Frontotemporal lobar degeneration (FTLD) covers a whole spectrum of neurodegenerative disorders which principally affect the frontal and temporal lobes of the brain.
Formerly, this group of diseases was referred to as Pick's disease, but there have been frequent changes to the name and the classification of frontotemporal lobar degeneration, as it has been a subject of consistent and strong debate.
Currently, the following disorders are grouped together under the overall title of FTLD:
- frontotemporal dementia (FTD) as behavioural variant,
- primary nonfluent aphasia (PNFA), and
- semantic dementia (SD) as language variants,
- amyotrophic lateral sclerosis with frontotemporal dementia (ALS+FTD),
- corticobasal syndrome (CBS) and
- progressive supranuclear palsy (PSP).
As well as the two principal groups of symptoms (changes to behavior and difficultier with speech), various extra-pyramidal motor symptoms may also become apparent, especially in the case of corticobasal syndrome and progressive supranuclear palsy.
There are very few studies regarding the frequency of these disorders, with estimates ranging from three patients per 100,000 of the population via 15 per 100,000 up to over 40 per 100,000. What is beyond doubt, however, is that the group of illness described under frontotemporal lobar degeneration represents the second-most frequent dementia in patients under 65 years of age. In terms of aetiology, there has been considerable progress in recent years, with neuropathology successfully identifying tau aggregates as well as TDP43 and FUS inclusions as contributing factors; meanwhile, genets has identified several genes which are risk factors. Among clinical practitioners, however, even symptomatic treatment of FTLD illnesses is controversial. One of this consortium's central goals is to develop and evaluate parameters which will help clinicans to diagnose FTLD at an early stage and follow its progression, with the overall aim of eventualle developing effective objective targets for therapeutic strategies.
Financed by the German Federal Ministry of Education and Research as part of the competence network for neurodegenerative dementias.