mvFTD

Question 1

mvFTD

Answer 1

Motor symptoms usually in addition to cognitive and behaviour symptoms

Variants are;
- Corticobasal syndrome (CBS)
- progressive supranuclear palsy (PSP)
- Motor neuron disease (MND)

Question 2

CBS

Answer 2

Corticobasal syndrome

Symptoms
- parkinsonism, apraxia, myoclonus, dystonia, alien hand
- cognitive impairment mainly in language and visuospatial functions
- patients may also meet criteria naPPA
- cognitive impairments can precede motor symptoms

Neuroimaging: Atrophy premotor cortex, parietal cortex, insula, basal ganglia

Question 3

PSP

Answer 3

Progressive supranuclear palsy (PSP)

Symptoms
- supranuclear gaze palsy (slow vertical saccades), instability and falls, parkinsonism (bradykinesia, rigidity)
- cognitive impairments mostly in EF, processing speed or language production
- behavioural symptoms frequent, e.g. apathy, disinhibition
- PSP can progress into bvFTD

Neuroimaging: typically atrophy in midbrain, subcortical gray matter, frontal lobe

Question 4

MND

Answer 4

Motor neuron disease

Symptoms:
- upper motor neuron signs (e.g. spasticity) and lower motor neuron signs (e.g. weakness, atrophy, fasciculations).
- Neuropsychological symptoms vary
- Behavioral symptoms generally similar to bvFTD

Neuroimaging: atrophy motor areas in frontal lobe