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Frontotemporal degeneration

  

Introduction

Frontotemporal degeneration (FTD) is a progressive disease that results in damage to the temporal lobes and (or) the frontal lobes of the brain.

 

It causes a group of brain disorders that have many clinical features in common. FTD is also referred to as frontotemporal dementia, fronto-temporal lobar degeneration (FTLD), or Picks disease.

 

Symptoms

Types of FTD are identified according to the symptoms that come forward first and most evident. Types:

  • Behavioral variant FTD (bvFTD),
  • Primary progressive aphasia (PPA) which affects language,
  • Movement disorders progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD).

 

FTD differs from other forms of dementia in the following ways:

  • The characteristic of FTD is a gradual, progressive decline in behavior and (or) language, in which memory usually stays fairly well. As the disease progresses, it becomes increasingly difficult for people to plan or organize activities, behave appropriately in social or work settings, interact with others, and care for oneself, resulting in increasing dependency on caregivers.
  • FTD often commences manifesting itself in a person’s 50s and 60s, but has been seen as early as 21 and as late as 80 years. Roughly 60% of cases occur in people 45-64 years old (Knopman, 2011), thus FTD can affect work and family in a way dementia in older patients does not.

 

Cause

The cause of FTD is unknown. There are three proteins that may be associated with one or more FTD types: TDP-43, tau and FUS.

 

In 25 to 40% of the cases frontotemporal dementia is hereditary. Some hereditary forms are caused by an abnormal gene. As a result, the tau protein, that plays a role in the transport of substances into the brain, is not functioning well, and the brain cell eventually dies.

 

Treatment

There are no treatments to slow down or stop the progression of the disease, but FTD research is expanding. Bij this, a greater understanding of the disorders will be reached. 

FTD is often misdiagnosed as a psychiatric problem or movement disorder, such as Parkinson’s disease. Alzheimer’s disease is another possible misdiagnosis.

 

Important! Accurate diagnosis is crucial, as some medications used to treat other disorders may be harmful in a person with FTD.

More information:

The association for Frontotemporal Degeneration

From the site of the association you can download this PDF:

 

Guide for Managing a New Diagnosis – FTD

 

More information about two frontotemporal diseases: