Frontotemporal dementia (Pick's disease)

Alternative names

Pick’s disease, frontotemporal dementia, FTD

Description

Frontotemporal degeneration or dementia (FTD) is one of the most common types of dementia in people under the age of 65. Symptoms occur due to the progressive loss or shrinking of nerve cells (neurons) in the frontal and temporal areas of the brain. These areas of the brain control our “executive functions” such as personality, decision-making, language and social behaviour.

Unlike Alzheimer’s disease, where loss of memory is a major symptom, FTD mostly results in dysfunctional behaviour and language. Someone with FTD retains key features of memory, i.e. ability to orientate space and time as well as keeping track of day-to-day events.

Causes

Most cases of FTD are sporadic, meaning that there’s no known family history of FTD.
However, approximately 40% of individuals with FTD have a family history that includes at least one other relative who also has or had a neurodegenerative disease like Parkinson’s or Alzheimer’s disease.

Because the same symptoms are seen in both inherited and sporadic FTD, the stronger the family history of FTD or other family members having an associated disease like dementia, the more likely genetics plays a role.

Symptoms

FTD involves two main types of symptoms, namely involving changes in:

• Behaviour and personality (frontal lobes), although memory is usually relatively preserved.
• Problems with language, speech and memory (temporal lobes).
Symptoms relating to personality or behaviour changes may include the following:
• Impulsiveness
• Distractibility
• Being bored and listlessness (apathetic)
• Inappropriate social behaviour
• Loss of insight into the behaviours of oneself and others
• An increased interest in sex
• Changes in food preferences
• Agitation or, conversely, blunted emotions
• Neglect of personal hygiene
• Repetitive or compulsive behaviour
• Decreased motivation and energy
• Change in motor (movement) skills

The second type of FTD symptoms mostly involves language disturbance, including difficulty expressing language or understanding speech and word meaning.

Diagnosis

Since FTD develops gradually and has a wide range of symptoms, it is often initially misdiagnosed as another neurological illness such as Parkinson’s or Alzheimer’s disease. A geriatrician, psychogeriatrician or neurologist is best qualified to evaluate changes in cognition and behaviour.

The specialist will take a medical history, do a detailed physical and neurological examination, possibly do blood tests and utilise various diagnostic brain-imaging techniques to establish the extent of decreased blood flow or shrinking (atrophy) of areas in the brain. Magnetic resonance imaging (MRI), positron emission tomography (PET) and single photon emission computed tomography (SPECT) are some of the investigations that may assist in diagnosis. Psychometric testing is done to define the nature of the cognitive impairment.

If you have a family history of FTD or another neurodegenerative disease, another option is to ask your doctor or a genetic counsellor about clinical genetic testing. Clinical laboratories that are certified to provide these specialised services will analyse a blood DNA sample for changes in the genes known to cause FTD.

As costs of genetic testing may vary, remember to ask whether your medical insurance will cover the costs. A clinical lab may take two weeks or a few months to deliver genetic test results.

Assessing the risk of other family members
If you have already been diagnosed with FTD, genetic testing may help identify the risk of FTD for other family members. If your family history does raise some concerns, the US Association for Frontotemporal Degeneration (AFTD) recommends the following important steps:

• Educate yourself by discussing your concerns with a neurologist/geriatrician.
• Get information about your family history from other family members. Some relatives may be reluctant to discuss this issue, but it’s important to find out as much as you can about the early symptoms of any family member affected with FTD or a related disease, and at what age the symptoms started.
• Ask your doctor to refer you to a genetic counsellor or another medical professional with experience in the genetics of adult neurological conditions.

Treatment

Since the causes of FTD are unknown, it is not possible to prevent, cure or slow its progression. According to the AFTD, while managing the disorder with medication is complicated, many of the symptoms can be treated and behavioural-therapy strategies are an essential supplement to medication.

It recommends that FTD patients with behaviour problems should be referred to academic/specialist centres that specialise in the disorder.

It is crucial to get an accurate FTD diagnosis because several medications used to treat other disorders may be harmful in someone with FTD.

While there are no medications that help with inappropriate behaviour and poor judgement often seen in FTD patients, some drugs such as selective serotonin reuptake inhibitors (SSRIs) may help with some FTD symptoms such as aggression, apathy, anxiety, agitation and depression.

Drugs used to treat Alzheimer’s disease (CHEIs) do not work and may cause harm and are therefore best avoided.

Prevention

While FTD cannot be prevented, there is a great deal of ongoing research by various organisations worldwide.

Outcome

Sadly, FTD has a poor outcome as it progresses steadily and often rapidly. In some people, the progression may be less than two years; in others, it may develop over more than 10 years.

The progressive decline in language and/or behaviour causes significant impairment in a person’s occupational or social functioning. Some people with FTD will eventually need 24-hour care and supervision at home or in a professional-care setting.

When to see a doctor

If you are aged between 50 and 60 years and notice any of the following, see your doctor:

• Language loss – this could be specific, such as being unable to name a familiar everyday object.
• Rigidity or difficulty in walking.
• Muscle weakness.
• If others notice an unexplained change in your behaviour (e.g. lack of concern for other people).

- (Reviewed by Dr Stanley Lipschitz, physician and geriatrician, March 2014)