Korean J Spine.  2016 Sep;13(3):170-172. 10.14245/kjs.2016.13.3.170.

Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease

Affiliations
  • 1Department of Neurosurgery, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea. ns4793@hanmail.net
  • 2Department of Radiology, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.

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.

Keyword

Hirayama disease; Juvenile spinal muscular atrophy; Myelopathy

MeSH Terms

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
Full Text Links
  • KJS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
    DB Error: unknown error