Can executive cognitive measures differentiate between patients with spinal- and bulbar-onset amyotrophic lateral sclerosis?

Arch Clin Neuropsychol. 2012 May;27(3):348-54. doi: 10.1093/arclin/acs031. Epub 2012 Mar 8.

Abstract

Although executive functions in sporadic non-demented amyotrophic lateral sclerosis (ALS) patients are mostly affected, it remains unclear whether executive measures can differentiate between patients with bulbar and spinal ALS forms. Thirty spinal and 18 bulbar-onset ALS patients (ALS-s and ALS-b, respectively) as well as 47 demographically related healthy controls were examined in executive processes (Trail Making Test-part B [TMT-(B-A)]; Stroop Neuropsychological Screening Test [SNST]; Similarities subtest of the Wechsler Adult Intelligence Scale [WAIS Similarities]; Wisconsin Card Sorting Test [WCST]). ALS subgroups were similar with regard to demographic characteristics and disease duration; yet, ALS-b showed greater disease severity compared with ALS-s patients (p = .006). Both ALS-s and ALS-b patients were significantly inferior to healthy controls on TMT-(B-A) (p < .001), SNST (p = .009 and p = .02, respectively) and WAIS Similarities (p = .031 and p = .021, respectively), whereas ALS-s performed significantly worse than controls on the WCST perseverative responses (p = .005). However, neuropsychological measures did not significantly differ between ALS subgroups (p > .05). Despite the fact that ALS-b patients may present greater disease severity, specific executive impairments that are present early in the course of ALS seems to be independent of the site of onset.

MeSH terms

  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis / classification
  • Amyotrophic Lateral Sclerosis / complications*
  • Cognition Disorders / etiology*
  • Disability Evaluation
  • Disease Progression
  • Executive Function / physiology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Psychiatric Status Rating Scales
  • Severity of Illness Index
  • Young Adult