title: Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials.
abstract: BACKGROUND
  Spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are motor neuron diseases. Sensory impairment is sometimes observed, and electrophysiological involvement has been described in the sensory nerves of SBMA patients. We hypothesized that a sensory nerve conduction study (NCS) could be used to discriminate SBMA from ALS.
  
  
  METHODS
  We compared the results from NCSs in a total of 120 SBMA cases confirmed by genetic analysis, 188 ALS cases, and 50 normal subjects.
  
  
  RESULTS
  Sensory nerve action potential (SNAP) amplitudes of the SBMA group were significantly lower than in the ALS and control groups. In addition, receiver-operating characteristic curve analysis for SNAP amplitude showed that a cut-off value of 13.8 μV for median, 10.7 μV for ulnar, and 9.9 μV for sural nerve best discriminated SBMA from ALS.
  
  
  CONCLUSIONS
  The specific decrease of SNAP amplitude in SBMA provides another useful tool for the differential diagnosis of motor neuron diseases.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho Showa-ku, Nagoya 466-8550, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Hama
authors.forename: Masaaki
authors.initials: M
authors.lastname: Hirayama
authors.forename: Takashi
authors.initials: T
authors.lastname: Hara
authors.forename: Tomohiko
authors.initials: T
authors.lastname: Nakamura
authors.forename: Naoki
authors.initials: N
authors.lastname: Atsuta
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Banno
authors.forename: Keisuke
authors.initials: K
authors.lastname: Suzuki
authors.forename: Masahisa
authors.initials: M
authors.lastname: Katsuno
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Tanaka
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000200:Action Potentials / Q000235:genetics / Q000502:physiology*; D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000503:physiopathology*; D000704:Analysis of Variance; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000235:genetics / Q000503:physiopathology*; D004558:Electric Stimulation; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000235:genetics / Q000502:physiology*; D010525:Peripheral Nerves / Q000503:physiopathology*; D012372:ROC Curve; D011944:Receptors, Androgen / Q000235:genetics; D019680:Trinucleotide Repeat Expansion / Q000235:genetics
chemical_list: C410798:AR protein, human; D011944:Receptors, Androgen