QuestionSBA
QuestionChoice
Frontotemporal dementia (FTD) is characterised by changes in behaviour, personality, and language. The patient in this scenario presents with symptoms that are consistent with FTD, due to degeneration of neurons in the frontal and temporal lobes. MCA is not a specific tool to diagnose FTD, it only indicates the patient's cognitive function which is normal in this case. The anterior temporal lobes are particularly affected in FTD, and this is reflected in the MRI finding of temporal lobe atrophy.
62008
a
Frontotemporal dementia
QuestionChoice
Parkinson's dementia occurs more than one year after the diagnosis of Parkinson's disease, which is not the case here. On top of that, it is characterised by three cardinal features: parkinsonism in extremities, visual hallucinations, and fluctuations in lucidity. MRI findings in Parkinson's dementia are atrophy of the hippocampus and the amygdala, and decreased gray matter volume in the frontal and temporal lobes.
62012
e
Parkinson's dementia
QuestionChoice
Alzheimer's disease is characterised by a gradual onset (over several years) of memory impairment, which is followed by difficulties in language and executive function. It should be suspected in adults who demonstrate long-lasting problems with spatial navigation and visual awareness, and usually manifests as wandering or becoming lost.
62010
c
Alzheimer's disease
QuestionChoice
Dementia with Lewy bodies (DLB) is characterised by initial cognitive deterioration, prominent deficits in attention, visual hallucinations, fluctuating cognitive function, followed by extrapyramidal symptoms such as rigidity, bradykinesia, gait instability, and autonomic dysfunction which develop within one year of onset.
62009
b
Lewy-body dementia
QuestionChoice
Vascular dementia usually presents in patients with history of multiple cerebrovascular events, either transient ischaemic attacks or strokes. Typically there is a step-wise deterioration of cerebral function with each event. It should be considered in those with a history of cerebrovascular disease with global impairment of cognition.
62011
d
Vascular dementia
Concept
Chapter
# Summary

Frontotemporal dementia (FTD) is a neurodegenerative disorder primarily affecting the frontal and temporal lobes of the brain. It characteristically presents at a younger age compared to other forms of dementia, with early personality changes and language impairments being key signs and symptoms. Investigations often involve neuroimaging and genetic testing, particularly in cases of suspected hereditary conditions. The management of FTD includes symptom-targeted strategies, with behavioural and psychological interventions playing a crucial role. One specific cause of FTD is Pick's disease, characterised by the accumulation of TAU proteins in neurons, identified post-mortem.

# Definition

Frontotemporal dementia (FTD) is a heterogeneous group of neurodegenerative disorders characterised by progressive atrophy of the frontal and/or temporal lobes of the brain, leading to impairments in behaviour, personality, language, and motor functions.

# Epidemiology

FTD is one of the most common forms of early-onset dementia, with the age of onset typically occurring during the sixth or seventh decade of life. The prevalence and incidence rates of FTD vary globally due to genetic and environmental factors.

# Aetiology

The precise aetiology of FTD is unknown; however, the condition is thought to result from the atrophy of the frontal and temporal lobes of the brain. Genetic mutations can play a role, particularly in familial cases. One specific cause is Pick's disease, characterised by the presence of "Pick's bodies" - accumulations of TAU protein within neurons. FTD is associated with ALS - 40% of people with FTD carry same genetic mutation which is commonest cause of ALS.

# Signs and Symptoms

FTD typically presents with:

- Personality changes: Patients often exhibit disinhibited behaviour and apathy early on.
- Language impairments: Early language difficulties can be present, varying from mild problems with word finding to severe aphasia.
- Cognitive decline: Although memory may be relatively preserved in the early stages, executive function is frequently impaired.
- Motor abnormalities: Some patients may develop features of motor neuron disease, including muscle weakness and dysarthria.

# Differential Diagnosis

The differential diagnosis for FTD includes other forms of dementia and neurological disorders, such as:

- Alzheimer's disease: Characterised by memory loss, confusion, trouble handling money and paying bills, and mood changes. Pathology is defined by the presence of beta-amyloid plaques and neurofibrillary tangles.
- Vascular dementia: Presents with step-wise cognitive decline, focal neurological signs, and evidence of cerebrovascular disease on neuroimaging.
- Creutzfeldt-Jakob disease: Rapidly progressive dementia with neurological signs such as myoclonus, ataxia, and visual disturbances. Periodic sharp wave complexes on EEG are suggestive.
- Primary progressive aphasia: Characterised by gradual, insidious decline in language abilities with relative preservation of other cognitive functions, at least in the early stages.

# Investigations

Diagnosis of FTD often involves:

- Neuroimaging: Structural imaging (MRI or CT) can show atrophy of the frontal and/or temporal lobes. Specifically, MRI can show focal gyral atrophy and knife-blade appearance.
- Genetic testing: This may be used in cases where an inherited form of FTD is suspected, especially if there is a strong family history.

# Management

There is currently no cure for FTD; management focuses on alleviating symptoms and providing support. Strategies include:

- Behavioural interventions: Counselling, behaviour modification strategies, and caregiver support can help manage behavioural symptoms.
- Pharmacotherapy: Selective serotonin reuptake inhibitors (SSRIs) and antipsychotics can help control behavioural symptoms, though their use must be carefully balanced against side effects.
- There is no good evidence that the cholinesterase inhibitors or memantine help people with FTD, including Pick's disease. In some people they may make symptoms worse.
- Supportive care: Speech and language therapy, physiotherapy, and occupational therapy can help manage the impacts of the disease on daily functioning.
  
# NICE Guidelines

[Click here to see information on NICE
CKS on dementia](https://cks.nice.org.uk/topics/dementia/)
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Frontotemporal dementia
Topic
18
Psychiatry
12463
A 65-year-old man presents to the GP clinic with his wife, complaining of binge eating, impulsiveness, and increased sexual activity. His wife states that people have a hard time understanding his speech, and he often forgets the names of his close friends. Otherwise, there are no other memory problems. He can care for himself, manage his accounts, and mingle well. His past and family history are insignificant.

Vital signs and physical and neurological examination are unremarkable. The Montreal Cognitive Assessment (MCA) score is 26/30, and a recent brain MRI shows asymmetrical anterior left temporal atrophy.

What is the most likely diagnosis?
a