Symptoms of frontotemporal dementia
What is the clinical picture of frontotemporal dementia?
This designation includes a group of entities with variable neuropathologic findings and similar clinical features.
Patients have early personality changes, particularly impulsivity and Klüver–Bucy-type symptoms (including hyperorality) or withdrawal and depression, but they may present with a semantic dementia (loss of appreciation for word meaning) or progressive nonfluent aphasia.
Psychiatric symptoms may precede dementia by several years. Memory and frontal executive tasks (e.g., planning, set-shifting, and set maintenance) are much more impaired than attention, language, and visuospatial skills. SPECT or PET studies may show hypofrontality.
Neuropathology includes Pick’s disease or primary degeneration at multiple brain sites (dementia lacking distinctive histology), usually with gliosis and often with protein inclusions such as tau, TDP-43, or ubiquitin.
Many of these cases have been linked to genetic mutations in the tau protein on chromosome 17 or to mutations in progranulin on chromosome.
Many of these cases have been linked to genetic mutations in either the tau protein or the progranulin gene, both located on chromosome 17.
