Degenerative diseases of the CNS Flashcards

1
Q

what are common features of neurodegenerative diseases

A
cause unknown
late onset
gradual progression
neuronal loss
structural imaging often normal
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2
Q

what is dementia

A

a syndrome of:

  • progressive impairment of multiple domains of cognitive function in alert patient
  • leading to loss of acquired skills
  • interference in occupational and social role
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3
Q

what is the prevalence of dementia in the UK

A

1,500 per 100,000

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4
Q

what is the link between dementia and age

A

incidence is age dependent

- risk increases with age

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5
Q

what is the difference between young and later onset dementia

A

young onset <65 years
late onset >65 years

some different causes

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6
Q

what are the main causes of young onset dementia

A
  1. alzheimers - 33%
  2. vascular - 15%
  3. frontotemporal - 15%
  4. other - 33%
    - toxic (alcohol)
    - genetic (huntingtons)
    - infection (HIV, CJD- Creutzfeldt-Jakob disease)
    - inflammatory (MS)
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7
Q

what are the main causes of late onset dementia

A
  1. alzheimers - 55%
  2. vascular - 20%
  3. lewy body - 20%
  4. others - 5%
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8
Q

what are some treatable causes of dementia

A

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

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9
Q

what are some mimics of dementia

A

Hydrocephalus (NPH)
Tumour
Depression: “pseudodementia”

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10
Q

how can dementia be diagnosed

A

history - independent witness
- type of deficit, progression, risk factors, FH

examination - cognitive function, neurological, vascualr

investigations - bloods, CT/MRI
CSF, EEG, functional imaging, gentics (biopsy)

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11
Q

what domains make up cognitive function

A

Memory
attention
language
visuospatial

Behaviour
emotion
executive function

Apraxias
agnosias

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12
Q

how can you examine cognitive function

A

mini-mental (MMSE)
montreal (MOCA)
neurophysiological assessment

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13
Q

when can a definitive dementia diagnosis be made

A

post mortem

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14
Q

what clues can help determine the cause of the dementia

A

the type of cognitive deficit

speed of progression

other neurological signs

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15
Q

what types of dementia can be diagnosed by speed of progression

A

CJD - rapid progression

vascular - stepwise progression

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16
Q

what types of dementia can be diagnosed by other neurological signs

A

Huntington’s - abnormal movement

Lewy body - parkinsonism

CJD - myoclonus

17
Q

what is alzheimers

A

the commonest neurodegenerative condition

mean onset 70yrs

involves tempro-parietal dementia

18
Q

what is the presentation of alzheimers temporo-parietal dementia

A

Early memory disturbance

Language and visuospatial problems

Personality preserved until later

19
Q

what is the presentation of frontotemporal dementia

A

Early change in personality / behaviour

Often change in eating habits

Early dysphasia

Memory / visuospatial relatively preserved

20
Q

what is the presentation of vascular dementia

A

mixed picture

stepwise decline

21
Q

what is the type of treatment for dementia

A

supportive treatment

non-pharmacological

pharmacological

22
Q

give examples of non-pharmacological management

A

Information & support, dementia services

Occupational therapy

Social work / support / respite / placement

Voluntary organisations

23
Q

give examples of symptoms that could use pharmacological treatment

A

Insomnia

Behaviour (care with antipsychotics)

Depression

24
Q

what is the specific treatment for alzheimers (+/-lewy body) dementia

A

cholinesterase inhibitors
- donepezil, galantamine

NMDA antagonist
- memantine

25
what is the specific treatment for frontotemporal dementia
none
26
what is the specific treatment for vascular dementia
? decrease vascular risk factors | BUT no good evidence
27
what is parkinsonism
a clinical syndrome of 2 or more: Bradykinesia (slowness of movement) Rigidity (stiffness) Tremor (shakiness) Postural instability (unsteadiness / falls)
28
where is the pathology of parkinsonism
basal ganglia predominantly dopamine loss
29
what can cause parkinsons
idiopathic parkinsons disease - dementia with lewy bodies drug induced - dopamine antagonists vascular parkinsonism - lower half parkinson plus syndrome - multiple system atrophy - progressive supranuclear palsy/corticobasal degeneration
30
what are lewy bodies
abnormal aggregates of protein that develop inside nerve cells
31
what is the relation between age and parkinsons
age dependent incidence increases from 60-85 incidence decreases from 85+
32
how is a definitive diagnosis of parkinsons made
post mortem
33
how is parkinsons diagnosed
Clinical: - Bradykinesia + ≥1 tremor, rigidity, postural instability - No other cause / atypical features supported by asymmetric rest tremor good response to dopamine replacement therapy functional imaging - dopamine transporter SPECT
34
what is the early medical treatment of parkinsons
levodopa + COMT inhibitor (entacapone) MAO-B inhibitor - selegiline - rasagiline - safinamide dopamine agonists - ropinirole - pramipexole - rotigotine
35
what are drug induced later complications of parkinsons
Motor fluctuations - levodopa wears off Dyskinesias - involuntary movements (levodopa) Psychiatric - hallucinations, impulse control
36
what are non-drug induced later complications of parkinson s
Depression (20%) Dementia (~50% after 10 yrs) Autonomic: BP, bladder, bowel Speech, swallow Balance
37
what is the late treatment of parkinsons
drugs: 1. prolong levodopa half life - MAO-B inhibitors - COMT inhibitor - slow release levodopa 2. add oral dopamine agonist 3. continuous infusion (apomorphone, duodopa) fucntional neurosurgery - deep brain stimulation allied health professionals +/- care package