J Korean Child Neurol Soc.  2018 Mar;26(1):52-56. 10.26815/jkcns.2018.26.1.52.

Two Cases of Hirayama Disease in a Pediatric Clinic

Affiliations
  • 1Department of Pediatrics, Children's Hospital, Asan Medical Center, University of Ulsan College of Medicine, Ulsan, Korea. tsko@amc.seoul.kr

Abstract

We report two pediatric cases with Hirayama disease"”a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study revealed decreased motor nerve action potential amplitudes in the ulnar nerve and radial nerve of the affected hands. The former patient showed normal magnetic resonance imaging (MRI) of the cervical spine, but the latter showed mild, asymmetric thinning of the anterior spinal cord at levels C5 to C7. Following active rehabilitation and avoidance of neck flexion, no further progression of neurological findings was noticed. These clinical findings were typical of Hirayama disease. We show that timely and accurate diagnosis for Hirayama disease is possible with awareness of disease history, careful physical examination, and the use of neurophysiological studies and MRI studies.

Keyword

Hirayama disease; Juvenile muscular atrophy; Monomelic amyotrophy

MeSH Terms

Action Potentials
Adolescent
Diagnosis
Hand
Humans
Magnetic Resonance Imaging
Male
Muscular Atrophy
Neck
Neural Conduction
Physical Examination
Radial Nerve
Rehabilitation
Spinal Cord
Spinal Muscular Atrophies of Childhood
Spine
Ulnar Nerve
Wrist
Full Text Links
  • JKCNS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr