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Major or mild frontotemporal NCD is a common cause of early-onset NCD in individuals younger than 65 years.
Population prevalence estimates are in the range of 2-10 per 100,000.
Approximately 20%-25% of cases of frontotemporal NCD occur in individuals older than 65 years.
Prevalence estimates of behavioral variant and semantic language variant are higher among males.
Prevalence estimates of nonfluent language variant are higher among females.
Approximately 40% of individuals with major or mild frontotemporal NCD have a family history of early-onset NCD.
Approximately 10% show an autosomal dominant inheritance pattern.
A number of genetic factors have been identified, such as mutations in the gene encoding the microtubule associated protein tau (MAPT), the granulin gene (CRN), and the C90RF72 gene.
The presence of motor neuron disease is associated with a more rapid deterioration.
The criteria are met for major or mild neurocognitive disorder.
The disturbance has insidious onset and gradual progression.
Either (1) or (2):
1. Behavioral variant:
Three or more of the following behavioral symptoms:
i. Behavioral disinhibition.
ii. Apathy or inertia.
iii. Loss of sympathy or empathy.
iv. Perseverative, stereotyped or compulsive/ritualistic behavior.
v. Hyperorality and dietary changes.
Prominent decline in social cognition and/or executive abilities.
2. Language variant:
Prominent decline in language ability, in the form of speech production, word finding, object naming, grammar, or word comprehension.
Relative sparing of learning and memory and perceptual-motor function.
The disturbance is not better explained by cerebrovascular disease, another neuro-degenerative disease, the effects of a substance, or another mental, neurological, or systemic disorder