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) :

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 :

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