title: Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease.
abstract: Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in few years. Herein, we report 2 cases of Hirayama disease on emphasis of diagnostic approach and describe the pathognomonic findings at flexion magnetic resonance imaging.
authors: Department of Neurosurgery, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.
authors: Kyoung Hee
authors: KH
authors: Lee
authors: Department of Radiology, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.
authors: Dae Seob
authors: DS
authors: Choi
authors: Department of Neurosurgery, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.
authors: Young Suk
authors: YS
authors: Lee
authors: Department of Neurosurgery, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.
authors: Dong Ho
authors: DH
authors: Kang
keywords: Hirayama disease; Juvenile spinal muscular atrophy; Myelopathy