Neurodegenerative diseases

Question 1

Give five common features of neurodegenerative diseases

Answer 1

Aetiology is largely unknown
Usually late onset (mendelian genetic cases tend to have an earlier onset)
Gradual progression
Neuronal loss (accelerated rate compared to normal ageing)
Structural imaging is 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

Question 3

List the criteria for the syndrome of dementia

Answer 3

Progressive
Multiple domains
Loss of acquired skills
Interference in occupation and/or social role

Question 4

Describe three types of late onset dementia

Answer 4

Vascular (multiple mini infarcts)
Alzheimer’s (temporo-parietal dementia)
Leury Body (type of Parkinsonism)

Question 5

Describe three main types of early onset dementia

Answer 5

Vascular
Early onset alzheimer’s
Frontotemporal (change in personality, behaviour, eating habits etc)

Question 6

Give four rarer causes of early onset dementia

Answer 6

Toxic (e.g. alcohol)
Genetic (e.g. Huntingdon’s)
Inflammatory (e.g. MS)
Infection (HIV, CJD)

Question 7

Which drugs can be used to specifically treat Alzheimer’s and Lewy body dementia?

Answer 7

Cholinesterase inhibitors
 - donepezil
 - rivastigmine
 - galantamine
NMDA agonist
 - memantine

Question 8

What clinical signs must be present in order to make a diagnosis of Parkinsonism

Answer 8

2 of:

• Bradykinesia
• Rigidity
• Tremor
• Postural instability

Question 9

Describe (in simple terms) the pathology of Parkinson’s disease

Answer 9

Pathology is in the basal ganglia; predominantly due to dopamine loss

Question 10

Describe how Parkinson’s is generally diagnosed

Answer 10

Only definitive diagnosis is an autopsy
At least 2 signs are needed
Supported by asymmetric rest tremor and good response to dopamine replacement treatment
Less likely if there is a rapid progression, early dementia, or other abnormal neurological signs

Question 11

Describe the causes of Parkinsonisms

Answer 11

Idiopathic (dementia with Lewy bodies)
Drug-induced (e.g. dopamine antagonists)
Vascular (particularly affects gait)
Parkinson's plus syndromes
 - Multiple system atrophy
 - Progressive supranuclear palsy / corticobasal degeneration (causes falls due to being unable to look down quickly)

Question 12

Give three dopamine antagonists. What are they used to treat?

Answer 12

Ropinirole
Pramipexole
Rotigodine
Used to treat early stages of Parkinsonisms

Question 13

Give three MAO-B inhibitors. What are they used to treat?

Answer 13

Selegiline
Rasogiline
Safinamide
Used to treat early stages of Parkinsonisms

Question 14

Give three drug types that can be used to treat late-stage Parkinsonism

Answer 14

MAO-B inhibitors
COMT inhibitor
Slow-release L-dopa

Question 15

Give three drug types that can be used to treat early stage Parkinsonism

Answer 15

L-dopa + enzyme inhibitor
Dopamine agonists
MAO-B inhibitor

Question 16

Give three enzyme inhibitors that are given with L-dopa to treat Parkinsonism

Answer 16

Carbidopa
Benserazide
COMT inhibitor

Question 17

Give an example of a hereditary neurodegenerative disease

Answer 17

Huntingdon’s