Frontotemporal dementia (FTD) is the third most common dementia following Alzheimer's disease and Lewy Body dementia. It is believed to have a worldwide prevalence of 3% to 26% in individuals ages 65 and older, with an average onset between 50 and 65 years of age (Stahl & Morrissette, 2019). Frontotemporal dementia is the second most common cause of early-onset dementia, with an average survival of 7.5 years (Khan & De Jesus, 2021). Although the origin of FTD is mostly sporadic, there are several genetic mutations that cause familial FTD in approximately 40% of cases (Khan & De Jesus, 2021).

Frontotemporal dementia occurs in the frontal and temporal lobes of the brain which are responsible for executive function, abstract thinking, learning and motivation, behavior, and personality. The initial changes are in the areas of language and behaviors, followed by loss of executive function and global cognitive abilities. Signs and symptoms include insomnia, daytime sleepiness, restless leg syndrome, falls, hypersexuality, and eating disorders such as lack of appetite, pica, and binge eating. Unlike Alzheimer's disease, there is typically not a rapid decline in memory, and the patient can perform in memory tasks if provided clues (Stahl & Morrissette, 2019). There are four subtypes of FTD: 1. Behavioral variant is characterized by personality changes, disinhibition, apathy, hyperorality, loss of empathy/sympathy, compulsive behaviors, and cognitive deficits, but the patient has a sparing of memory and visuospatial abilities. 2. Semantic Variant Primary Progressive Aphasia is characterized by impairments in random naming and single-word comprehension, difficulties in object knowledge, dysgraphia, and dyslexia. Speech may be fluent but empty of meaning, and with progression they may become almost mute with a very limited repertoire of phrases. 3. Nonfluent Variant Primary Progressive Aphasia presents with episodic memory lapses, in addition to severe deficits in speech output and semantic memory loss. 4. Logopenic Variant Primary Progressive Aphasia is characterized by prolonged word finding pauses, impaired naming of everyday objects, and a limited ability to process/construct a sentence (Stahl & Morrissette, 2019).

A complete medical and psychological work up is necessary to rule out other medical conditions. The diagnostic evaluation process can be lengthy, and pertinent data must be obtained from family members to secure an accurate diagnosis. Brain scans can help confirm diagnosis. Magnetic resonance imaging can show changes in the brain size and shape and help rule out stroke or a tumor, and a positron emission tomography may be useful to measure the brain's blood flow, oxygen, and glucose usage, and/or to rule out Alzheimer's disease.

Once diagnosed, there are currently no cures or disease modifying treatments, just symptomatic treatment to improve quality of life. Physical, occupational, and/or speech therapies, exercise, and dopamine replacement therapy may offer limited relief (Stahl & Morrissette, 2019). Cholinesterase inhibitors and memantine (frequently used in other dementias) are not recommended as they may worsen behavioral symptoms, motor function, and cognition. Families will need education on disease progression, symptom management (such as sleep hygiene measures and the importance of a consistent routine), respite services, support groups, the importance of obtaining a power of attorney, and financial planning. Finally, palliative care and hospice should be discussed to prepare families for the final stage of the terminal illness.

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