to improve its diagnosis. Clinical data of two HD cases admitted to Fujian Provincial Hospital, Fuzhou during 2005 to 2008 were analyzed with literatures review. HD occurred more in males, often onset at their adolescence with muscular weakness and atrophy in one or two upper limbs, but without sensory dysfunction or pyramidal signs. Neuro-electrophysiology showed neurogenic abnormality in the body areas dominated by the 4th cervical vertebra to the 1 st thoracic vertebral (C4-T1) spinal nerves in all the patients. Magnetic resonance imaging (MRI) showed slight atrophy of the lower cervical spinal cord at routine position and its compression and forward displacement to varied extent at flexion position, with posterior epidural capsular space widening, crescent or striped high signal, and voids of vessels in some patients. Enhancement magnetic resonance scanning showed crescent sign enhanced in some patients. Biopsies of the inflicted muscles appeared slight neurogenic abnormality in some cases, and normal in other cases. HD is rarely seen clinically, but it can usually be diagnosed according to its symptoms, neuro-electrophysiology and MRI.