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Systems: Neurology AB > Degenerative Diseases of the CNS > Flashcards

Degenerative Diseases of the CNS Flashcards

1
Q

What common features are there of neurodegenerative 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

Define 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 the incidence and prevalence of dementia?

A
  • Incidence 200 per 100,000
  • Prevalence 1,500 per 100,000
  • > 750,000 patients in UK
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4
Q

What is the financial cost of dementia?

A
  • £20 billion/year (£6 billion by carers)

- 66% of those in care homes

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5
Q

How does dementia incidence vary with age?

A

Increases with age from around age 60 with steep increases after 80 years and 90 years

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6
Q

What is the demarcation between early and late onset dementia?

A
  • Early onset <65 years old

- Late onset >65 years old

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7
Q

What are the types of late onset dementia

A
  • Alzheimers (55%)
  • Vascular (20%)
  • Lewy body (20%)
  • Others
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8
Q

What are the types of early onset dementia?

A

-Alzheimers (33%)
-Vascular (15%)
-Frontotemporal (15%)
-Other (33%)
What

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9
Q

What are treatable causes of dementia?

A
  • Vitamin deficiency: B12
  • Endocrine: thyroid disease
  • Infective: HIV, syphilis
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10
Q

What dementia mimics are there?

A
  • Hydrocephalus
  • Tumour
  • Depression:’pseudodementia’
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11
Q

How is dementia diagnosed?

A

History
-Type of deficit, progression, risk factors, FH

Examination
-Cognitive function, neurological, vascular

Investigations

  • Routine: bloods, CT/MRI
  • Other: CSF, EEG, functional imaging, genetics (biopsy)
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12
Q

What domains are examined when assessing cognitive function?

A
  • Memory
  • Attention
  • language
  • Visuospatial
  • Behaviour
  • Emotion
  • Executive function
  • Apraxia’s
  • Agnosias
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13
Q

What screening tests can be used to examine cognitive function?

A
  • Mini mental state examination (MMSE)
  • Montreal (MOCA)
  • Neuropsychological assessment
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14
Q

When is a definitive dementia diagnosis made?

A

Post-mortem

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15
Q

What clues are there in the diagnosis of dementia?

A

Type of cognitive deficit

Speed of progression

  • Rapid progression (CJD)
  • Stepwise progression (vascular)

Other neurological signs

  • Abnormal movements (Huntington’s)
  • Parkinsonism (Lewy body)
  • Myoclonus (CJD)
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16
Q

What is the commonest neurodegenerative condition?

A

Alzheimer’s disease

17
Q

What is the mean age onset for Alzheimer’s disease?

A

70 years

18
Q

How does temporo-parietal dementia present?

A
  • Early memory disturbance
  • Language and visuospatial problems
  • Personality preserved until later
19
Q

How does frontotemporal dementia present?

A
  • Early change in personality / behaviour
  • Often change in eating habits
  • Early dysphasia
  • Memory / visuospatial relatively preserved
20
Q

How does vascular dementia present?

A
  • Mixed picture

- Stepwise decline

21
Q

How can dementia symptoms be managed/eased?

A

Non pharmacological:

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

Pharmacological:

  • Insomnia
  • Behaviour (care with antipsychotics)
  • Depression
22
Q

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

A

Cholinesterase inhibitors

  • Donepezil, rivastigmine, glantamine
  • Small symptomatic improvement in cognition (wash-out)
  • No delay in institutionalisation

NMDA antagonist
-Memantine

23
Q

What specific treatment is there for frontotemporal dementia?

A

None

24
Q

How effective is decreasing vascular risk factors in the treatment of vascular dementia?

A

No good evidence

25
Q

Define Parkinsonism

A

A clinical syndrome with 2 or more of:

  • Bradykinesia (slowness of movement)
  • Rigidity (stiffness)
  • Tremor (shakiness)
  • Postural instability (unsteadiness / falls)
26
Q

Where is the pathology in Parkinsonism?

A
  • Predominantly dopamine loss in the basal ganglia

- Associated with substantia nigricans

27
Q

What are 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

  • Multiple system atrophy
  • Progressive supranuclear palsy / corticobasal degen
28
Q

What is the epidemiology of Parkinsonism?

A
  • 2nd commonest neurodegenerative disease
  • Incidence 15-20 per 100,000 per year
  • Crude prevalence 150-300 per 100,000
29
Q

How does the incidence of Parkinsonism differ with age?

A
  • Increases with age

- Decreases 80-90

30
Q

When is definitive diagnosis of Parkinsonism made?

A

Post-mortem

31
Q

How is a diagnosis od Parkinsonism made clinically?

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

-Supported by asymmetric rest tremor, good response to dopamine replacement treatment

32
Q

How can Parkinsonism be imaged?

A

Functional imaging: Dopamine transporter SPECT

33
Q

What early medical treatment is there for Parkinsonism?

A

Dopamine agonists

  • Ropinirole
  • Pramipexole
  • Rotigotine

MAO-B inhibitor

  • Selegiline
  • Rasagiline
  • Safinamide

COMT inhibitor

  • Levodopa
  • Entacapone
34
Q

What drug induced complications may there be with Parkinsonism?

A
  • Motor fluctuations: levodopa wears off
  • Dyskinesias: involuntary movements (levodopa)
  • Psychiatric: hallucinations, impulse control
35
Q

What non-drug induced complications may there be with Parkinsonism?

A
  • Depression (20%)
  • Dementia (~50% after 10 yrs)
  • Autonomic: BP, bladder, bowel
  • Speech, swallow
  • Balance
36
Q

What late treatment is there for Parkinsonism?

A

Drugs:

  • Prolong levodopa half life: MAO-B inhibitors, COMT inhibitor, slow release levodopa
  • Add oral dopamine agonist
  • Continuous infusion (apomorphine, Duodopa)

Functional neurosurgery (deep brain stimulation)

Allied health professionals +/- care package

Systems: Neurology AB (51 decks)

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