title: Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy.
abstract: OBJECTIVE
  We report decremental responses to repetitive nerve stimulation (RNS) in 11 patients diagnosed with X-linked spinobulbar muscular atrophy (X-SBMA).
  
  
  METHODS
  The compound muscle action potential (CMAP) of the right abductor digiti minimi (ADM) and trapezius (TZ) in response to a 3-Hz stimulation of the ulnar nerve at the wrist and accessory nerve at the neck were recorded by surface electrodes.
  
  
  RESULTS
  A decremental response to RNS was observed in 90.9% of the TZ muscle and 27.2% in the ADM muscle of patients with X-SBMA.
  
  
  CONCLUSIONS
  These electrophysiological features of X-SBMA are considered to be useful for diagnosis of X-SBMA. Furthermore, the waning phenomena that mostly appeared in the TZ muscle and increment of CMAP in RNS after the exercise also suggest a unique manifestation in X-SBMA.
authors.affiliation: Department of Neurology, Kawasaki Medical School, Okayama, Japan. kenin_04@yahoo.co.jp
authors.forename: K
authors.initials: K
authors.lastname: Inoue
authors.forename: S
authors.initials: S
authors.lastname: Hemmi
authors.forename: M
authors.initials: M
authors.lastname: Miyaishi
authors.forename: Y
authors.initials: Y
authors.lastname: Kutoku
authors.forename: T
authors.initials: T
authors.lastname: Murakami
authors.forename: K
authors.initials: K
authors.lastname: Kurokawa
authors.forename: Y
authors.initials: Y
authors.lastname: Sunada
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000175:diagnosis* / Q000503:physiopathology*; D004558:Electric Stimulation / Q000379:methods*; D004568:Electrodiagnosis / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018763:Muscle Fatigue / Q000502:physiology*