Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease.
10.14245/kjs.2016.13.3.170
- Author:
Kyoung Hee LEE
1
;
Dae Seob CHOI
;
Young Suk LEE
;
Dong Ho KANG
Author Information
1. Department of Neurosurgery, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea. ns4793@hanmail.net
- Publication Type:Case Report
- Keywords:
Hirayama disease;
Juvenile spinal muscular atrophy;
Myelopathy
- MeSH:
Anterior Horn Cells;
Atrophy;
Cervical Cord;
Humans;
Magnetic Resonance Imaging;
Male;
Motor Neuron Disease*;
Motor Neurons*;
Neck;
Rare Diseases;
Spinal Cord Diseases;
Spinal Muscular Atrophies of Childhood;
Upper Extremity
- From:Korean Journal of Spine
2016;13(3):170-172
- CountryRepublic of Korea
- Language:English
-
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.
