Examining the language and behavioural profile in FTD and ALS-FTD

J Neurol Neurosurg Psychiatry. 2017 Aug;88(8):675-680. doi: 10.1136/jnnp-2017-315667. Epub 2017 Jun 8.


Background: A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone.

Methods: A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment.

Results: A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p=0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups.

Conclusions: Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD.

Keywords: amyotrophic lateral sclerosis; aphasia; frontotemporal dementia.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyotrophic Lateral Sclerosis / diagnosis*
  • Aphasia, Broca / diagnosis
  • Auditory Perceptual Disorders / diagnosis
  • Cohort Studies
  • Diagnosis, Differential
  • Disease Progression
  • Early Diagnosis
  • Female
  • Frontotemporal Dementia / diagnosis*
  • Humans
  • Language Disorders / diagnosis*
  • Male
  • Middle Aged
  • Mood Disorders / diagnosis
  • Neuropsychological Tests
  • Retrospective Studies
  • Social Behavior Disorders / diagnosis*