Degenerative Diseases of the CNS Flashcards

1
Q

What are some common features of neurodegenerative diseases?

A
  • Aetiology often unknown
  • Usually late onset
  • Gradual progression
  • Neuronal loss
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2
Q

What is dementia?

A

A neurodegenerative disease resulting in progressive impairment of MULTIPLE domains of cognitive functioning - resulting in LOSS OF ACQUIRED SKILLS and interference in occupational and social role

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3
Q

What are the main causes of dementia?

A
  • Alzheimers
  • Vascular
  • Lewy body (old patients)
  • Frontotemporal (younger patients)
  • Creutzfeldt-Jakob Disease
  • If dementia is young onset (<65yrs), other causes such as alcohol and genetics account for a higher percentage of cases
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4
Q

What are some of the treatable causes of dementia?

A
  • Vitamin B12 deficiency
  • Endocrine: thyroid disease
  • Infection: HIV, syphilis
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5
Q

Important patient history questions for dementia diagnosis? Clinical examinations?

A

History:

  • Type of deficit
  • Progression
  • FH / risk factors

Examination:

  • Cognitive function
  • Neurological / vascular
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6
Q

Investigations for suspected dementia?

A
  • Routine: bloods / CT / MRI (rule out hydrocephalus / haematoma)
  • others: CSF, EEG, functional imaging, genetics
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7
Q

What are some of the domains of cognitive functions that may be affected by dementia?

A
  • Memory
  • Attention
  • Language
  • Visuospatial
  • Behaviour
  • Emotion
  • Executive function
  • Apraxias (ability to plan movement)
  • Agnosias (processing sensory info)
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8
Q

What screening tests are used to help diagnose dementia?

A
  • Mini-mental (MMSE)

- Montreal (MOCA)

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9
Q

What are some signs that help with determining which of the different causes of dementia a patient is suffering from?

A

Speed of progression

  • Rapid (CJD)
  • Stepwise (vascular)

Other neurological signs:

  • Abnormal movements (Huntingtons)
  • Parkinsonism (Lewy Body)
  • Myoclonus (CJD)
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10
Q

What is the most common cause of dementia?

A
  • Alzheimer’s disease
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11
Q

Risk factors for developing Alzheimer’s?

A
  • Genetic
  • Smoking
  • Diet
  • Alcohol
  • Exercise
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12
Q

Signs of frontotemporal dementia?

A
  • Early change in personality / behaviour
  • Change in eating habits
  • Early dysphasia
  • Memory / visuospatial ability relatively preserved
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13
Q

Signs of vascular dementia?

A
  • Mixed picture symptoms-wise (motor + cognitive)

- Stepwise progression of disease

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14
Q

Treatment of dementia? (broad)

A

Non-pharma:
- Occupational therapy / social work

Pharmacological:

  • Insomnia meds
  • Behavior (antipsychotics)
  • Antidepressants
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15
Q

Alzheimer’s (+/- Lewy Body) dementia specific treatment?

A
  • Cholinesterase inhibitors (Donepezil / Rivastigmine)

- NMDA antagonist

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16
Q

Specific treatment of frontotemporal / Vascular dementia?

A

Frontotemporal:
- None

Vascular:
- None supported by robust evidence

17
Q

What is Parkinsonism?

A

A clinical syndrome with two or more of:

  • Bradykinesia (slow movement)
  • Rigidity
  • Tremors
  • Postural instability
18
Q

Pathophysiology of Parkinsons?

A
  • Degeneration of dopaminergic neurones of the basal ganglia (esp. substantia nigra)
19
Q

Causes of Parkinsons?

A
  • Idiopathic (dementia with Lewy Bodies)
  • Drug induced (eg. dopamine antagonists)
  • Vascular Parkinsons
20
Q

Clinical signs indicating Parkinsons?

A

Bradykinesia + tremor / rigidity / postural instability

  • Supported by asymmetric rest tremor and a good response to dopamine replacement therapy
21
Q

What are some signs that would NOT SUPPORT a Parkinson’s diagnosis?

A
  • Rapid progression
  • Symmetrical (tremors etc.)
  • Poor response to dopamine replacement therapy
  • Early falls
  • Early dementia
22
Q

What investigation can be good for diagnosing / staging Parkinsons? What does it measure?

A
  • Dopamine Transporter SPECT (Single-photon emission computed tomography)
  • Measures the efficiency of dopamine transporters in the presynaptic cleft that bring in dopamine for re-use or degradation (best seen at caudate and putamen)
23
Q

Treatment of early Parkinson’s disease?

A

Increase amount of available dopamine (inhibit breakdown enzymes):

  • COMT inhibitors
  • MAO-B inhibitors

Dopamine agonists
- Ropinirole / Pramipexole

24
Q

What are some late complications that can occur with Parkinson’s disease?

A

Drug induced:

  • Motor Fluctuations (levodopa wears off)
  • Dyskinesias (involuntary movements - levodopa)
  • Psychiatric (hallucinations / impulse control)

Non-drug induced:

  • Depression
  • Dementia
  • Speech / swallow / balance issues
25
Q

Late treatment of Parkinson’s disease?

A
  • MAO-B / COMT inhibitors
  • Slow release levodopa
  • Dopamine agonists

Surgery:

  • Deep brain stimulation (implanted electrodes)
  • Radio-frequency ablation (pallidotomy / thalamotomy)
26
Q

How is the frequency of neurodegenerative diseases changing?

A
  • Increasing

becoming more common