title: Clinical evolution of pure upper motor neuron disease/dysfunction (PUMMD).
abstract: BACKGROUND
  PLS is defined as pure upper motor neuron disease/dysfunction (PUMND) beyond 48 months after symptom onset. We know little about its early stages, but such knowledge would help to identify the mechanisms underlying PLS and ALS and determine why PLS patients seem to be protected against lower MND (LMND).
  
  
  METHODS
  We reviewed 622 MND cases during a 4-year period and identified 34 patients with PUMND (5.4%).
  
  
  RESULTS
  Among 23 cases with follow-up data/electromyograms (EMGs; 2 had only 1 EMG), 13 (57%) remained classified as PUMND, and 8 (35%) developed LMND (mean, 51.4 months after onset). Of these 8, LMND developed in 3 after 48 months from symptom onset. Patients with PUMND and LMND were more functionally impaired (P = 0.02). Separately, we identified 5 patients with PUMND who developed LMND long after 48 months (range, 50-127 months).
  
  
  CONCLUSIONS
  PLS belongs to the ALS spectrum, and perhaps all cases eventually develop LMND.
authors.affiliation: Eleanor and Lou Gehrig MDA/ALS Center, Columbia University Medical Center, 710 West 168th Street, New York, NY 10032, USA.
authors.forename: Emanuele
authors.initials: E
authors.lastname: D'Amico
authors.forename: Meredith
authors.initials: M
authors.lastname: Pasmantier
authors.forename: Yei-Won
authors.initials: YW
authors.lastname: Lee
authors.forename: Louis
authors.initials: L
authors.lastname: Weimer
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mitsumoto
mesh_terms: D000328:Adult; D000368:Aged; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012189:Retrospective Studies