1st February 2024

Background

It is a rare form of dementia. It is a progressive neurodegenerative cognitive decline, which constitutes approximately around 2% of all cases of dementia. It is a condition characterised by deterioration of personality and cognitive issues. There is evidence of atrophy in the frontal and temporal lobes.

It is generally characterised by difficulties in thinking and behaviour. They usually present with unusual and unwarranted behaviours, emotional problems, difficulties in communication (speech and language), motivation and preservative behaviours.

Epidemiology

• Unlike Alzheimer’s dementia, this is higher in males.
• It is a disease of middle-aged and older people.
• It constitutes 2% of all cases of dementia

Pathophysiology

There is prominent frontal and temporal lobar atrophy.

FTD is a broad term that includes a few subtypes:

1. Behavioural FTD or frontal variant FTD (bvFTD) – 60% of patients with FTD have this type of behavioural FTD. It causes changes in judgement, personality, behaviour and emotions. Behavioural changes are seen initially.
2. Semantic dementia or primary progressive aphasia: (sv FTD or PPA): Here, mainly there is language difficulty (speech production is spared). However, during early stages, the other aspects such as judgement, behaviour and personality are not disabled; during later stages, they are impacted.
3. Progressive non-fluent Aphasia (PNFA): Here mainly there is language difficulty- mainly speaking. Grammar is affected more so in PNFA.
4. Progressive Supranuclear palsy: This involves the brainstem, frontal cortex and basal ganglia.
5. Corticobasal degeneration: This is a neurodegenerative disorder classified as an atypical Parkinsonian syndrome
6. FTD with Motor Neuron disease: Early cognitive and behavioural changes with MND

Risk factors

• No specific risk factors. 
• Men and women are equally affected
• More likely to run in families