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
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
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
Treatment algorithm
Contributors
Authors
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 reviewers
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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