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what area is affected early in alzheimers

nucleus basalis of meynert (group of CHOLINERGIC neurones)


what is the treatments for alzheimers

acetylcholinesterase inhibitors- donzepezil, galantamine, rivastigime (monotherapies) for milf to mod
memantine (NMDA receptor antagonist) for severe/ AChEi CI


what mutation= 100% penetrance of huntingtons

40 or more CAG repeats


what types of inheritance does huntingtons have

genetic anticipation
autosomal dominant


what are parkinson plus syndromes

diseases that cause parkinsonism + other symptoms:
- multi system atrophy
- dementia with lewey bodies
- progressive supranuclear palsy
- corticobasal degeneration


what is multi system atrophy

group of disorders that progressively affect the central and autonomic nervous by system damaged oligodentrocytes (myelination) by accumulation of alpha synuclein

two types- MSA-P: characterised by parkinsons motor symptoms, deteriorates faster. MSA-C: cerebellar, lack of coordination and balance, intention tremor, slurred speech and difficulty swallowing (dysphasia)

both types characterised by autonomic symptoms- orthostatic hypotension, constipation, increased sweating, sleep apnoea, sexual dysfunction


what protein is found in dementia with lewey bodies

alpha synuclein


what are the symptoms of DLB

cognitive impairments, visual hallucinations, inability to focus, inflexible thinking and paranoia. DLB patients initially have these cognitive symptoms but also develop motor symptoms similar to PD such as bradykinesia, tremor, rigidity, and shuffling walk. Similar to PD, DLB patients also have REM sleep behavior disorder in which they act out their dreams.


what is progressive supranuclear palsy

chronic neurodegenerative syndrome which affects cognition, eye movements and posture


what are the symptoms of progressive supranuclear palsy

supranuclear (primarily vertical) gaze palsy
extrapyramidal - akinesia
cognitive dysfunction
postural instability

vague fatigue, HAs, arthralgia, dizziness, depression, modd changes, progressive dementia


what is a supranuclear palsy

the inability to look in a particular direction as a result of cerebral impairment:
-frontal lobe (frontal eye field)-> pontine horizontal gaze centre (PPRF) -> abducens nucleus-> lateral rectus
-occipital cortex-> oculomotor nucleus -> CN3 and medial longitudinal fasciulus -> pontine horizontal gaze centre

loss of voluntary movements of eye
as brainstem intact, all reflex conjugate eye movements normal


how do you distinguish a vertical gaze palsy from a cranial nerve palsy

both eyes affected
pupils unequal but fixed
no diplopia
reflexes intact


what is corticobasal degeneration

where multiple areas of the brain atrophy
begins around 60
start unilateral then go to other side
- poor coordination, rigidity, impaired balance, dysphagia
-personality changes, inappropriate behaviours, compulsive behaviours


what causes most postural tremors

essential- autosomal dominant inheritance, gets better when you drink alcohol


what is a treatment for an essential tremor



what does an intention tremor suggest

cerebellar diseases


how does huntingtons usually present

strange movements and behavioural changes


what is sydenham chorea

form of chorea that is more common in females adolescents and linked to rheumatic fever (group A beta haemolytic strep infection)


what does myoclonus look like

brief electric shock like movements

e.g. 20 y/o guy gets milk but drops it= juveline myoclonic epilepsy


why is alzheimers linked to downs syndrome

alzheimers cause by beta amyloid precursor protein (insoluble, forms plaques which disrupt neuronal signalling) and the gene that codes for this is on chromosome 21 (trisomy 21= more production of amyloid= build up of plaques)


loss of dopaminergic neurones in the substantia nigra =?

parkinsons disease


what does the globus paladus alway inhibit

the thalamus - main job of direct pathway is to reduce this inhibition


what is the tremor like in parkinsons

4-6 Hz
pill rolling
starts on one side then becomes bilateral


sudden onset bilateral parkisons with resting tremor= ?

drug induces parkinsonism


name two drugs that can cause drug induced parkinsons

dopamine antagonists: haloperidol, metoclopramide


what is hemiballism

wild movements of one arm/leg caused by an infarct of the subthalamic nucleus (part of the indirect pathway)


how is hemiballism treated

self limiting
treated over a few months with antipsychotics


parkinsonism with orthostatic hypotension and has a poor response to levodopa =?

multi system atrophy


where is the lesion in vascular parkinsons

infarcts in basal ganglia


how do the symptoms of vascular parkinsons differ from normal parkinsons

vascular is more prominent in the lower limbs, resting tremor in legs