Senior Resident.
Postgraduate Resident.
J Assoc Physicians India. 2023 Mar;71(3):11-12.
Hirayama disease, or brachial monomelic amyotrophy, is not a common neurological disease characterized by unilateral or asymmetric bilateral lower motor weakness of distal upper limbs. The basic pathophysiology is compression of the dural sac and spinal cord during flexion of the neck. A case of a 21-year-old male presented with chief complaints of tremors in both hands (right more than left) with gradually progressive weakness of the right hand and forearm. Electromyography (EMG), nerve conduction velocity (NCV), and magnetic resonance imaging (MRI) neck in flexion showed focal atrophy of lower cervical myotomes and confirmed the diagnosis of monomelic amyotrophy.
平山病，又称臂丛下运动神经元病，是一种不常见的神经系统疾病，其特征为单侧或不对称性双侧上肢远端下运动神经元无力。其基本病理生理学是在颈部弯曲时硬脑膜囊和脊髓受压。一位 21 岁男性，主要表现为双手震颤（右侧重于左侧），右手及前臂进行性无力，来诊。肌电图（EMG）、神经传导速度（NCV）和颈椎弯曲磁共振成像（MRI）显示下颈段肌节局限性萎缩，确诊为单肢运动神经元病。
