Electrophysiological features of Hirayama disease
Rong-Kuo Lyu1, Ying-Chih Huang, Yih-Ru Wu
1Section of Neuromuscular Disease, Department of Neurology, Chang Gung Memorial Hospital and University Medical College, Linkou Medical Center, No. 5, Fushing Street, Gueishan Township, Taoyuan County, Taoyuan 33305, Taiwan.
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.
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