Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis

M. Witgert, A. R. Salamone, A. M. Strutt, A. Jawaid, P. J. Massman, M. Bradshaw, D. Mosnik, S. H. Appel, P. E. Schulz

Research output: Contribution to journalArticlepeer-review

102 Scopus citations


Background: Cognitive impairment secondary to frontal lobe atrophy exists in 40-60% of Amyotrophic Lateral Sclerosis (ALS) cases. We aimed to determine the prevalence of frontal-lobe mediated behavioral impairment in (ALS) and to ascertain its relationship to cognitive impairment. Methods: Two-hundred and twenty five patients diagnosed with sporadic ALS were evaluated for behavioral dysfunction using the Frontal Systems Behavior Scale (FrSBe), a validated measure used to examine frontal-lobe mediated behaviors, specifically apathy, executive dysfunction and disinhibition; a total behavior score is also provided. Additionally, a subset of patients also underwent a comprehensive neuropsychological evaluation. Results: Changes in the total FrSBe scores were observed in 24.4% of the patients and 39.6% of the patients had impairment in at least one behavioral domain with symptoms of Apathy being the most common (31.1%). Cognitively impaired ALS patients had worse total (P = 0.05) and apathy scores (P < 0.01); however, behavioral dysfunction was also present in 16% of the cognitively intact patients. Half of the behaviorally intact patients exhibited cognitive impairment. Significant correlations were observed for performance on certain neuropsychological tests (Animal fluency, Block Design, Logical Memory I and Verbal Series Attention Test) and severity of behavioral dysfunction on certain FrSBe sub scores. Conclusions: Frontal-lobe mediated behavioral dysfunction appears to be common in ALS. Cognitively impaired ALS patients had greater behavioral dysfunction. Recognition of behavioral and cognitive dysfunction may assist health-care providers and care-givers recognize changes in decision-making capacity and treatment compliance of patients with ALS.

Original languageEnglish (US)
Pages (from-to)103-110
Number of pages8
JournalEuropean Journal of Neurology
Issue number1
StatePublished - Jan 2010


  • Amyotrophic lateral sclerosis
  • Behavior
  • Frontotemporal dementia
  • Neuropsychological evaluation

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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