Agarwal Arjit, Chandak Shruti, Joon Pawan
Assistant Professor, Department of Radiodiagnosis, Teerthanker Mahaveer Medical College and Research Centre, Teerthanker Mahaveer University , Moradabad,(U.P.), India .
Assistant Professor, Department of Radiodiagnosis, Teerthanker Mahaveer Medical College and Research Centre , Teerthanker Mahaveer University, Moradabad,(U.P.), India .
J Clin Diagn Res. 2015 Aug;9(8):TD10-1. doi: 10.7860/JCDR/2015/13148.6374. Epub 2015 Aug 1.
Hirayama disease is a rare type of neurological disease commonly manifesting as brachial monomelic amyotrophy in young males of Asian origin, easily understood as juvenile non-progressive cervical amyotrophy. The first case was reported by Hirayama in 1959. The pathogenesis is attributed towards chronic compression of cervical spinal cord during flexion movements of neck in cases where there is detachment of posterior dura mater. This chronic event, invariably leads to features of cord atrophy along with other MRI features. We report a case of 21-year-old male who presented with atrophy of distal muscles of his right hand and was sent for MRI of cervical spine which revealed prominent posterior epidural venous plexus without significant cord atrophy. Clinico-radiologic profile of the patient leads toward the diagnosis of Hirayama disease which was considered as borderline because of asymmetrical cord atrophy which is a not a routine imaging feature of the entity.
平山病是一种罕见的神经疾病，常见于亚洲裔年轻男性，表现为臂丛单肢肌萎缩，可简单理解为青少年非进行性颈肌萎缩。首例病例由平山于1959年报告。其发病机制归因于后硬膜分离时颈部屈曲运动过程中颈脊髓的慢性受压。这一慢性事件总是导致脊髓萎缩以及其他MRI特征。我们报告一例21岁男性，其右手远端肌肉萎缩，接受颈椎MRI检查，结果显示硬膜外后静脉丛突出，无明显脊髓萎缩。患者的临床放射学特征提示平山病诊断，但因其不对称性脊髓萎缩（这并非该疾病的常规影像学特征）而被认为处于临界状态。
