Fronto-temporal Lobar Degeneration (FTLD)

 

FTLD affects adults aged 40-60 and early symptoms can be similar to depression or Alzheimer’s disease.

Specialist diagnosis is required as drug's used for Alzheimer's are not effective and can make the symptoms worse.

 

What is fronto-temporal lobar degeneration (FTLD)?

Symptoms and treatment of FTLD
FTLD is the term used to describe a group of dementias that affect the frontal lobes (the front top part of the brain) and/or the temporal lobes (the lower left and right sides of the brain). FTLD is the third most common kind of dementia in under 65 year olds.

 

The most common types of FTLD are:

Behavioural variant fronto-temporal dementia (also known as Pick’s disease) – accounts for 60% of FTD.

Patients suffering from FTDs may show personality change and impaired or altered social behaviour and a loss of inhibition. They may neglect personal hygiene, show signs of obsessive behaviour and have difficulties with spoken language, among other symptoms.

Semantic dementia – accounts for 20% of FTLD dementia.

Semantic dementia is caused by a shrinking of the temporal lobe of the brain, and affects our knowledge of objects, people, concepts and words (known as the semantic memory). It affects a slightly older group than the other dementias in this group, usually striking between the ages of 50 and 65.

People with semantic dementia may find they forget the meaning of words, and fail to recognise faces and objects. They may even find it hard to recognise familiar sounds, smells and tastes. Their speech is not affected however, and their autobiographical memories are often unaffected.

Progressive nonfluent aphasia – accounts for 20% of FTLD dementia

In primary progressive aphasia (PPA) the main sign is a breakdown in speech and language. Over a fairly long period of time the sufferer will lose their ability to speak, read, write, and/or understand what they hear. This is because the disease affects the part of the brain that controls our ability to use language. As with the FTDs, this illness mainly affects adults between 40 and 60.

 

What causes FTLD?

These dementias are caused by damage or atrophy to the front and temporal lobes of the brain, while the rest of the brain remains undamaged. Between one-third and a half of patients have a family history of the disease. The cause of non-inherited FTLD is not known.

 

The importance of specialist diagnosis:

FTLD dementias are sometimes mistaken for mental illnesses, such as depression or Alzheimer’s disease, because of the nature of the early symptoms.

Specialists can diagnose the conditions by taking very detailed histories and arranging cognitive and other tests. Although a positive diagnosis is not possible through blood tests, these may help rule out other conditions. CT and other scans may also help specialists determine the extent of damage to the brain.

 

Treatment of FTLD

At present there is no cure for FTLD dementias, and it is not possible to slow the progression of the diseases. Drugs used for Alzheimer’s disease, such as Donepezil / Aricept, are not effective in treating FTLD and may even make symptoms worse.

The aim of treatment is therefore to lessen the effect of the symptoms. This is one reason why a proper diagnosis is so important. The more that is known about the person’s condition, the easier it is to help them and their families cope, particularly with the behavioural changes associated with these diseases.

 

Some useful therapies:

• Patients with FTD and semantic dementia sometimes benefit from treatment with SSRI antidepressants.
• Behavioural therapy may be helpful in reinforcing positive behaviour in patients showing challenging symptoms.
• Spectacles and hearing aids may help in the case of hearing and visual impairment.
• Speech therapists may be helpful for language problems.
• Carers and relatives of FTLD sufferers may benefit from help in developing coping strategies to deal with the person’s altered behaviour rather than confronting them directly.