Degenerative CNS Disorders

Question 1

What are common features of neurodegenerative diseases?

Answer 1

Largely unknown aeriology
Usually late onset
Gradual progression
Neuronal loss
Structural imaging often normal

Question 2

What is dementia?

Answer 2

A syndrome consisting of progressive impairment of multiple domains of cognitive function in an alert patient leading to loss of acquired skills and interference in occupational and social role

Alzheimer’s
Frontotemporal dementia
Vascular dementia

Question 3

What are some treatable causes of cognitive impairment?

Answer 3

Vitamin B12 deficiency
Endocrine disorders e.g. thyroid disease
Infective e.g. HIV, syphilis
Normal pressure hydrocephulus

Question 4

What are the symptoms/signs of each dementia type?

Answer 4

Alzheimer’s/Temporoparietal

• most common
• mean onset 70
• early memory disturbance
• language and visuospatial problems
• personality preserved until later

Frontotemporal Dementia

• early change in personality/behaviour
• often change in eating habits
• early dysphasia
• memory/visuospatial relatively preserved

Vascular dementia

• mixed picture
• stepwise decline

Question 5

What investigations are performed in suspected dementia?

Answer 5

History - type of deficit, progression, risk factors
Examination - cognitive function
FBC
CT/MRI
CSF
EEG
Functional imaging
Genetics (biopsy)
Investigations only usually in <65

Question 6

What cognitive functions may be examined in suspected dementia?

Answer 6

Memory
Attention
Language
Visuospatial
Behaviour
Emotion
Executive Function
Apraxia
Agnosia

Question 7

What signs may suggest something other than typical dementia?

Answer 7

Speed of progression - rapid? Possible CJD

Abnormal movements? - Huntington’s, Parkinsonism (Lewy body), CJD (myoclonus)

Question 8

How is dementia treated non-pharmacologically?

Answer 8

Information and support, dementia services
Occupational therapy
Social work, support, respite
Voluntary organisations

Question 9

How is dementia treated pharmacologically?

Answer 9

Alzheimer’s +/- Lewy body dementia

• AChE inhibitors - donezepil, rivastigmine, galantamine
• small symptomatic improvement in cognition, no delay in institutionalisation
• NMDAR antagonist - memantine

Frontotemporal - none
Vascular dementia - no good evidence for decreasing vascular risk factors

Control insomnia, behaviour (antipsychotics), depression

Question 10

What is Parkinsonism?

Answer 10

A clinical syndrome with 2+ of:

• bradykinesia (slow movement)
• rigidity
• tremor
• postural instability

Slowly progressive (>5-10 years)
Parkinson's less likely if rapid progression, symmetrical, lack of rest tremor, poor treatment response, early falls, early dementia, other abnormal neurological signs

Question 11

What are the causes (pathology)/risk factors for Parkinson’s disease?

Answer 11

Pathology in basal ganglia
- predominantly dopamine loss

Idiopathic - dementia with Lewy bodies
Drug induced e.g. Dopamine antagonists
Vascular Parkinsonism
Parkinson’s plus syndromes (multiple system atrophy, progressive supranuclear palsy/corticobasal degeneration)

Question 12

How is Parkinson’s disease treated? (Early vs Late)

Answer 12

Early

• levodopa (Dopamine precursor)
• COMT inhibitor - entacapone (prevents breakdown)
• MAO-B inhibitors (prevents breakdown)
• Dopamine agonists - ropinirole, pramipexole, rotigotine

Late

• Prolong levodopa half-life (MAO-Bi, COMTi, slow release levodopa)
• Add oral dopamine agonist
• Continuous infusion (apomorphine, duodopa)
• Functional neurosurgery (deep brain stimulation)
• Allied Health Professionals +/- care package

Question 13

What are some non-drug induced complications of Parkinson’s?

Answer 13

Depression (20%)
Dementia (50% after 10 years)
Autonomic problems - BP, bladder, bowel
Speech, swallow problems
Balance

Question 14

What are some drug-induced complications of Parkinson’s?

Answer 14

Motor fluctuations - levodopa wears off
Dyskinesias - involuntary movements (levodopa)
Psychiatric - hallucinations, impulse control