title: Compound Muscle Action Potential Decrement to Repetitive Nerve Stimulation Between Hirayama Disease and Amyotrophic Lateral Sclerosis.
abstract: OBJECTIVE
  To compare repetitive nerve stimulation (RNS) between Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS).
  
  
  METHODS
  The 3-Hz RNS test was performed on bilateral abductor pollicis brevis, abductor digiti minimi, middle deltoid, and upper trapezius muscles in 33 HD patients and 37 ALS patients.
  
  
  RESULTS
  In HD patients, none of tested muscles showed any abnormal decrements (≥10%). Significant decrements were observed in 73% of the ALS patients, and decrements were more frequently observed in proximal muscles (deltoid: 70.3%; trapezius: 48.6%). Illness duration did not correlate with decrement percentage in either patient group, and there was no relationship between decrement incidence and ALS diagnostic category (P > 0.05).
  
  
  CONCLUSIONS
  The significantly different RNS results between ALS and HD patients support the application of RNS, especially performing RNS in proximal muscles, as a supplementary test in distinguishing these two diseases, even in the early stages. These results may also imply a difference in underlying pathophysiology between ALS and HD.
authors: Departments of *Orthopedics and †Neurology, Huashan Hospital, Fudan University, Shanghai, China; ‡Department of Orthopedics, The Fifth People's Hospital, Fudan University, Shanghai, China; and §Department of Physical Medicine and Rehabilitation, Upstate Medical University, State University of New York at Syracuse, Syracuse, New York, U.S.A.
authors: Chaojun
authors: C
authors: Zheng
authors: Dongqing
authors: D
authors: Zhu
authors: Feizhou
authors: F
authors: Lu
authors: Yu
authors: Y
authors: Zhu
authors: Xiaosheng
authors: X
authors: Ma
authors: Xinlei
authors: X
authors: Xia
authors: Robert
authors: R
authors: Weber
authors: Jianyuan
authors: J
authors: Jiang
mesh_terms: D000200:Action Potentials / Q000502:physiology*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology*; D004558:Electric Stimulation; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology*; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology*; D055815:Young Adult