- Frontotemporal dementia (FTD) is a group of brain disorders caused by progressive degeneration of the frontal and/or temporal lobes of the brain. Unlike Alzheimer’s disease, which usually begins with memory loss, FTD typically affects personality, behavior, and language first. It is one of the most common types of early-onset dementia, often occurring between the ages of 40 and 65, though it can also appear later in life.
- FTD is divided into several clinical syndromes based on the regions of the brain affected. The two most common forms are behavioral variant FTD (bvFTD) and primary progressive aphasia (PPA). Behavioral variant FTD is marked by dramatic changes in personality and social conduct, including apathy, impulsivity, loss of empathy, inappropriate behavior, and poor judgment. Patients may become socially withdrawn or exhibit compulsive behaviors. Primary progressive aphasia, on the other hand, presents with gradual loss of language skills. It is further subdivided into three types: nonfluent/agrammatic, semantic, and logopenic variants, each with distinct patterns of speech and comprehension difficulties.
- The underlying pathology of FTD involves the abnormal accumulation of certain proteins in the brain, most commonly tau, TDP-43, or FUS. These misfolded proteins form inclusions inside neurons, leading to cellular dysfunction and death. The specific type of proteinopathy varies among FTD subtypes and can overlap with other neurodegenerative diseases such as ALS (amyotrophic lateral sclerosis). Some cases of FTD are linked to genetic mutations, especially in the MAPT, GRN, and C9orf72 genes, which are associated with inherited forms of the disease.
- Diagnosis of FTD can be challenging, particularly in its early stages, as its symptoms may resemble psychiatric disorders or other dementias. A detailed clinical history, neurological examination, and cognitive testing are essential. Brain imaging—especially MRI or PET scans—often reveals focal atrophy in the frontal or temporal lobes. Genetic testing may be offered in cases with a strong family history. Biomarkers for FTD are currently under development but are not yet routinely used in clinical practice.
- There is currently no cure for frontotemporal dementia, and treatment focuses on symptom management and support. Unlike Alzheimer’s disease, FTD does not typically respond well to cholinesterase inhibitors or memantine. Behavioral symptoms may be addressed with antidepressants or antipsychotics, but these must be used with caution. Speech and language therapy can be helpful for those with language variants of FTD, and structured routines and behavioral interventions may reduce caregiver stress and improve quality of life.
- Research into FTD is ongoing, with a focus on identifying disease-specific biomarkers, understanding genetic mechanisms, and developing targeted therapies. As a cause of early-onset dementia, FTD has significant implications for patients and families, especially because it often strikes individuals at the peak of their personal and professional lives. Increasing awareness and improving early diagnosis are critical for better management and care planning.
