title: Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  The potential linkage between upper (UMN) and lower motor neuron (LMN) involvement in amyotrophic lateral sclerosis (ALS) has not yet been fully elucidated. There is ongoing discussion as to whether ALS is primarily a disease of UMNs or LMNs.
  
  
  METHODS
  We performed a retrospective analysis of 189 ALS patients from our ALS outpatient database to investigate the different spreading patterns of UMN and LMN affection in disease progression in relation to the onset region.
  
  
  RESULTS
  The body region with the highest UMN involvement at onset in general also had the highest frequency of LMN signs and vice versa. This is in line with the hypothesis of a focal onset of disease, which then spreads to adjacent areas. However, there was a great variation between ALS phenotypes.
  
  
  CONCLUSIONS
  These observations support the hypothesis of focal damage of a localized group of motor neurons, which then spreads to adjacent motor neurons.
authors.affiliation: Department of Neurology, Hannover Medical School, Carl Neuberg Strasse 1, 30625 Hannover, Germany. koerner.sonja@mh-hannover.de
authors.forename: Sonja
authors.initials: S
authors.lastname: Körner
authors.forename: Katja
authors.initials: K
authors.lastname: Kollewe
authors.forename: Marion
authors.initials: M
authors.lastname: Fahlbusch
authors.forename: Antonia
authors.initials: A
authors.lastname: Zapf
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dengler
authors.forename: Klaus
authors.initials: K
authors.lastname: Krampfl
authors.forename: Susanne
authors.initials: S
authors.lastname: Petri
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000503:physiopathology*; D015331:Cohort Studies; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000503:physiopathology; D009046:Motor Neurons / Q000473:pathology / Q000502:physiology*; D018482:Muscle, Skeletal / Q000503:physiopathology; D012189:Retrospective Studies