|Abbreviated Mental Test Score (AMTS)
| Dementia with Lewy bodies
| Frontotemporal dementia
| Corticobasal degeneration
| Creutzfeldt Jakob disease
| Vascular Dementia
| Primary progressive aphasia
| Anti Dementia Drugs
| AIDS Dementia Complex
| Normal Pressure Hydrocephalus
| Acetylcholinesterase inhibitors
| Mental Capacity Act 2005
| Behavioural and Psychological Symptoms of Dementia
Some cases demonstrate intracytoplasmic tau positive inclusion bodies so sometimes termed a tauopathy (also seen with AD)
- Rare form of dementia with anterior temporal and frontal lobe features
- Altered behaviour and disinhibition .
- Age 50-70 years old. Onset mean age is 58
- Mutations in microtubule-binding protein tau.
- Right prefrontal lobe or anterior temporal involvement leads to the typical syndrome.
- Left prefrontal involvement leads to aphasia.
- Neuronal loss and gliosis
- Spongiform degeneration in superficial layers of frontal/temporal lobes
- Pick bodies in 1/4 round intracellular inclusions in small cells
- Pick cells - large ballooned neurons in the superficial cortex
- Tau positive inclusion bodies seen in the cytoplasm
- Corticobasal degeneration - alien limb on the side contralateral to cortical atrophy, apraxia, stiff jerky limb movements
- Behavioural variant FTD
- Primary progressive aphasia: deficits in language and speech. Difficulties with grammar and reading/writing predominate. Fluent (semantic dementia) and non-fluent forms.
- Motor neurone disease type dementia: may precede the diagnosis of MND. Usually men less than 65.
- Behavioural symptoms predominate early. Loss of empathy
- Apathy, Overactive, Impulsive, Overeating, weight gain
- Personality change - inappropriate sexual behaviour
- Socially disinhibited e.g. poor hygiene, unkempt
- Loss of safety awareness, Emotional Lability
- Abnormalities in speech and language may be seen
- Loss of executive functions, Apathetic, Memory loss is later, No insight
- Asymmetrical akinetic rigid. Limb apraxia, Pseudobulbar palsy, Spastic dysarthria
- Vertical gaze palsy and axial rigidity and dystonia and alien hand syndrome or MND
- Dementia tests: FBC, U&E, CRP, LFT, TFT, B12, Folate, Syphilis serology
- CT/MRI may show frontal and temporal loss
- EEG : normal
- MRI: Right prefrontal and anterior temporal atrophy. Atrophy may be asymmetrical. Left frontal/temporal changes in PPA.
- EMG in those with Motor neurone disease type dementia
- SPECT/PET shows anterior perfusion deficits
- No known treatments available. No role for cholinesterase inhibitors or memantine.
- Some symptoms may be helped with SSRIs - depression, hyperorality, compulsiveness
- Management is supportive with social services and support to allow the patient to remain independent.
- Quetiapine may be used to manage behavioural changes