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 tests
1st tests 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
Tests 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
all patients
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.
Differentials
- Alzheimer disease (AD)
- Dementia with Lewy bodies (DLB)
- Vascular dementia
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