Concomitant Unilateral Hirayama Disease and Contralateral Disc Protrusion of the Cervical Spine
WCS Chan, JPK Tsang, MK Yuen
Hong Kong J Radiol 2015;18:e7-11
DOI: 10.12809/hkjr1514285
Hirayama disease is a type of cervical myelopathy affecting young men and causing wasting of the small muscles of the hand. The underlying pathophysiology is thought to be due to disproportionate growth of the vertebral column and the spinal canal resulting in a tight dural sac. The presentation of Hirayama disease is usually that of muscular atrophy with no sensory symptoms. Hirayama disease is not progressive. Magnetic resonance imaging with flexion views is required to make the diagnosis, and demonstrates the distended epidural venous plexus that results from the disproportionate growth and compresses on the spinal canal. The distended venous plexus can be unilateral or bilateral, but is usually unilateral. The underlying cause is unknown, and it is not known why Hirayama disease is usually unilateral. This report presents a patient with a magnetic resonance diagnosis of Hirayama disease with atypical clinical features. Magnetic resonance imaging with flexion views confirmed unilateral Hirayama disease and concomitant contralateral disc protrusion. We propose that these two findings may be inter-related.
中文摘要
單側平山病併發對側頸椎椎間盤突出症
陳煥章、曾佩琪、袁銘強
平山病是一種頸部脊髓病變，會造成手部小肌肉萎縮；多好發於年輕男性。平山病的發病機制認為與脊柱和椎管之間的發育不平衡並壓迫硬脊膜有關。平山病不會惡化，典型的表現為肌肉萎縮，一般沒有疼痛等的感覺障礙表現。診斷用屈曲位磁共振成像（MRI），表現為因發育不均衡而導致脊硬膜壁被拉直而壓迫脊椎。擴張的靜脈叢多為單側，雖然也可累及雙側。平山病的致病機制仍不甚明確，亦不清楚為何單側受累多見。本文報告一名呈現非典型臨床症狀的平山病患者的MRI診斷。屈曲位MRI證實為單側平山病，並伴隨對側頸椎椎間盤突出。這兩個診斷表現可能互相關聯。