Degenerative Diseases of the CNS Flashcards

1
Q

What are common features amongst neurodegenerative diseases?

A

Gradual neuronal loss

Structural imaging is often normal

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2
Q

What does dementia consist of?

A

Disease progression results in loss of cognitive function leading to a loss of acquired skills and interference in occupational and social role.

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3
Q

What is the diagnosis of dementia?

A

Based on history and examination - has to be progressive (gets worse over time)

MULTIPLE areas of cognitive loss

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4
Q

What is the commonest reason to go into a nursing home

A

Dementia

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5
Q

What are the causes of dementia (late onset)?

A

Alzheimer’s (55%)

Vascular (20%)

Lewy body (20%)

Others (5%)

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6
Q

What are the causes of dementia (early onset)?

A

Alzheimer’s (33%)

Vascular (15%)

Frontotemporal (15%)

Other (33%)

  • Toxic (alcohol)
  • Genetic (Huntington’s)
  • Infection (HIV, CJD)
  • Inflammatory (MS)
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7
Q

What are the treatable causes and mimics of dementia?

A

Vitamin deficiency - B12

Endocrine - thyroid disease

Infective - HIV, syphilis

Mimics: Hydrocephalus

Tumour

Depression: “pseudodementia”

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8
Q

What is the examination and investigation for dementia?

A

Examination: Cognitive function, neurological, vascular

Investigations: Routine - bloods, CT / MRI

Others: CSF, EEG, functional imaging, genetics (biopsy)

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9
Q

What are the various domains of cognitive function?

A

Memory

Attention

Language

Visuospatial

Behaviour

Emotion

Executive function

Apraxias (can’t perform actions)

Agnosias (inability to interpret sensations and hence recognise things)

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10
Q

What are the screening tests for dementia?

A

Mini-mental (MMSE)

Montreal (MOCA)

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11
Q

What speed of progression does CJD and Vascular dementia have?

A

CJD - rapid progression - patients usually die within a year

Vascular - stepwise progression

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12
Q

What would abnormal movements (chorea) and myoclonic movements be suggestive of?

A

Other neurological signs

–abnormal movements (Huntington’s)

–parkinsonism (Lewy body)

–myoclonus (CJD)

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13
Q

What is the commonest neurodegenerative condition?

A

Alzheimers (temperoparietal dementia)

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14
Q

What are the different types of dementia?

A

Tempero-parietal

Frontotemporal

Vascular

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15
Q

What are the features of tempero-parietal dementia?

A

Early memory disturbance

Language and visuospatial problems

Personality is preserved until later

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16
Q

What are the features of frontotemporal dementia?

A

Early change in personality / behaviour

Often change in eating habits

Early dysphasia

Memory / visuospatial relatively preserved

17
Q

What are the features of vascular dementia?

A

Mixed picture

Stepwise decline

18
Q

What is the non pharmacological symptomatic treatment for dementia?

A

Information & support, dementia services

Occupational therapy

Social work / support / respite / placement

–Voluntary organisations

19
Q

What is the pharmacological intervention for symptomatic treatment of dementia?

A

Treatment for insomnia

Treatment for behaviour (care with antipsychotics)

Depression

20
Q

What is the specific treatment for alzheimers?

A

Cholinesterase inhibitors (donepezil, rivastigmine)

Acetylcholine is lost in dementia, the effect of a cholinesterase inhibitor is to increase the amount of acetylcholine - although this doesn’t stop the degeneration of nerves, doesn’t delay progressive disability or entry into a care home

NMDA antagonist (memantine)

21
Q

What is the treatment for frontotemporal dementia?

A

None

22
Q

What is the specific treatment for vascular dementia?

A

No good evidence for reduced vascular risk factors

23
Q

What is the clinical syndrome of parkinsonism?

A

2 or more of:

Bradykinesia (slowness of movement)

Rigidity (stiffness)

Tremor (shakiness)

Postural instability (unsteady, falls)

24
Q

Where is the pathology of parkinsonism?

A

Basal ganglia

(REMEMBER - dementia - frontal, temporal and parietal lobes)

As a result of dopamine loss

25
Q

What are the causes of parkinsons?

A

Idiopathic parkinsons

  • Dementia with lewy bodies

Drug induced (dopamine antagonists)

Vascular parkinsonism

Parkinson’s plus syndromes (multiple system atrophy, progressive supranuclear palsy / corticobasal degeneration)

26
Q

What is the diagnosis of parkinsons?

A

Must have bradykinesia (with on or more of tremor, rigidity and postural instability)

Slowly progressive

No other cause / atypical features

Asymetric rest tremor

Good response to dopamine repacement treatment

27
Q

When is it less likely ot be parkinsons?

A

Rapid progression

Symmetrical

Lack of rest tremor

Poor response to treatment

Early falls

Early dementia

Other abnormal neurological signs

28
Q

When does movement decrement in parkinsons?

A

Decrements after lots of repetitive movement on the affected side

29
Q

What is the functional imaging scan for parkinsons?

A

DAT scan - dopamine transporter scan

30
Q

What areas are seen to be reduced on a DAT scan?

A

Putamen

Caudate

31
Q

What is the early medical treatment of PD?

A

Levadopa

COMT inhibitor (entacapone)

MAO - B inhibitor

  • selegiline
  • rasagiline
  • safinamide

Dopamine Agonists

  • ropinirole
  • pramipexole
  • rotigotine
32
Q

What are drug induced complications associated with parkinsons?

A

Motor fluctuations - levadopa wears off

Dyskinesias - involuntary movements (levadopa)

Psychiatric - hallucinations, impulse control

33
Q

What are the later complications associated with parkinsons (non-drug induced)?

A

Depression - more likely in patients with parkinsons

Dementia

Autonomic: BP, Bladder, Bowel

Speech

Swallow

Balance

34
Q

What is late treatment for parkinsons?

A

DRUGS:

Aim is to increase levadopa half life:

MAO-B inhibitors

COMT inhibitors

Slow release levadopa

Or

Add oral dopamine agonist

Continuous infusion (apomorphine, duodopa - this is via peg)

FUNCTIONAL NEUROSURGERY: Deep brain stimulation

Allied health professionals