[Neuropathology of frontotemporal dementia]

The syndrome of progressive fronto-temporal dementia represents the clinical expression of several pathological processes, mostly degenerative, preferentially involving the frontal and temporal lobes. These processes include dementia with Pick bodies, cortico-basal degeneration, dementia with ITSNU (Inclusions Tau and Synuclein Negative, Ubiquitinated), also known as dementia of motorneuron disease type, dementia with basophilic inclusion bodies, and dementia lacking specific histopathology, and in addition all variants linked to mutations in the tau gene, located in chromosome 17. The term "Pick complex" encompasses these processes and their clinical manifestations, which in addition to fronto-temporal dementia include primary progressive aphasia and apraxic-motor syndromes. The pathologic processes are better discriminated by histopathology than distribution of atrophy, but the latter is the main determinant of the clinical presentation.