title: Use of Bayesian MUNE to show differing rate of loss of motor units in subgroups of ALS.
abstract: OBJECTIVE
  To evaluate differences among patients with different clinical features of ALS, we used our Bayesian method of motor unit number estimation (MUNE).
  
  
  METHODS
  We performed serial MUNE studies on 42 subjects who fulfilled the diagnostic criteria for ALS during the course of their illness. Subjects were classified into three subgroups according to whether they had typical ALS (with upper and lower motor neurone signs) or had predominantly upper motor neurone weakness with only minor LMN signs, or predominantly lower motor neurone weakness with only minor UMN signs. In all subjects we calculated the half life of MUs, defined as the expected time for the number of MUs to halve, in one or more of the abductor digiti minimi (ADM), abductor pollicis brevis (APB) and extensor digitorum brevis (EDB) muscles.
  
  
  RESULTS
  The mean half life of MUs was less in subjects who had typical ALS with both upper and lower motor neurone signs than in those with predominantly upper motor neurone weakness or predominantly lower motor neurone weakness. In 18 subjects we analysed the estimated size of the MUs and demonstrated the appearance of large MUs in subjects with upper or lower motor neurone predominant weakness. We found that the appearance of large MUs was correlated with the half life of MUs.
  
  
  CONCLUSIONS
  Patients with different clinical features of ALS have different rates of loss and different sizes of MUs.
  
  
  CONCLUSIONS
  These findings could indicate differences in disease pathogenesis.
authors.affiliation: Department of Neurology, Royal Brisbane & Women's Hospital, Queensland, Australia.
authors.forename: F
authors.initials: F
authors.lastname: Baumann
authors.forename: R D
authors.initials: RD
authors.lastname: Henderson
authors.forename: P G
authors.initials: PG
authors.lastname: Ridall
authors.forename: A N
authors.initials: AN
authors.lastname: Pettitt
authors.forename: Pamela A
authors.initials: PA
authors.lastname: McCombe
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology; D001499:Bayes Theorem; D002452:Cell Count; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology / Q000503:physiopathology; D009410:Nerve Degeneration / Q000473:pathology*; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors