Rare Diseases Day: Frontotemporal dementia

For Rare Diseases Day last year, we shared Dennis Frost’s story about his frontotemporal dementia diagnosis experience, and his journey to become a dementia advocate. This year, the recent announcement of celebrity actor Bruce Willis’s diagnosis with frontotemporal dementia is helping to generate more awareness about this condition.

What is frontotemporal dementia

Frontotemporal dementia describes a collection of symptoms caused by disorders affecting the brain. It causes gradual loss of brain tissue in the frontal (front) and temporal (side) lobes or regions of the brain. Frontotemporal dementia is relatively rare, but it is one of the most common causes of dementia in people under the age of 65.

What are the subtypes and their symptoms?

Symptoms of frontotemporal dementia are often different from other dementias.  Frontotemporal dementia can affect behaviour, personality, language and movement. Memory can remain unaffected in the early stages.

Clinicians may take longer to recognise, diagnose and treat this type of dementia. People with frontotemporal dementia often have to educate others (including health professionals) about their condition.

Semantic variant of primary progressive aphasia

With this subtype of frontotemporal dementia, the person starts to lose the meanings of words. People with semantic dementia have trouble expressing themselves with words, understanding language, reading and writing. They may benefit from speech therapy with experts in improving communication (see 3.11 Speech and occupational therapy).

Progressive non-fluent aphasia

With this subtype, the person has difficulty speaking fluently. People with progressive, non-fluent aphasia have trouble expressing themselves and following conversations. They may also benefit from speech therapy with experts in improving communication (see 3.11 Speech and occupational therapy).

Behavioural-variant frontal dementia

With this subtype, the person’s behaviour, mood or personality changes. People with behavioural-variant frontal dementia may become apathetic, socially disinhibited or socially unaware. They often have trouble with judgement, making decisions or problem solving. They might develop new habits that they do repeatedly, including eating and drinking different foods or drinks. Treatment of symptoms may involve behavior modification, or medications for symptoms such as aggression, agitation, or dangerous behaviors. Psychological therapy may be helpful and it is important to make  contact with and get advice from Dementia Australia and/or Dementia Support Australia.