The current clinical syndrome frontotemporal dementia (FTD) was first described in 1892 by the Czech
psychiatrist Arnold Pick. He described a patient with aphasia and behavioural changes with on
macroscopic examination marked left frontotemporal atrophy. In 1911, Alois Alzheimer described the
detailed microscopic changes, including argyrophilic neuronal inclusions, which are still known as Pick
bodies. The term Pick’s disease was introduced in 1926 and was used till the early 90’s to describe the
clinical and pathological entity. To date, Pick’s disease is used for a neuropathological subgroup of
FTD patients.
FTD encompasses distinct canonical syndromes: the behavioural variant of FTD (bvFTD), and two
language variants, semantic dementia (SD), and progressive non-fluent aphasia (PNFA). FTD is
accompanied by motor neuron disease (MND) in 5 – 15 % of the cases. FTD patients characteristically
present at presenile age with variable behavioural changes and language disturbances.
The clinical syndrome FTD is part of a wide clinicopathological spectrum designated by the term
frontotemporal lobar degeneration (FTLD). The last few years have seen major advances in our
understanding of FTD, its genetic causes and pathological substrates.
In 1994, a genetic-epidemiological study on FTD was started at the Erasmus University Medical Center
of Rotterdam. Since then, over 400 patients have been included in our FTD cohort.
The aim of this thesis was to describe and determine the relationship between the clinical presentation,
genetics and pathology of FTD, with emphasis on the hereditary form of FTD.http://repub.eur.nl/res/pub/22791/110323_Seelaar%2C%20Harro.pdf
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