Last reviewed:December 2019
Last updated:August  2018

Summary

Definition

History and exam

Key diagnostic factors

  • coarsening of personality, social behavior, and habits
  • progressive loss of language fluency or comprehension
  • development of memory impairment, disorientation, or apraxias
  • progressive self-neglect and abandonment of work, activities, and social contacts

Other diagnostic factors

  • age at onset peak in mid-50s
  • FHx of FTD
  • altered eating habits
  • inattentiveness, puerile preoccupations, economy of effort, and impulsive responding
  • signs of amyotrophic lateral sclerosis (ALS)
  • parkinsonian symptoms
  • fasciculations, atrophy, hyperreflexia, and other signs of motor neuron disease
  • glabellar, snout, sucking, rooting, or grasp reflex
  • loss of bladder and bowel control

Risk factors

  • mutations in MAPT gene
  • mutations in PGRN gene
  • head trauma
  • thyroid disease

Diagnostic investigations

Treatment algorithm

Contributors

Consultant Psycho-geriatrician

Waitemata District Health Board

Auckland

New Zealand

Disclosures

RKR worked for BMJ Clinical Evidence in 2012 and received honorarium. RKR declares that he has no other competing interests.

Consultant Psycho-geriatrician

Waitemata District Health Board

Honorary Senior Lecturer in Old Age Psychiatry

University of Auckland

Auckland

New Zealand

Disclosures

SJ declares that he has no competing interests.

Dr Raghava Kurup Radhakrishnan and Dr Sachin Jauhari would like to gratefully acknowledge Dr Lawrence Whalley and Dr Chiadi Onyike, the previous contributors to this topic. LW and CO declare that they have no competing interests.

Peer reviewersVIEW ALL

Director

Memory Disorders Program

Department of Neurology

University of North Carolina at Chapel Hill

NC

Disclosures

DK has received research support, speaking honoraria, and consulting fees from Eisai, Forest Laboratories, Johnson & Johnson, Medivation, Novartis, Ortho-McNeil, and Pfizer.

Professor of Neurology

University of California

San Francisco

CA

Disclosures

BLM declares that he has no competing interests.

Professor of Neurology

Department of Neurology/Alzheimer Center

VU University Medical Center

Amsterdam

The Netherlands

Disclosures

PS declares that he has no competing interests.

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