Frontotemporal dementia is an umbrella term for a group of uncommon brain disorders that primarily affect the frontal and temporal lobes of the brain. These areas of the brain are generally associated with personality, behavior and language.
In frontotemporal dementia, portions of these lobes shrink (atrophy). Signs and symptoms vary, depending on which part of the brain is affected. Some people with frontotemporal dementia have dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use language properly.
Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease. But frontotemporal dementia tends to occur at a younger age than Alzheimer's. Frontotemporal dementia often begins between the ages of 40 and 65.
Signs and symptoms of frontotemporal dementia can be different from one individual to the next. Signs and symptoms get progressively worse over time, usually over years. Clusters of symptom types tend to occur together, and people may have more than one cluster of symptom types, which include:
In frontotemporal dementia, the frontal and temporal lobes of the brain shrink. In addition, certain substances accumulate in the brain. What causes these changes is usually unknown.
There are genetic mutations that have been linked to frontotemporal dementia. But more than half of the people who develop frontotemporal dementia have no family history of dementia.
Recently, researchers have confirmed shared genetics and molecular pathways between frontotemporal dementia and amyotrophic lateral sclerosis (ALS). More research needs to be done to understand the connection between these conditions, however.
Your risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.
The diagnosis of bvFTD (behaviour variant Frontotemporal Dementia) and PPA (Primary Progressive Aphasia) are based on expert evaluation by a doctor who is familiar with these disorders. The type of problems experienced by the patient and the results of neurological exams are the core of the diagnosis. Brain scans such as magnetic resonance imaging (MRI) and glucose positron emission scans are very helpful additional tests, but they must be interpreted in the context of the patient’s history and neurological exam.