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Hirayama disease.

David C Kieser1, P J Cox2, S C J Kieser3

  • 1Department of Orthopaedic Surgery and Musculoskeletal Medicine, Canterbury School of Medicine, Canterbury District Health Board, University of Otago, 2 Riccarton Avenue, Christchurch, 8011, New Zealand. kieserdavid@gmail.com.

European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
|March 16, 2018
PubMed
Summary
This summary is machine-generated.

Hirayama disease, a rare condition causing progressive weakness, results from cervical spinal cord compression during neck flexion. Early diagnosis and cervical collar treatment are key for managing this condition in young males.

Keywords:
Asymmetric segmental spinal muscular atrophyAtrophyHirayamaJuvenile muscular atrophyJuvenile spinal muscular atrophyMonomelic amyotrophyOblique amyotrophy

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Area of Science:

  • Neurology
  • Rare Diseases
  • Spinal Cord Disorders

Background:

  • Hirayama disease is a rare, progressive neurological disorder.
  • It affects young males, often of Asian descent, causing hand and forearm weakness.
  • The condition involves compression of the lower cervical spinal cord due to neck flexion.

Observation:

  • A case study of a young Indian male with progressive hand and forearm weakness is presented.
  • Clinical presentation, diagnostic imaging (flexion MRI), and management strategies are discussed.

Findings:

  • Flexion MRI revealed anterior dural translation and compression of the anterior cervical cord horns.
  • The patient experienced successful treatment and symptom relief using a cervical collar.

Implications:

  • This review aims to increase clinician awareness of Hirayama disease.
  • Optimizing diagnosis and management of Hirayama disease can improve patient outcomes.