Degenerative disease of the CNS Flashcards by Sarah Anderson

What is common features of neruodegerative diseases

aetiology largely unknown
(mendelian genetic cases rare, often younger onset)

usually late onset

gradual progression

neuronal loss (specific neuropathology)

structural imaging often normal (atrophy)

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What is dementia

A syndrome consisting of progressive impairment of multiple domains of cognitive function in alert patient leading to loss of acquired skills and interference in occupational and social role

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What is three causes of late onset dementia (>65years)

Alzheimers
Vascular
Lewy body

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What is the potentially causes of younger onset (<65 years) dementia

Alzheimer’s

Vascular

Frontotemporal

Toxic (alcohol)

Genetic (Huntington’s)

Infection (HIV, CJD)

Inflammatory (MS)

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What are treatable causes of dementia

Vitamin deficiency - B12
Endocrine - thyroid disease
Infective - HIV, syphilis

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What are treatable disorders that mimic dementia

Hydrocephalus
Tumour
Depression: pseudo-dementia

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What information do you gather from the patient when diagnosing dementia

type of deficit
progression
risk factors
family history

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What is examined when diagnosing a patient with dementia

Cogntive function
neurological examination
Vascular examination

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What is the routine investigation for dementia

Bloods
CT
MRI

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What are further investigation for dementia

CSF
EEG
Functional imaging 
Genetic 
Biopsy

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What is examined in cognitive function

Memory,

Attention,

Language,

Visuospatial,

Behaviour,

Emotion,

Executive function

Apraxias - difficulty with motor planning

Agnosia - inability to interpretate sensations and recognise

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What are the screening tests used in examining cognitive functions

Mini- mental (MMSE)

Montreal (MOCA)

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What assessment take place when examining cognitive function

Neuropsychological assessment

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Rapid progression dementia is usually a sign of what type of dementia

CJD dementia

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A stepwise progression is usually as signs to what kind dementia

Vascular dementia

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Demential with abnormal movement is usually a sign to what kind of dementia

Huntingtons dementia

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Demential with parkinsonism is usually a sign to what kind of dementia

Lewy body dementia

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Demential with myoclonus is usually a sign to what kind of dementia

CJD dementia

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What is the commonest neurodegenerative condition

alzheimers disease

mean age onset >70years

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What is the pathology of alzheimers disease

proteins build up in the brain to form structures called ‘plaques’ and ‘tangles’ leading to the loss of connections between nerve cells, and eventually to the death of nerve cells and loss of brain tissue

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What is the affect of alzheimers

Tempo -parietal dementia

What temp-parietal dementia associated with

early memory disturbance
language and visuospatial problems
personality preserved until later

What is frontotemporal dementia associated with

Early change in personality / behaviour

Often change in eating habits
Early dysphasia
Memory / visuospatial relatively preserved

What is vascular dementia associated with

A mixed picture of symptoms with a stepwise decline

What is the non pharmacological symptomatic treatment of dementia

Information & support, dementia services Occupational therapy Social work / support / respite / placement Voluntary organisations

What symptoms can be pharmacological treatment for dementia

Insomnia Behaviour (care with antipsychotics) Depression

What is the specific treatment for Alzheimer’s (+/- Lewy body dementia)

Cholinesterase inhibitors NMDA antagonist

how does Cholinesterase inhibitors work in treating Alzheimers

inhibits the acetylcholinesterase enzyme from breaking down acetylcholine, thereby increasing both the level and duration of action of the neurotransmitter acetylcholine leading to small symptomatic improvement in cognition

What is three examples of Cholinesterase inhibitors

Donepezil, rivastigmine, galantamine

How does NMSA antagonist work in the treatment of alzheimers

regulating the activity of glutamate, an important neurotransmitter in the brain involved in learning and memory

What is an example of NMSA antagonist

memantine

What is the specific treatment for frontotemporal dementia

Nothing

Parkinsonism is a clinical syndrome with 2 or more of what four symptoms

Bradykinesia (slowness of movement) Rigidity (stiffness) Tremor (shakiness) Postural instability (unsteadiness / falls)

what is the pathology of Parkinsonism

Occurs in the basal ganglia due to loss of pigmented dopaminergic neurons of the substantia nigra

What can also be present in parkinsonism

Lewy bodies

What is the causes of parkinsonism

Idiopathic Parkinson’s disease -Dementia with Lewy bodies Drug-induced (e.g. dopamine antagonists) Vascular parkinsonism (lower-half) Parkinson’s plus syndromes

What parkinson plus syndromes

Group of neurodegenerative diseases featuring the classical features of Parkinson's disease

Give examples of parkinson plus syndromes

Multiple system atrophy Progressive supranuclear palsy corticobasal degeneration

What is the cause of vascular parkinsonsim

"atypical parkinsonism" in which parkinsonian symptoms are produced by one or more small strokes

What is the clinical presentation that leads to the diagnosis is parkinson

Bradykinesia + ≥1 tremor, rigidity, postural instability No other cause / atypical features Slowly progressive (> 5-10 yrs)

What supports your diagnosis of parkinson disease

good response to dopamine replacement treatment

What signs make it less likely to be parkinson disease

rapid progression, symmetrical, lack of rest tremor, poor response to treatment, early falls, early dementia, other abnormal neurological signs

What specific symptoms indicates the diagnosis to be parkinson s

asymmetric rest tremor

What imaging technique is used for the diagnosis of parkinson

Domapine transporter SPECT

What does early medication of parkinsons disease involve

Increasing dopaminergeric neurone

What is examples of how dopaminergeric neurone is increased | and used in the treatment of Parkinson's

Levodopa COMT inhibitor MAO-B inhibitor Dopamine agonist

What is three examples of MAO-B inhibitors

selegiline rasagiline safinamide

What is three examples of dopamine agonists

ropinirole pramipexole rotigotine

What are drug induced later complications of parkinson s

Motor fluctuations - levodopa wears off Dyskinesias - involuntary movements (due to levodopa) Psychiatric - hallucinations, impulse control

What is non motor later complications of Parkinson's

Depression Dementia Autonomic: BP, bladder, bowel Speech, swallow Balance

What is the is the late treatment for parkinsons

prolong levodopa half life add oral dopamine agonist Functional neurosurgery - deep brain stimulation allied health professional +/- care package