Degenerative disease of the CNS Flashcards

1
Q

What is common features of neruodegerative diseases

A

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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2
Q

What is dementia

A

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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3
Q

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

A

Alzheimers
Vascular
Lewy body

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4
Q

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

A

Alzheimer’s

Vascular

Frontotemporal

Toxic (alcohol)

Genetic (Huntington’s)

Infection (HIV, CJD)

Inflammatory (MS)

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5
Q

What are treatable causes of dementia

A

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

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6
Q

What are treatable disorders that mimic dementia

A

Hydrocephalus
Tumour
Depression: pseudo-dementia

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7
Q

What information do you gather from the patient when diagnosing dementia

A

type of deficit
progression
risk factors
family history

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8
Q

What is examined when diagnosing a patient with dementia

A

Cogntive function
neurological examination
Vascular examination

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9
Q

What is the routine investigation for dementia

A

Bloods
CT
MRI

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10
Q

What are further investigation for dementia

A
CSF
EEG
Functional imaging 
Genetic 
Biopsy
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11
Q

What is examined in cognitive function

A

Memory,

Attention,

Language,

Visuospatial,

Behaviour,

Emotion,

Executive function

Apraxias - difficulty with motor planning

Agnosia - inability to interpretate sensations and recognise

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12
Q

What are the screening tests used in examining cognitive functions

A

Mini- mental (MMSE)

Montreal (MOCA)

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13
Q

What assessment take place when examining cognitive function

A

Neuropsychological assessment

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14
Q

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

A

CJD dementia

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15
Q

A stepwise progression is usually as signs to what kind dementia

A

Vascular dementia

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16
Q

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

A

Huntingtons dementia

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17
Q

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

A

Lewy body dementia

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18
Q

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

A

CJD dementia

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19
Q

What is the commonest neurodegenerative condition

A

alzheimers disease

mean age onset >70years

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20
Q

What is the pathology of alzheimers disease

A

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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21
Q

What is the affect of alzheimers

A

Tempo -parietal dementia

22
Q

What temp-parietal dementia associated with

A
  • early memory disturbance
  • language and visuospatial problems
  • personality preserved until later
23
Q

What is frontotemporal dementia associated with

A

Early change in personality / behaviour

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

24
Q

What is vascular dementia associated with

A

A mixed picture of symptoms with a stepwise decline

25
Q

What is the non pharmacological symptomatic treatment of dementia

A

Information & support, dementia services

Occupational therapy

Social work / support / respite / placement

Voluntary organisations

26
Q

What symptoms can be pharmacological treatment for dementia

A

Insomnia

Behaviour (care with antipsychotics)

Depression

27
Q

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

A

Cholinesterase inhibitors

NMDA antagonist

28
Q

how does Cholinesterase inhibitors work in treating Alzheimers

A

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

29
Q

What is three examples of Cholinesterase inhibitors

A

Donepezil, rivastigmine, galantamine

30
Q

How does NMSA antagonist work in the treatment of alzheimers

A

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

31
Q

What is an example of NMSA antagonist

A

memantine

32
Q

What is the specific treatment for frontotemporal dementia

A

Nothing

33
Q

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

A

Bradykinesia (slowness of movement)

Rigidity (stiffness)

Tremor (shakiness)

Postural instability (unsteadiness / falls)

34
Q

what is the pathology of Parkinsonism

A

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

35
Q

What can also be present in parkinsonism

A

Lewy bodies

36
Q

What is the causes of parkinsonism

A

Idiopathic Parkinson’s disease
-Dementia with Lewy bodies

Drug-induced
(e.g. dopamine antagonists)

Vascular parkinsonism (lower-half)

Parkinson’s plus syndromes

37
Q

What parkinson plus syndromes

A

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

38
Q

Give examples of parkinson plus syndromes

A

Multiple system atrophy

Progressive supranuclear palsy

corticobasal degeneration

39
Q

What is the cause of vascular parkinsonsim

A

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

40
Q

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

A

Bradykinesia + ≥1 tremor, rigidity, postural instability

No other cause / atypical features

Slowly progressive (> 5-10 yrs)

41
Q

What supports your diagnosis of parkinson disease

A

good response to dopamine replacement treatment

42
Q

What signs make it less likely to be parkinson disease

A

rapid progression,

symmetrical,

lack of rest tremor,

poor response to treatment,

early falls,

early dementia,

other abnormal neurological signs

43
Q

What specific symptoms indicates the diagnosis to be parkinson s

A

asymmetric rest tremor

44
Q

What imaging technique is used for the diagnosis of parkinson

A

Domapine transporter SPECT

45
Q

What does early medication of parkinsons disease involve

A

Increasing dopaminergeric neurone

46
Q

What is examples of how dopaminergeric neurone is increased

and used in the treatment of Parkinson’s

A

Levodopa

COMT inhibitor

MAO-B inhibitor

Dopamine agonist

47
Q

What is three examples of MAO-B inhibitors

A

selegiline
rasagiline
safinamide

48
Q

What is three examples of dopamine agonists

A

ropinirole
pramipexole
rotigotine

49
Q

What are drug induced later complications of parkinson s

A

Motor fluctuations - levodopa wears off

Dyskinesias - involuntary movements (due to levodopa)

Psychiatric - hallucinations, impulse control

50
Q

What is non motor later complications of Parkinson’s

A

Depression

Dementia

Autonomic: BP, bladder, bowel

Speech, swallow

Balance

51
Q

What is the is the late treatment for parkinsons

A

prolong levodopa half life
add oral dopamine agonist

Functional neurosurgery - deep brain stimulation

allied health professional +/- care package