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Hirayama disease in children from North America

Partha S Ghosh1, Manikum Moodley, Neil R Friedman

  • 1Pediatric Neurology Center, Children's Hospital, Cleveland Clinic, Cleveland, Ohio 44195, USA.

Journal of Child Neurology
|November 25, 2011
PubMed

Insights

Hirayama disease, a rare neurological disorder, affects young males in North America. Early diagnosis and cervical collar use are key for managing this condition.

Area of Science:

  • Neurology
  • Pediatric Neurology

Background:

  • Hirayama disease is predominantly reported in Asia, with limited data from North America.
  • This condition primarily affects adolescents and young adults, presenting with progressive weakness in the upper extremities.

Purpose of the Study:

  • To describe the clinical characteristics and diagnostic findings of Hirayama disease in pediatric patients in North America.
  • To emphasize the importance of recognizing this rare condition for timely intervention.

Main Methods:

  • Retrospective chart review of patients under 18 diagnosed with Hirayama disease over 10 years at a single center.
  • Analysis of clinical presentation, electromyography (EMG) findings, and cervical magnetic resonance imaging (MRI) results.

Main Results:

  • Six pediatric patients (4 boys, mean age 15.1 years) were diagnosed with Hirayama disease.
  • Common symptoms included unilateral/bilateral asymmetric distal upper extremity weakness, oblique amyotrophy, and cold paresis.
  • EMG revealed denervation in C8/T1 and C7 myotomes, sparing C5-C6. Cervical MRI was abnormal in 3 patients.
  • Symptoms progressed over a mean of 16.5 months; treatment involved cervical collars.

Conclusions:

  • Hirayama disease can occur in North American pediatric populations, presenting with characteristic upper extremity weakness.
  • Increased awareness among pediatric neurologists is crucial for early diagnosis and to avoid unnecessary investigations.
  • Cervical collar treatment may help manage symptom progression.

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