Frontotemporal Dementia

Question 1

Define

Answer 1

Frontotemporal Dementia (2% of cases)

Characterised by progressive degeneration of the frontal and/or temporal lobes

Insidious onset and gradual progression, typically presenting in middle age

Some cases may present very early (30s) or very late (90s)

Often involve mutations in genes encoding for tau and progranulin

Pick’s disease is a form of frontotemporal dementia, have Pick’s bodies (cytoplasmic inclusion bodies of tau protein)

Rarer causes: Parkinson’s disease dementia, Progressive supranuclear palsy, Huntington’s disease, Prion disease, Normal pressure Hydrocephalus, Chronic subdural haematoma, Benign tumours, Metabolic and endocrine disorders, Vitamin deficiencies, Infections

Risk factors: age, mild cognitive impairment, learning difficulties, genetics, cardiovascular disease risk factors, Parkinson’s disease, stroke, depression, heavy alcohol consumption

Question 2

Symptoms

Answer 2

Personality change and behavioural disturbance (apathy or social/ sexual disinhibition)- insidious development

Early loss of insight

Can present fairly young (50-60s)

Memory loss and disorientation (can occur later on in Pick’s disease)

Memory tends to be affected much later (unlike in AD where it may be the first thing to be affected)

Pick’s disease: disinhibition, inattention, antisocial behaviour, Pick’s bodies, atrophy of the frontal and temporal lobes (knife-blade atrophy)

Question 3

Management

Answer 3

Antidepressants (treat frontal lobe syndrome)

• SSRIs may help control the loss of inhibitions, overeating and compulsive behaviours seen in some people
• (Antipsychotics are considered only in very severe cases if SSRIs haven’t worked e.g., risk of harm to self/others)

Agitation, Aggression, Distress and Psychosis

Conservative interventions:
- Explore reasons for distress
- And check for and address clinical or environmental causes

Pharmacological treatment:
- Antipsychotics- PO Risperidone or Haloperidol
- Only offered if at risk of harming themselves or others
- OR experiencing agitation, hallucinations or delusions that are causing them severe distress

Antipsychotics should be AVOIDED in DLB or Parkinson’s dementia as it can worsen motor features

Question 4

Prognosis

Answer 4

o Death in 5-10 years