The evolution of caregiver burden in frontotemporal dementia with and without amyotrophic lateral sclerosis

Sharpley Hsieh, Cristian E. Leyton, Jashelle Caga, Emma Flanagan, Cassandra Kaizik, Claire M. O'Connor, Matthew C. Kiernan, John R. Hodges, Olivier Piguet, Eneida Mioshi

Research output: Contribution to journalArticlepeer-review

27 Citations (Scopus)


BACKGROUND AND AIMS: Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) represent a disease spectrum. Caregiver burden in subtypes of FTD has not yet been directly compared with those patients who have co-existent FTD and ALS (ALSFTD).

METHOD: Perceived caregiver burden was evaluated using the short Zarit Burden Interview (ZBI) in patients with behavioral-variant FTD (bvFTD, n = 21), semantic dementia (SD, n = 18), and ALSFTD (n = 15) at the initial clinical presentation and follow-up assessments. The Mini-Addenbrooke's Cognitive Examination (M-ACE) and the Motor Neuron Disease Behaviour Scale (MiND-B) were also used. Linear mixed effects models examined longitudinal changes on the ZBI, M-ACE, and MiND-B across groups.

RESULTS: Burden at baseline was highest for the bvFTD group. Longitudinally, perceived burden increased for the SD and ALSFTD groups whereas in bvFTD, the level of burden which was high at baseline and remained high with disease progression. The severity of abnormal behaviors at baseline, as assessed by the MiND-B, correlated with baseline levels of caregiver burden and further accounted for 23% of the variance in caregiver burden at clinical follow-up.

CONCLUSIONS: The trajectory of perceived burden differs across the FTD-ALS spectrum, with SD and ALSFTD caregivers demonstrating an increased burden that develops over time, compared to a persistently high level for bvFTD caregivers, evident throughout the disease course. The evolution of burden in these three syndromes likely reflects the initial presentation and clinical characterization that develops with time. Psycho-education programs for caregivers, which provide better coping strategies for challenging behaviors, may reduce levels of burden experienced with disease progression.

Original languageEnglish
Pages (from-to)875-885
Number of pages11
JournalJournal of Alzheimer's Disease
Issue number3
Publication statusPublished - 2016
Externally publishedYes


  • Adaptation, Psychological
  • Aged
  • Amyotrophic Lateral Sclerosis/complications
  • Analysis of Variance
  • Caregivers/psychology
  • Case-Control Studies
  • Cost of Illness
  • Disease Progression
  • Female
  • Frontotemporal Dementia/complications
  • Humans
  • Interviews as Topic/methods
  • Longitudinal Studies
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Surveys and Questionnaires

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