Degenerative diseases

Question 1

What are the common features of degenerative diseases?

Answer 1

• Unknown aetiology
• Late onset
• Gradual progression
• Neuronal loss
• Normal structural imaging

Question 2

What is dementia?

Answer 2

Progressive impairment of multiple domains of cognitive function in alert patient

Question 3

What are the subtypes of dementia?

Answer 3

Alzheimer’s disease
Vascular dementia
Lewy body dementia
Frontotemporal dementia

Question 4

What is Alzheimer’s disease?

Answer 4

Neuropsychiatric disorder causing progressive cognitive impairment
Leading cause of dementia

Question 5

What is Vascular dementia?

Answer 5

Cumulative effect of many small strokes
Evidence of arteriopathy

Question 6

What is Lewy body dementia?

Answer 6

Caused by abnormal clumps of protein (called Lewy bodies) gathering inside brain cells
Detailed visual hallucinations
Later parkinsonism

Question 7

What is frontotemporal dementia?

Answer 7

Frontal and temporal atrophy with loss of >70% of spindle neurons

Question 8

What examinations may be done for dementia?

Answer 8

Cognitive function
Neurological
Vascular

Question 9

What investigations may be done for dementia?

Answer 9

Bloods: FBC, U&E, LFTs, Calcium, Glucose, TFTs, Vitamin B12, Folate levels
CT/MRI
CSF
EEG
Functional Imaging
Genetics

Question 10

What screening tests are used for cognitive function?

Answer 10

• Mini mental state examination (MMSE)
• Montreal Cognitive Assessment (MOCA)
• Addenbrooke’s Cognitive examination (ACE-II)

Question 11

What is the non- pharmacological management of dementia?

Answer 11

• Information and support
• Dementia services
• Occupational therapy
• Social work/support/respite/placement
• Voluntary organisations

Question 12

What is the specific treatment for Alzheimer’s dementia?

Answer 12

Cholinesterase inhibitors
NMDA antagonist

Question 13

What is the specific treatment for vascular dementia?

Answer 13

Lower vascular risk factors?

Question 14

What is Parkinsonism?

Answer 14

Clinical syndrome with >2 of:

• Bradykinesia (slowness of movement)
• Rigidity (stiffness)
• Tremor (shakiness)
• Postural instability (falls)

Question 15

What is Parkinson’s disease caused by?

Answer 15

Progressive degeneration of dopaminergic neurones in the substantia nigra leading to dopamine loss

Question 16

What are the causes of Parkinson’s?

Answer 16

Idiopathic- Dementia with Lewy bodies
Drug induced- dopamine antagonists
Vascular Parkinsonism- affects lower half of body

Question 17

What are the features of parkinsonism?

Answer 17

Bradykinesia, tremor, rigidity, postural instability
No other cause
Slowly progressive (>5-10 years)
-Good response to dopamine replacement therapy

Question 18

What is the early treatment of parkinsons?

Answer 18

Dopaminergic neuron- increase dopaminergic function

Give levodopa (precursor for dopamine)

Question 19

What is the late treatment of parkinson’s?

Answer 19

Prolong levodopa half life:
- MAO-B inhibitors
- COMT inhibitors
- Slow release levodopa
Add oral dopamine agonist- continuous infusion
Functional neurosurgery

Question 20

What drugs should never be used in Parkinsons?

Answer 20

Atypical antipsychotics
Have strong anti-dopaminergic action so will worsen disease

Question 21

What are Parkinsons plus syndromes?

Answer 21

A group of degenerative neurological disorders, which differ from the classical idiopathic Parkinson’s disease:
- Different associated clinical features,
- Poor response to levodopa
- Distinctive pathological characteristics
- Poor prognosis.

Question 22

What are examples of Parkinson’s plus syndromes?

Answer 22

Progressive supranuclear palsy
Multiple system dystrophy
Cortico-basal degeneration
Lewy body dementia