Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Diseaseopen access
- Authors
- 이경희; 최대섭; 이영석; 강동호
- Issue Date
- Sep-2016
- Publisher
- 대한척추신경외과학회
- Keywords
- Hirayama disease; Juvenile spinal muscular atrophy; Myelopathy
- Citation
- Neurospine, v.13, no.3, pp 170 - 172
- Pages
- 3
- Journal Title
- Neurospine
- Volume
- 13
- Number
- 3
- Start Page
- 170
- End Page
- 172
- URI
- https://scholarworks.bwise.kr/cau/handle/2019.sw.cau/71968
- DOI
- 10.14245/kjs.2016.13.3.170
- ISSN
- 2586-6583
2586-6591
- 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.
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