Frontotemporal lobar degeneration
EBM Guidelines
Sep 8, 2021 • Completely updated
Table of contents
Extract
- Frontotemporal lobar degeneration (FTLD) is a group of progressive memory disorders
with atrophy in the frontotemporal lobes of the brain.
- Frontotemporal dementia
- Primary progressive aphasia
- Progressive nonfluent aphasia
- Semantic dementia
- Logopenic progressive aphasia
- FTLD plus syndromes
- Progressive supranuclear palsy (PSP)
- Corticobasal degeneration
- About 30% of all progressive memory disorders belong to the FTLD group.
- In about 50% of patients, excluding those with FTLD plus syndromes, there is a positive family history.
- The most common genetic aetiology associated with frontotemporal lobar degeneration
is a non-coding repeat expansion of the C9orf72 (chromosome 9 open-reading frame 72)
gene.
- Study of the C9orf72 mutation may be beneficial for the diagnosis of these diseases.
- FTLD patients of non-Finnish origin commonly have mutations in GRN and MAPT genes, as well.
- Genetic testing is done in specialized care.
- As frontotemporal lobar degeneration may have several underlying neuropathological subtypes, there are no specific biomarkers available for the disease.
- CSF biomarker (tau, p-tau and amyloid β) tests used for the diagnosis of Alzheimer’s disease do not reliably distinguish between frontotemporal lobar degeneration and Alzheimer’s disease.
- Normal findings in conventional cognitive screening tests (CERAD and MMSE) do not exclude diseases of the FTLD group.
- The disease should primarily be diagnosed and treatment started by a neurologist, geriatrician or other physician with expertise in memory disorders.
Search terms
Dementia, F03, Frontotemporal dementia, Frontotemporal lobar degeneration, G31.1, Geriatrics, Memory Disorders, Neurology, Progressive nonfluent aphasia, Psychiatry, Semantic dementia