Frontotemporale Demenz
Frontotemporal dementia (FTD) is an umbrella term reflecting the clinical syndromes of a range of diseases that cause frontotemporal lobar degeneration (FTLD), which is characterized by selective atrophy of the frontal and/or temporal cortices.
Terminology
Previously, Pick's disease has been used as an alternative term for frontotemporal dementia .
Epidemiology
Frontotemporal dementia is a major cause of young-onset dementia with most patients (60%) being diagnosed between 45-60 years and 10% being diagnosed <45 years old. Prevalence is estimated at between 1-26 cases per 100,000 population .
Clinical presentation
Patients with frontotemporal dementia present with changes in behavior, executive function and/or language .
Pathology
The major clinical syndromes of frontotemporal dementia include (N.B. variable nomenclature) :
- behavioral variant frontotemporal dementia
- primary progressive aphasia (a.k.a. language variant frontotemporal dementia)
- overlap syndromes
- progressive supranuclear palsy
- corticobasal syndrome
- FTD with motor neuron disease
Radiographic features
Frontotemporal dementia is characterized by selective atrophy of the frontal and/or temporal cortices, with frontoinsular atrophy considered to be particularly indicative of the diagnosis . Please see specific variant articles for further details.
History and etymology
Frontotemporal dementia was first described by Arnold Pick (1851–1924), Czech psychiatrist, neurologist and neuropathologist, in 1892 .
Differential diagnosis
Clinically, behavior and language changes can mimic other diseases :
- psychiatric disorders, e.g. obsessive-compulsive disorder, bipolar disease, schizophrenia, personality disorders
- other neurodegenerative diseases, e.g. Alzheimer disease, dementia with Lewy bodies
Siehe auch:
- Kortikobasale Degeneration
- Typen der Demenz
- frontotemporal lobar degeneration (FTLD)
- Atypische Parkinson-Syndrome
- frontal variant frontotemporal dementia (fvFTD)
- Morbus Alzheimer
und weiter:
Assoziationen und Differentialdiagnosen zu Frontotemporale Demenz:
