Kieser David C, Cox P J, Kieser S C J
Department of Orthopaedic Surgery and Musculoskeletal Medicine, Canterbury School of Medicine, Canterbury District Health Board, University of Otago, 2 Riccarton Avenue, Christchurch, 8011, New Zealand.
Division of Neuroradiology, University of Oxford, Oxford, UK.
Eur Spine J. 2018 Jun;27(6):1201-1206. doi: 10.1007/s00586-018-5545-9. Epub 2018 Mar 14.
Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. It is primarily seen in young males of Indian or Asian descent. With increasing dispersion of these populations this condition is increasingly being encountered internationally. This grand round reviews this rare but increasingly recognized condition.
We present a classic case of a young Indian male with progressive hand and forearm weakness. We discuss the typical clinical presentation, appropriate investigations and management of this condition.
Our patient presented with oblique amyotrophy and underwent a diagnostic flexion MRI scan which revealed anterior translation of the posterior dura with compression of the anterior horns of the lower cervical cord. He has been successfully treated in a cervical collar.
This case illustrates the typical presentation, diagnostic investigations and treatment of Hirayama syndrome. It is hoped that this review will alert clinicians of this condition and optimize the management of affected individuals.
平山病是一种最初呈进行性发展的疾病，由颈部前屈压迫下颈段脊髓前角所致。该病主要见于印度或亚洲血统的年轻男性。随着这些人群分布范围的扩大，国际上越来越多地遇到这种疾病。本次临床病例讨论会对这种罕见但日益受到认可的疾病进行综述。
我们展示了一名患有进行性手部和前臂无力的年轻印度男性的典型病例。我们讨论了该病的典型临床表现、适当的检查及治疗方法。
我们的患者表现为斜方肌萎缩，并接受了诊断性前屈磁共振成像扫描，结果显示硬脊膜后移伴下颈段脊髓前角受压。他通过佩戴颈托已成功治愈。
本病例说明了平山综合征的典型表现、诊断性检查及治疗方法。希望本次综述能提醒临床医生注意这种疾病，并优化对患者的管理。
