J Korean Child Neurol Soc.  2011 Dec;19(3):257-261.

A Case of the Hirayama Disease in Young Male Adolescent

• Yeom JS
• Cho JY
• Lim JY
• Park CH
• Woo HO
• Youn HS
• Park KJ
• Kwak B
• Kim YS

Abstract

In the present study, we report muscular atrophy of the right distal upper extremity in a 14-year-old boy. The disease progressed insidiously for about 2 years, and during our first examination, he exhibited weakness and wasting in the right hand, and paresthesia on the C6-8 dermatomal area in the right upper extremity. Electromyography revealed neurogenic changes in atrophic muscles. Conduction velocity of the ulnar nerve of the affected hand was decreased. Magnetic resonance imaging (MRI) of the cervical spine in the neutral position revealed focal spinal cord atrophy and a small area of high signal intensity at C5-6 level. In the flexion-induced cervical spine MRI scan, the spinal cord was noticed to be compressed by the posterior dural sac with a forward shift and flow voids in the epidural space. All these parameters led to the diagnosis of Hirayama disease (HD). This is the first report of HD in Korea by pediatrician, even though it is characterized by juvenile onset.

Keyword

Hirayama disease; Muscular atrophy; Compressive myelopathy

MeSH Terms

Adolescent
Atrophy
Electromyography
Epidural Space
Hand
Humans
Korea
Magnetic Resonance Imaging
Male
Muscles
Muscular Atrophy
Paresthesia
Spinal Cord
Spinal Cord Compression
Spinal Muscular Atrophies of Childhood
Spine
Ulnar Nerve
Upper Extremity
Spinal Muscular Atrophies of Childhood