Frontotemporal dementia
Demência frontotemporal
CategoryDefinition
Frontotemporal dementia (FTD) is a group of primary neurodegenerative disorders primarily affecting the frontal and temporal lobes. Onset is typically insidious with a gradual and worsening course. Several syndromic variants (some with an identified genetic basis or familiality) are described that include presentations with predominantly marked personality and behavioral changes (such as executive dysfunction, apathy, deterioration of social cognition, repetitive behaviours, and dietary changes), predominantly language deficits (that include semantic, agrammatic/nonfluent, and logopenic forms), predominantly movement-related deficits (progressive supranuclear palsy, corticobasal degeneration, multiple systems atrophy, or amyotrophic lateral sclerosis), or a combination of these deficits. Memory function often remains relatively intact, particularly during the early stages of the disorder.
Coding Note
Diagnostic Criteria
- All diagnostic requirements for Dementia are met.
- Dementia is presumed to be attributable to underlying frontotemporal disease or atrophy, as demonstrated by neuropsychological test data, neuroimaging data, genetic testing, medical tests, family history, and/or clinical history.
- Frontotemporal Dementia variants include primary progressive aphasia (logopenic, semantic, and agrammatic subtypes), behavioural frontotemporal dementia, and motoric frontotemporal dementia (corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis).
- Frontotemporal Dementia is progressive, with variants identified based on initial symptoms.
- Frontotemporal Dementia, behavioural variant is characterized by personality changes, often including apathy and progressively inappropriate social behaviour. Neurocognitive functioning may be preserved in the early stages, though may later involve deficits in executive functioning (e.g., planning, problem solving), with comparatively intact memory skills.
- Another variant, primary progressive aphasia, is characterized by progressive impairment in language skills, initially in the absence of impairment in other cognitive skills. Subtypes of primary progressive aphasia are often determined based on neuropsychological/cognitive testing, clinical presentation, and sometimes neuroimaging, and are characterized by primary deficits in word finding (logopenic subtype), word meaning (semantic subtype), or word production (agrammatic subtype).
- Frontotemporal Dementia, motoric variant involves progressive impairment in motor functioning, sometimes in the context of progressive neurocognitive impairment (typically characterized by impairment in attention, executive functioning, and visuospatial skills, with comparatively intact memory skills). Frontotemporal Dementia, motoric variant can include progressive supranuclear palsy (e.g., poor balance, frequent falls, and horizontal gaze palsy, etc.), corticobasal degeneration (e.g., limb apraxia, tripping, rigidity, and dystonia, etc.), and amyotrophic lateral sclerosis (e.g., muscle weakness, muscle atrophy, fasciculations, and spasticity, etc.).
Note: A diagnosis of 8A23 Frontotemporal Lobar Degeneration should also be assigned.