Electrophysiological features of Hirayama disease.

Abstract

INTRODUCTION:

The purpose of this study was to compare the pattern of hand muscle involvement in Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS).

METHODS:

We reviewed findings of upper limb nerve conduction studies of 46 HD patients and 60 ALS patients. The findings from 54 healthy subjects were used for comparison.

RESULTS:

In HD, the ulnar compound muscle action potential (CMAP) amplitude was more severely reduced than the median one, and the reverse pattern was observed in ALS. The mean ulnar/median (U/M) CMAP amplitude ratio was significantly lower in HD (0.64 ± 0.79) and abnormally higher in ALS (2.15 ± 1.77) compared with normal subjects (0.89 ± 0.23). An abnormally low U/M CMAP amplitude ratio (<0.6) was encountered in 34 patients with HD and in 1 with ALS. A U/M CMAP amplitude ratio ≥4.5 or absent median motor response was found only in ALS.

CONCLUSION:

Our findings demonstrate different patterns of hand muscle involvement between these two diseases.

• Authors: Rong-Kuo Lyu, Ying-Chih Huang, Yih-Ru Wu, Hung-Chou Kuo, Long-Sun Ro, Chiung-Mei Chen, Hong-Shiu Chang
• Journal: Muscle & nerve (Muscle Nerve) Vol. 44 Issue 2 Pg. 185-90 (Aug 2011) ISSN: 1097-4598 [Electronic] United States
• PMID: 21755506 (Publication Type: Journal Article)
• Copyright: Copyright © 2011 Wiley Periodicals, Inc.

Topics

• Action Potentials (physiology)
• Adolescent
• Adult
• Aged
• Amyotrophic Lateral Sclerosis (physiopathology)
• Child
• Electric Stimulation
• Evoked Potentials, Motor (physiology)
• Female
• Hand (innervation, physiopathology)
• Humans
• Male
• Median Nerve (physiopathology)
• Middle Aged
• Muscle, Skeletal (innervation, physiopathology)
• Neural Conduction (physiology)
• Retrospective Studies
• Spinal Muscular Atrophies of Childhood (physiopathology)
• Ulnar Nerve (physiopathology)