Last reviewed: 5 Nov 2021
Last updated: 17 Sep 2021

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

  • early age at onset
  • family history of FTD
  • altered eating habits
  • inattentiveness, puerile preoccupations, economy of effort, impulsive responding, and compulsive behaviors
  • 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 GRN gene
  • mutations in C9orf72 gene
  • traumatic brain injury

Diagnostic investigations

1st investigations to order

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

Investigations to consider

  • brain fluorodeoxyglucose (FDG)-PET
  • brain single-photon emission computed tomography (SPECT)
  • brain biopsy
  • genetic testing
  • connective tissue panel
  • serum erythrocyte sedimentation rate

Emerging tests

  • cerebrospinal fluid analysis

Treatment algorithm

Contributors

Authors

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

Consultant Psycho-geriatrician

Waitemata District Health Board

Auckland

New Zealand

Disclosures

RKR declares that he has no competing interests.

Sachin Jauhari, DMH, MRCPsych, FRANZCP

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.

Acknowledgements

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, and colleagues Dr Ann Boston (clinical psychologist), Dr Zara Godinovich (clinical psychologist), and Diane Ellis (clinical nurse specialist) for their suggestions.

Disclosures

LW, CO, AB, ZG, and DE declare that they have no competing interests.

Peer reviewers

Daniel Kaufer, MD

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.

Bruce L. Miller, MD

Professor of Neurology

University of California

San Francisco

CA

Disclosures

BLM declares that he has no competing interests.

Philip Scheltens, MD, PhD

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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