|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
A very rare cause of adult-onset parkinsonism usually presents with unilateral arm rigidity dyspraxia and dystonia
- Also called Corticobasal ganglia degeneration
- A movement disorder with dementia and speech affected - Alien limb
- Probably a variant of frontotemporal dementia
- Tauopathy which affects nigrostriatal tracts and subcortical structures
- Areas include the thalamus, subthalamic nuclei, pallidum, red nucleus, dentate nucleus
- Large pale ballooned neurons in the basal ganglia
- An initially unilateral parkinsonism + cortical signs
- The dyspraxia is typically evidenced by the difficulty
in copying unfamiliar hand positions and/or difficulty in
- Alien limb phenomenon where the hand makes involuntary complex movements.
- Dystonic hand wrist and thumb flexed across palm which is apraxic and useless
- Asymmetrical akinetic-rigid syndrome, jerky dystonias
- Myoclonus, supranuclear gaze palsy in horizontal and lateral directions.
- Cortical Apraxias, Dysphasias, Alien limb phenomenon
- Disinhibition, depression, dementia
- FBC, U&E, ESR, CRP, LFT, TFT B12 folate, ECG, CXR
- MRI - Frontoparietal cortical atrophy often contralateral to the affected side. Asymmetrical parietal atrophy.
- PET scanner: reduced uptake in basal ganglia of 18-F flourodopa
- Management is supportive with social services and support to allow the patient to remain independent. No role for cholinesterase inhibitors or memantine.
- Quetiapine may be used to manage behavioural changes but regular review to assess ongoing need. Increased attention to reducing the use of antipsychotics in dementia where possible.
- Variable (often no) response to L-Dopa.
- Dystonias may respond to clonazepam, valproate, anticholinergics, baclofen, botulinum toxin, benzodiazepines.
- However these agents can impair cognition and should be used with caution.
- PEG tube for severe dysphagia