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NEURO > Degenerative diseases of the CNS > Flashcards

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
Q

what is the specific treatment for frontotemporal dementia

A

none

26
Q

what is the specific treatment for vascular dementia

A

? decrease vascular risk factors

BUT no good evidence

27
Q

what is parkinsonism

A

a clinical syndrome of 2 or more:

Bradykinesia (slowness of movement)

Rigidity (stiffness)

Tremor (shakiness)

Postural instability (unsteadiness / falls)

28
Q

where is the pathology of parkinsonism

A

basal ganglia

predominantly dopamine loss

29
Q

what can cause parkinsons

A

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
Q

what are lewy bodies

A

abnormal aggregates of protein that develop inside nerve cells

31
Q

what is the relation between age and parkinsons

A

age dependent

incidence increases from 60-85

incidence decreases from 85+

32
Q

how is a definitive diagnosis of parkinsons made

A

post mortem

33
Q

how is parkinsons diagnosed

A

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
Q

what is the early medical treatment of parkinsons

A

levodopa + COMT inhibitor (entacapone)

MAO-B inhibitor

  • selegiline
  • rasagiline
  • safinamide

dopamine agonists

  • ropinirole
  • pramipexole
  • rotigotine
35
Q

what are drug induced later complications of parkinsons

A

Motor fluctuations - levodopa wears off

Dyskinesias - involuntary movements (levodopa)

Psychiatric - hallucinations, impulse control

36
Q

what are non-drug induced later complications of parkinson s

A

Depression (20%)

Dementia (~50% after 10 yrs)

Autonomic: BP, bladder, bowel

Speech, swallow

Balance

37
Q

what is the late treatment of parkinsons

A

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

NEURO (51 decks)

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