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Published on: August 22, 2012

Hirayama disease in children from mainland of China

Guang Yang1, Xiaosu Yang, Ming Zhang

  • 11Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China.

Journal of Child Neurology
|April 4, 2013
PubMed

Insights

Hirayama disease, a rare neurological disorder, affects young individuals with limb weakness. Early diagnosis and cervical collar use can halt the progression of this condition in children.

Area of Science:

  • Neurology
  • Pediatrics
  • Clinical Medicine

Background:

  • Hirayama disease presents with asymmetrical distal upper limb weakness and atrophy.
  • Onset typically occurs in adolescence or early adulthood.

Purpose of the Study:

  • To review the clinical features of Hirayama disease in children (onset before 18 years) in mainland China.
  • To identify diagnostic and therapeutic insights for early-stage intervention.

Main Methods:

  • Retrospective study of 65 children diagnosed with Hirayama disease.
  • Clinical feature review, electrophysiology studies, and flexion cervical MRI.

Main Results:

  • Mean age of onset was 15.7 years.
  • Electrophysiology revealed C7-T1 denervation; MRI showed posterior dural sac shifting and epidural venous plexus engorgement.
  • Cervical collar intervention led to disease progression arrest.

Conclusions:

  • Hirayama disease in Chinese children shares features with adult cases.
  • Early diagnosis and cervical collar treatment are crucial for managing the condition.
  • Increased awareness can improve early detection and intervention.

Keywords:
Hirayama diseasedenervationelectrophysiologymagnetic resonance imaging (MRI)

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