Frontotemporal dementia is a neurodegenerative disorder of the brain, which arises due to the progressive deterioration of neurons located at the level of the frontal and temporal lobes of the brain proper .
With repercussions on language, behavior, ability to think and part of the movements, frontotemporal dementia was once called Pick's disease, in honor of Arnold Pick, who described it for the first time in 1892 .
Pick identified one of the typical characteristics of the disease, namely the presence, within the neurons of the frontal and temporal lobes, of protein aggregates formed predominantly by tau, a fundamental protein for the life of the cell.
These aggregates were identified with the name of " bodies of Pick ", a name which they still retain today.
What causes the formation of Pick's bodies still remains a mystery.
NEW DISCOVERIES AND CHANGE OF TERMINOLOGY
Studies conducted in the last decades on frontotemporal dementia have revealed that there are alternative structures to the so-called Pick bodies : they are always protein agglomerates, but at their origin there is a genetic mutation and, in some cases, instead of being formed only by tau, also present a protein called TDP-43 .Thus, after these new discoveries, the researchers acknowledged the existence of at least three subtypes of frontotemporal dementia:
- Frontotemporal dementia in Pick bodies, also called Pick's disease .
With the recognition of the three subtypes, to consider synonymous the term Pick's disease and the term frontotemporal dementia is incorrect.
Pick's disease usually arises after the age of 50.
- Frontotemporal dementia with parkinsonism, linked to chromosome 17 .
It is in effect a hereditary disease, characterized by an alteration of the MAPT gene and the presence of masses of tau protein (NB: MAPT deals with the synthesis of the tau protein).
- Primary progressive aphasia .
With a typical onset around the age of 40 and with slow and gradual symptom progression, it can present itself as a disease of unknown causes or as an inherited disease linked to mutations in the tau and TDP-43 genes.
