Frontotemporal dementia

A type of dementia affecting the frontal and temporal lobes of the brain

Frontotemporal dementia (FTD) is a group of brain disorders caused by progressive cell degeneration in the brain's frontal lobes or its temporal lobes. These areas of the brain are generally associated with personality, behavior, and language.

Overview

Frontotemporal dementia is characterized by a gradual, progressive decline in behavior and/or language. The symptoms can vary significantly from person to person, depending on which parts of the frontal and temporal lobes are affected. Unlike other forms of dementia, memory is often relatively preserved in the early stages of FTD.

Types of Frontotemporal Dementia

FTD is classified into several subtypes based on the symptoms and the areas of the brain affected:

Behavioral Variant FTD

This is the most common form of FTD, marked by changes in personality, behavior, and judgment. Patients may exhibit socially inappropriate behavior, apathy, or loss of empathy.

Primary Progressive Aphasia

This subtype affects language skills, including speaking, writing, and comprehension. It is further divided into:

• Semantic variant PPA: Characterized by loss of word meaning.
• Nonfluent/agrammatic variant PPA: Marked by difficulty in forming words and sentences.

FTD with Motor Neuron Disease

Some individuals with FTD also develop motor neuron disease, which affects movement and muscle function.

Pathophysiology

Pick bodies in neurons, characteristic of Pick's disease, a type of FTD

The underlying cause of FTD is the accumulation of abnormal proteins in the brain, such as tau or TDP-43. These proteins form clumps that disrupt normal cell function and lead to cell death. The specific pattern of protein accumulation can vary, leading to the different subtypes of FTD.

Diagnosis

Diagnosis of FTD is based on clinical evaluation, neuropsychological testing, and brain imaging. MRI and PET scans can help identify patterns of brain atrophy typical of FTD. Genetic testing may be recommended if there is a family history of the disease.

Management

There is currently no cure for FTD, and treatment focuses on managing symptoms. This may include medications to address behavioral symptoms, speech therapy for language difficulties, and supportive care to improve quality of life.

Prognosis

FTD is a progressive disease, and the rate of progression can vary. On average, individuals live 6 to 8 years after the onset of symptoms, but this can range from 2 to over 20 years.

Related pages

• Dementia
• Alzheimer's disease
• Parkinson's disease
• Amyotrophic lateral sclerosis