title: Neuropsychological functioning in PLS: a comparison with ALS.
abstract: OBJECTIVE
  In order to characterize the nature and extent of neuropsychological dysfunction in primary lateral sclerosis (PLS), we studied prospectively cognitive, emotional, and behavioral functioning in PLS, and compared performances to functioning in amyotrophic lateral sclerosis (ALS).
  
  
  METHODS
  Eighteen patients with PLS and 13 patients with ALS completed a neuropsychological test battery assessing both cognitive skills and emotional/behavioral functioning.
  
  
  RESULTS
  Both PLS and ALS groups scored broadly within normal limits (mean T-scores greater than 40) on all cognitive measures and no significant between-group differences were found with the exception of one variable. However, when examined on a case by case basis, the data revealed considerable heterogeneity amongst patients in both groups. Overall, 39% of PLS patients and 31% of ALS patients were considered cognitively impaired. Ahigher than expected frequency of abnormal scores was noted for several tests of executive function in both groups, and a majority of PLS patients also exhibited abnormal behavioural symptoms. There was no relationship in PLS or ALS groups between cognitive functioning and disease duration, current site of disease, site of onset, functional status, and respiratory variables. Comparison between the PLS and ALS groups indicated virtually no differences in cognitive test scores and overall emotional/behavioural symptoms.
  
  
  CONCLUSIONS
  We observed deficits in cognition and behaviour in a significant proportion of PLS patients which were comparable to those observed in ALS cases. Although deficits were not in the range of frontotemporal dementia, both ALS and PLS cases demonstrated deficits most prominently on tests of executive functioning.
authors.affiliation: Clinical Neurological Sciences and Psychological Services, London Health Sciences Centre, Ontario.
authors.forename: Gloria M
authors.initials: GM
authors.lastname: Grace
authors.forename: J B
authors.initials: JB
authors.lastname: Orange
authors.forename: Ann
authors.initials: A
authors.lastname: Rowe
authors.forename: Karen
authors.initials: K
authors.lastname: Findlater
authors.forename: Morris
authors.initials: M
authors.lastname: Freedman
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Strong
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000523:psychology; D001288:Attention / Q000502:physiology; D001526:Behavioral Symptoms / Q000175:diagnosis / Q000209:etiology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D004644:Emotions / Q000502:physiology; D056344:Executive Function / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000523:psychology; D009483:Neuropsychological Tests; D014796:Visual Perception / Q000502:physiology