Last reviewed: 4 Jul 2021
Last updated: 16 Aug 2018



History and exam

Key diagnostic factors

  • coarsening of personality, social behaviour, 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, hyper-reflexia, 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

1st investigations to order

  • formal cognitive testing
  • brain MRI
  • brain CT
  • FBC
  • serum erythrocyte sedimentation rate
  • serum CRP
  • serum thyroid-stimulating hormone (TSH)
  • free T4
  • metabolic panel
  • serum urea
  • serum creatinine
  • LFTs
  • serum vitamin B12 levels
  • serum folate levels
  • syphilis serology
  • HIV testing
  • serum enzyme-linked immunosorbent assay

Investigations to consider

  • brain single photon emission computed tomography
  • brain fluorodeoxyglucose (FDG)-PET
  • brain biopsy

Emerging tests

  • genetic testing
  • cerebrospinal fluid analysis

Treatment algorithm



Raghava Kurup Radhakrishnan, DPM(NIMHANS), DNB(Psych), MRCPsych

Consultant Psycho-geriatrician

Waitemata District Health Board


New Zealand


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

Sachin Jauhari, DMH, MRCPsych, FRANZCP

Consultant Psycho-geriatrician

Waitemata District Health Board

Honorary Senior Lecturer in Old Age Psychiatry

University of Auckland


New Zealand


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 reviewers

Daniel Kaufer, MD


Memory Disorders Program

Department of Neurology

University of North Carolina at Chapel Hill



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

Bruce L. Miller, MD

Professor of Neurology

University of California

San Francisco



BLM declares that he has no competing interests.

Philip Scheltens, MD, PhD

Professor of Neurology

Department of Neurology/Alzheimer Center

VU University Medical Center


The Netherlands


PS declares that he has no competing interests.

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