Hirayama disease

Yen Lin Huang, Chi-Jen Chen

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)939-950
Number of pages12
JournalNeuroimaging Clinics of North America
Volume21
Issue number4
DOIs
Publication statusPublished - Nov 2011

Fingerprint

Spinal Cord Diseases
Upper Extremity
Spinal Muscular Atrophies of Childhood
Spinal Muscular Atrophy
Muscle Weakness
Horns
Neck
Monomelic amyotrophy
Cervical Cord

Keywords

  • Adolescent
  • Cervical myelopathy
  • Hirayama disease
  • Neck flexion

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

Cite this

Hirayama disease. / Huang, Yen Lin; Chen, Chi-Jen.

In: Neuroimaging Clinics of North America, Vol. 21, No. 4, 11.2011, p. 939-950.

Research output: Contribution to journalArticle

Huang, Yen Lin ; Chen, Chi-Jen. / Hirayama disease. In: Neuroimaging Clinics of North America. 2011 ; Vol. 21, No. 4. pp. 939-950.
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