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Electrophysiological features of hirayama disease
Author(s) -
Lyu RongKuo,
Huang YingChih,
Wu YihRu,
Kuo HungChou,
Ro LongSun,
Chen ChiungMei,
Chang HongShiu
Publication year - 2011
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.22028
Subject(s) - amyotrophic lateral sclerosis , compound muscle action potential , medicine , electrophysiology , cardiology , ulnar nerve , median nerve , electromyography , anatomy , physical medicine and rehabilitation , disease , elbow
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. Muscle Nerve, 2011