Degenerative Diseases of the CNS Flashcards

1
Q

Name two of the main diseases of the CNS

A

Dementia and parkinsonism

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2
Q

Define dementia

A

It is a syndrome consisting on:
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

Describe the impact of dementia

A
  • Areas of cognition deteriote, it is not just a memory deficit
  • There is history of loss of cognitive function – not due to ability but they can’t process the action
  • Can’t maintain same social relationships due to impaired cognitive function
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4
Q

Name the causes of dementia with a late onset (65yrs)

A
  • Alzheimer’s
  • Vascular*
  • Lewy body**
  • Other
  • Repetitive little strokes, might not even be aware
  • *Form of parkinsons – abnormal aggregates of protein that develop inside nerve cells
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5
Q

Name the causes of dementia with an early onset (<65yrs)

A
• Alzheimer’s 
• Vascular 
• Frontaltemporal 
• Other 
   o Toxic (alcohol)
   o Genetic (Huntington’s)
   o Infection (HIV, CJD)
   o Inflammatory (MS)
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6
Q

What are three treatable causes of dementia?

A
  • Vit deficiency - B12
  • Endocrine - thyroid disease
  • Infective - HIV, syphilis
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7
Q

What are three conditions which mimic dementia?

A
  • Hydrocephalus
  • Tumour
  • Depression: ‘pseudodementia’
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8
Q

To diagnose dementia, what do you need to find out in the history?

A

From an independent witness:
• Types of deficit – areas where theyre struggling; memory, getting lost, wandering, failing with finances, change in behaviour (drive, motivation) etc
• Progression
Family history

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9
Q

To diagnose dementia, what do you need to find out in the examination?

A

Cognitive function neurological, vascular

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10
Q

To diagnose dementia, what do you need to find out in the lab results in dementia?

A
  • Routine – bloods (not B12 deficient, hypothyroid, screen for HIV), CT/MRI
  • Others (for under 65yrs) – CSF, EEG, functional imaging, genetics (biopsy)
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11
Q

What is involved in examination of cognitive function in dementia?

A
  • Various domains
  • Screening tests
  • Neuropsychological assessment
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12
Q

What do you look for in the various domains involved in examination of cognitive function in dementia?

A
  • Memory, attention, language, visuospatial,
  • Behaviour, emotion, executive function
  • Apraxia (can’t do a motor take as you can’t plan it), agnosias (inability to put sensory meaning to something)
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13
Q

Name two screening test for cognitive function of dementia

A

Mini-mental (MMSE) and Montreal (MOCA)

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14
Q

Name three clues to diagnosis of dementia

A
  • Type of cognitive deficit
  • Speed of progression
  • Other neurological signs
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15
Q

What are two different types of speed progression in dementia

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

*Creutzfeldt-jakob disease (CJD) is a fatal form of dementia caused by a protein found in the brain called prion

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16
Q

What is alzheimer’s disease?

A

Commonest neurodegenerative condition (mean age onset 70yr) which causes temporo-parietal dementia

17
Q

What are the features of dementia caused by alzheimer’s disease (temporo-parietal dementia)?

A

Temporo-parietal dementia:
• Early memory disturbance
• Language and visuospatial problems
• Personality preserved until later (as it doesn’t affect frontal lobe)
• “Does he/she have the same personality, just more forgetful?”

18
Q

What are the features of frontotemporal dementia?

A
  • Early change in personality / behaviour
  • Often change in eating habits (frontal and temporal lobes drive appetite)
  • Early dysphasia
  • Memory / visuospatial relatively preserved
19
Q

What is speed of progression in vascular dementia?

A

Stepwise decline (i.e. worsen with each stroke)

20
Q

What is the non-pharmacological symptomatic treatment of dementia?

A
  • Information & support, dementia services
  • OT
  • Social work / support / respite / placement
21
Q

What is the pharmacological symptomatic treatment of dementia?

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

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

A

• Cholinesterase inhibitors (cholinergic deficit) – prevents break down of ACh in the brain so more is available for communication between brain cells
o Donepezil, rivastigmine, galanamine
• NMDA antagonist (memantine)

23
Q

Is there a specific treatment for frontotemporal dementia?

A

No

24
Q

What are the treatment measures for vascular dementia?

A

No good evidence for decrease in vascular risk factors

25
Q

What is parkinsonism and its features?

A
A clinical syndrome with  >= 2 of:
• Bradykinesia (slowness of movement)
• Rigidity (stiffness)
• Tremor (shakiness)
• Postural instability (unsteadiness / falls)
26
Q

What is the pathology in basal ganglia causing parkinsons?

A

Loss of nerves in the substantia nigra which leads to a reduction in dopamine in the brain. Dopamine plays a role in regulating movement in the body, so a reduction causes the signs that you see in Parkinson’s.

27
Q

What are the causes of parkinson’s?

A

• Idiopathic Parkinson’s Disease dementia with Lewy bodies
• Drug induced (i.e. dopamine antagonists)
• Vascular parkinsonism (lower-half)
• Parkinson’s plus syndrome
o Multiple system atrophy – postural hypotension, impotence, incontinence
o Progressive supranuclear palsy / corticobasal degeneration

28
Q

How do you get a definitive diagnosis of parkinsons?

A

Post mortem

29
Q

What are the clinical features of parkinson’s?

A
  • Bradykinesia + >= tremor, rigidity, postural instability
  • No other cause / atypical features
  • Slowly progressive (> 5-10yrs)
30
Q

What investigation in used for parkinsons?

A

Functional Imaging: Dopamine Transporter SPECT (Not diagnostic)

31
Q

What is the early treatment of PD?

A
  • Levadopa is a precursor of dopamine, give with carbidopa or benserazide to block the enzymes
  • CMOT inactivates levodopa, so can use COMT inhibitor (i.e. entacapone)
  • These drugs are purely symptomatic, no prevention of progression of disease
32
Q

What are drug induced later complications that occur in PD?

A
  • Motor fluctuations – levodopa wears off
  • Dyskinesias – involuntary movements (levodopa)
  • Psychiatric – hallucinations, impulse control
33
Q

What are non-drug induced later complications that occur in PD?

A
  • Depression
  • Dementia
  • Autonomic: BP, bladder, bowel
  • Speech, swallow
  • Balance
34
Q

What is the later treatment of PD?

A
• Prolong levodopa half life:
  o MAO-B inhibitors 
  o COMT inhibitor 
  o Slow release levodopa 
• Add oral dopamine agonist 
• Continuous infusion (apomorphine, duodena)

Function neurosurgery (deep brain stimulation)

Allied health professional +/- care package