Frontotemporal Dementia

Symptoms

Signs and symptoms of frontotemporal dementia can be different from one individual to the next. Signs and symptoms get progressively worse over time, usually over years. Clusters of symptom types tend to occur together, and people may have more than one cluster of symptom types, which include:

• Behavioural changes - Increasingly inappropriate social behaviour such as:
  • Loss of empathy and other interpersonal skills, such as having sensitivity to another's feelings
  • Lack of judgment
  • Loss of inhibition
  • Lack of interest (apathy), which can be mistaken for depression
  • Repetitive compulsive behaviour, such as tapping, clapping or smacking lips
  • A decline in personal hygiene
  • Changes in eating habits, usually overeating or developing a preference for sweets and carbohydrates
  • Eating inedible objects
  • Compulsively wanting to put things in the mouth
• Speech and language problems – Some subtypes of frontotemporal dementia lead to language problems or impairment or loss of speech. Primary Progressive Aphasia (PPA), semantic dementia and progressive agrammatic (non-fluent) aphasia are all considered to be frontotemporal dementia, such as:
  • Increasing difficulty in using and understanding written and spoken language, such as having trouble finding the right word to use in speech or naming objects
  • Trouble naming things, possibly replacing a specific word with a more general word such as "it" for pen
  • No longer knowing word meanings
  • Having hesitant speech that may sound telegraphic
  • Making mistakes in sentence construction
• Movement disorders – Rarer subtypes of frontotemporal dementia are characterized by problems with movement, similar to those associated with Parkinson's disease or amyotrophic lateral sclerosis (ALS). Movement-related problems may include:
  • Tremor
  • Rigidity
  • Muscle spasms
  • Poor coordination
  • Difficulty swallowing
  • Muscle weakness
  • Inappropriate laughing or crying

Causes

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink. In addition, certain substances accumulate in the brain. What causes these changes is usually unknown.

There are genetic mutations that have been linked to frontotemporal dementia. But more than half of the people who develop frontotemporal dementia have no family history of dementia.

Recently, researchers have confirmed shared genetics and molecular pathways between frontotemporal dementia and amyotrophic lateral sclerosis (ALS). More research needs to be done to understand the connection between these conditions, however.

Risk Factors

Your risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.

Diagnosis

The diagnosis of bvFTD (behaviour variant Frontotemporal Dementia) and PPA (Primary Progressive Aphasia) are based on expert evaluation by a doctor who is familiar with these disorders. The type of problems experienced by the patient and the results of neurological exams are the core of the diagnosis. Brain scans such as magnetic resonance imaging (MRI) and glucose positron emission scans are very helpful additional tests, but they must be interpreted in the context of the patient’s history and neurological exam.