Frontotemporal dementia should be considered in adults aged 50–75 years presenting with behavioural or language changes
After Alzheimer disease, it is the second most common cause of dementia among adults younger than 65 years.1 Frontal and temporal lobe degeneration results in behavioural or language impairment (Appendix 1, available at www.cmaj.ca/lookup/doi/10.1503/cmaj.230407/tab-related-content).2
When frontotemporal dementia is suspected, other causes for symptoms, such as psychiatric diagnoses, should be considered
Cognitive screening with tests sensitive for executive dysfunction (e.g., Montreal Cognitive Assessment) should be used. Brain imaging, preferably magnetic resonance imaging (MRI), should be used to rule out structural brain lesions and investigate for frontotemporal atrophy. Laboratory investigations should be used to exclude treatable causes of behavioural impairment (e.g., vitamin B12, thyroid-stimulating hormone). Clinicians should then refer the patient to a neurologist.
Functional disability occurs within 3 years of diagnosis, but is variable between patients
Patients who present initially with language symptoms develop behavioural symptoms and vice versa. Apathy can be a prominent symptom that may eventually override disinhibition. Patients succumb to late-stage complications including malnutrition, falls and aspiration pneumonia. Earlier age of onset, specific genetic variations, behavioural-variant frontotemporal dementia and concomitant motoneuron disease portend a worse prognosis.3,4
Multidisciplinary care assists in diagnosis and in addressing functional and social problems
Where available, a behavioural neurologist or neuropsychiatrist should be involved in patient care. Consultation with a clinical geneticist should be arranged if the patient has a strong family history. Given the disease’s negative effects on cognition, impulsivity and judgment, advanced care planning can address safety concerns (e.g., driving) and legal matters (e.g., power of attorney).4
The course of frontotemporal dementia is not modifiable with existing treatment options
Cholinesterase inhibitors used for Alzheimer disease are not beneficial and may worsen neuropsychiatric symptoms. Antidepressants (e.g., trazodone) can reduce behavioural symptoms. Antipsychotics are indicated for patients who exhibit dangerous behaviours, but should otherwise be avoided because of adverse effects.5 Nonpharmacological strategies for symptom control are of limited efficacy.
Footnotes
Competing interests: Mario Masellis reports grant funding from the Ontario Brain Institute, the Canadian Institutes of Health Research, Woman’s Brain Health Initiative, Brain Canada, Weston Brain Institute, Washington University, Roche and Alector Pharmaceuticals. He has received royalties from Henry Stewart Talks and consulting fees from Ionis Pharmaceuticals, Alector Pharmaceuticals, Wave Life Sciences, Biogen Canada and Eisai. He is a board member with the Alzheimer’s Society Canada and Parkinson Canada. No other competing interests are declared.
This article has been peer reviewed.
This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY-NC-ND 4.0) licence, which permits use, distribution and reproduction in any medium, provided that the original publication is properly cited, the use is noncommercial (i.e., research or educational use), and no modifications or adaptations are made. See: https://creativecommons.org/licenses/by-nc-nd/4.0/
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- Frontotemporal dementia should be considered in adults aged 50–75 years presenting with behavioural or language changes
- When frontotemporal dementia is suspected, other causes for symptoms, such as psychiatric diagnoses, should be considered
- Functional disability occurs within 3 years of diagnosis, but is variable between patients
- Multidisciplinary care assists in diagnosis and in addressing functional and social problems
- The course of frontotemporal dementia is not modifiable with existing treatment options
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