Frontotemporal lobar degenerations (FTLD) refers to a group of neurodegenerative disorders characterized by the progressive degeneration and atrophy of the frontal and temporal lobes of the brain. These brain regions play significant roles in personality, behavior, language, and executive functions, explaining the common clinical manifestations of this condition, such as changes in behavior, personality, and language difficulties.
FTLD is also known as frontotemporal dementia (FTD) due to the similarity in symptoms with other types of dementia. However, FTLD is unique in that it primarily affects the frontal and temporal lobes rather than the hippocampus, the region predominantly affected in Alzheimer's disease.
There are several subtypes of FTLD, each demonstrating distinct pathological and genetic characteristics. The most common subtypes are behavioral variant frontotemporal dementia (bvFTD), semantic variant primary progressive aphasia (svPPA), and non-fluent variant primary progressive aphasia (nfvPPA). Each subtype present with specific clinical symptoms and different patterns of atrophy and neuronal loss in the brain.
The exact cause of FTLD is still unknown, but certain gene mutations have been identified as risk factors for developing the condition, suggesting a genetic component. However, a significant proportion of cases are sporadic, with no apparent family history.
As the disease progresses, individuals with FTLD experience a decline in cognitive functions, resulting in significant impairments in daily activities. Unfortunately, there are currently no disease-modifying treatments for FTLD, and management mainly focuses on symptom relief and support for the affected individuals and their families.
