Magnetic Resonance Imaging Findings of Hirayama’s Disease
KYK Tang, CY Chu, KW Leung, JSF Shum, SSW Lo, CS Cheng, JLS Khoo
Hong Kong J Radiol 2012;15:110-2
Hirayama’s disease is a rare form of cervical myelopathy resulting in juvenile muscular atrophy of the upper limb. We describe the magnetic resonance imaging findings of Hirayama’s disease in a 20-year-old male, who demonstrates abnormal detachment and forward displacement of the posterior cervical dura during neck flexion, with the lower cervical cord abutting on the posterior border of the lower cervical vertebral bodies. This compression causes ischaemic changes in the lower cervical cord and hence the subsequent denervation and atrophy of the distal upper limb muscles. A high index of clinical suspicion and magnetic resonance imaging of the cervical spine with neck flexion are the key to diagnosis.
中文摘要
平山病的磁共振成像表現
鄧業勤、朱志揚、梁錦榮、岑承輝、盧成瑋、鄭志成、邱麗珊
平山病是一種罕有的頸髓病，病者一般會在青少年時期出現上肢肌萎縮。本文報告一名20歲平山病 男性青年患者的磁共振成像。當他頸部向前屈曲時，頸椎後硬膜出現異常的分離及向前移位，而下 頸髓則靠近下頸椎椎體的後緣。這壓迫現象使下頸髓出現缺血性改變，繼而引致上肢肌神經性萎 縮。診斷此症的關鍵是要對它的臨床症狀有高度警覺，並留意病人頸椎前屈時之磁共振成像表現。