Hirayama disease

Neuroimaging Clin N Am. 2011 Nov;21(4):939-50, ix-x. doi: 10.1016/j.nic.2011.07.009.


Hirayama disease (juvenile muscular atrophy of distal upper extremity) is a cervical myelopathy. Predominantly affecting male adolescents, it is characterized by progressive muscular weakness and atrophy of distal upper limbs, followed by spontaneous arrest within several years. Although the cause of cervical myelopathy remains unclear, neuropathologic and neuroradiologic findings suggest a forward displacement of the posterior cervical dural sac during neck flexion, causing compression of the cervical cord, and results in atrophic and ischemic changes in the anterior horn. A good understanding of Hirayama disease is essential because early recognition and management can effectively halt the progressive deterioration.

Publication types

  • Review

MeSH terms

  • Cervical Vertebrae / pathology
  • Humans
  • Magnetic Resonance Imaging
  • Neuroimaging*
  • Predictive Value of Tests
  • Sensitivity and Specificity
  • Spinal Cord / pathology
  • Spinal Muscular Atrophies of Childhood / diagnosis*
  • Spinal Muscular Atrophies of Childhood / physiopathology
  • Spinal Muscular Atrophies of Childhood / therapy

Supplementary concepts

  • Amyotrophy, monomelic