Psych · Old age psychiatry — neurocognitive disorders
Frontotemporal dementia
Also known as Frontotemporal lobar degeneration · FTD · FTLD · Behavioural variant FTD · bvFTD · Primary progressive aphasia · Pick disease · Semantic dementia
Exam-exhaustive fellowship reference on frontotemporal dementia — Rascovsky bvFTD criteria; Gorno-Tempini PPA variants (nfv/sv/lv); proteinopathies (tau, TDP-43, FUS); genetics (C9orf72, MAPT, GRN); psychiatric misdiagnosis; ALS-FTSD overlap; work-up; non-pharmacological care; SSRI/trazodone symptomatic options; avoid routine AChEI; antipsychotic mortality caution; capacity and risk. FRANZCP-primary, globally tagged.
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Target exams
Red flags
- New middle-age personality change, disinhibition, or loss of empathy with progressive course — do not label as 'midlife crisis' or primary bipolar without neurocognitive work-up
- Psychosis or late-onset schizophrenia-like picture plus family ALS/FTD history — consider C9orf72 spectrum
- Progressive language collapse (nonfluency, loss of word meaning, or logopenic pauses) — classify PPA variant; do not assume ordinary stroke aphasia if insidious
- Fasciculations, bulbar symptoms, or progressive weakness with behavioural/language FTD — urgent MND pathway (ALS-FTSD)
- Antipsychotic as first response to 'agitation' without medical screen or behavioural plan — mortality risk signal in elderly dementia
- Unsafe driving, financial exploitation, sexual disinhibition, or child-care risk — capacity and safeguarding, not only a cognitive score
- Rapidly progressive course — widen differential (autoimmune, CJD, space-occupying, metabolic) before locking pure FTD
Meet the patient
A 59-year-old builder is brought in by his daughter after he was charged with shoplifting. He took four chocolate bars, in full view of the security guard, and cannot see what the fuss is about.[1][10]
His wife says he stopped noticing when she cried about two years ago. He now eats sugar by the spoonful, drinks eight cups of sweet tea a day, and checks the front door lock in a fixed sequence every hour. He has been diagnosed with depression twice and bipolar disorder once.[1][10][14]
His brother died of motor neurone disease at 55. Two exam questions are live. Why did three psychiatrists get this wrong? And what must you examine before he leaves the room? Everything below answers both.[4][14][15]
References18ShowHide
- [1]Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Brain, 2011.PMID 21810890
- [2]Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants Neurology, 2011.PMID 21325651
- [3]Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria Neurology, 1998.PMID 9855500
- [4]DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS Neuron, 2011.PMID 21944778
- [5]Renton AE, Majounie E, Waite A, et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD Neuron, 2011.PMID 21944779
- [6]Hutton M, Lendon CL, Rizzu P, et al. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17 Nature, 1998.PMID 9641683
- [7]Baker M, Mackenzie IR, Pickering-Brown SM, et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17 Nature, 2006.PMID 16862116
- [8]Cruts M, Gijselinck I, van der Zee J, et al. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21 Nature, 2006.PMID 16862115
- [9]Bang J, Spina S, Miller BL Frontotemporal dementia Lancet, 2015.PMID 26595641
- [10]Piguet O, Hornberger M, Mioshi E, Hodges JR Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management Lancet Neurol, 2011.PMID 21147039
- [11]Hodges JR, Patterson K Semantic dementia: a unique clinicopathological syndrome Lancet Neurol, 2007.PMID 17945154
- [12]Huey ED, Putnam KT, Grafman J A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia Neurology, 2006.PMID 16401839
- [13]Lebert F, Stekke W, Hasenbroekx C, Pasquier F Frontotemporal dementia: a randomised, controlled trial with trazodone Dement Geriatr Cogn Disord, 2004.PMID 15178953
- [14]Woolley JD, Khan BK, Murthy NK, et al. The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with frontotemporal dementia J Clin Psychiatry, 2011.PMID 21382304
- [15]Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria Amyotroph Lateral Scler Frontotemporal Degener, 2017.PMID 28054827
- [16]Mackenzie IR, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update Acta Neuropathol, 2010.PMID 19924424
- [17]Schneider LS, Dagerman KS, Insel P Risk of death with atypical antipsychotic drug treatment for dementia: meta-analysis of randomized placebo-controlled trials JAMA, 2005.PMID 16234500
- [18]Appelbaum PS Clinical practice. Assessment of patients' competence to consent to treatment N Engl J Med, 2007.PMID 17978292