Last reviewed: June 2018
Last updated: November  2017

Summary

Definition

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
Full details

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
Full details

Risk factors

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

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 ELISA
Full details

Investigations to consider

  • brain single photon emission computed tomography
  • brain FDG-PET
  • brain biopsy
Full details

Emerging tests

  • genetic testing
  • CSF analysis
Full details

Treatment algorithm

ACUTE

all patients

ONGOING

end-of-life care

Contributors

Authors VIEW ALL

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 monograph. LW and CO declare that they have no competing interests.

Peer reviewers VIEW 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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