Degenerative Diseases of the Central Nervous System

Question 1

What can be said generally about the aetiology of neurodegenerative diseases?

Answer 1

It is largely unknown

The diseases are usually late in onset and with gradual progression

Question 2

What is a feature of the imaging of neurodegenerative disease?

Answer 2

It is often normal

Question 3

Define dementia

Answer 3

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

Question 4

What is the incidence of dementia in the UK?

Answer 4

200 per 100,000

Question 5

What proportion of patients diagnoses with dementia are living in care homes?

Answer 5

66%

Question 6

What proportion of late onset dementia (65+ years) is made up by Alzheimer’s?

Answer 6

55%

Question 7

What are some treatable causes of dementia?

Answer 7

B12 deficiency
Thyroid disease
HIV, syphilis

Question 8

What are some things that can mimic the effects of dementia?

Answer 8

Hydrocephalus
Tumour
Depression

Question 9

What are the routine Ix for dementia?

Answer 9

Bloods, CT/MRI

Question 10

Give two cognitive screening tests

Answer 10

MMSE (mini-mental)

Montreal (MOCA)

Question 11

What does a stepwise progression of dementia suggest?

Answer 11

Vascular type dementia

Question 12

What does a rapid progression of dementia suggest?

Answer 12

CJD
Tumour
Infection

Question 13

What is the mean age of onset in Alzheimer’s disease?

Answer 13

70

Question 14

What are the characteristics of temporo-parietal dementia caused by Alzheimer’s?

Answer 14

Early memory disturbance
Language and visuospatial problems
Personality initially preserved

Question 15

What are the characteristics of frontotemporal dementia caused by Alzheimer’s?

Answer 15

Early change in behaviour/personality
Change in eating habits
Early dysphagia
Memory/visuospatial relatively preserved

Question 16

Outline the non-pharmacological symptomatic treatment for Alzheimer’s

Answer 16

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

Question 17

What should be treated with a pharmacological strategy in Alzheimer’s?

Answer 17

Insomnia
Behaviour
Depression

Question 18

Outline the specific treatment for Alzheimer’s disease

Answer 18

Cholinesterase inhibitors e.g. donepezil, rivastigmine, galantamine
No delay in institutionalism, small symptomatic improvement in cognition

Question 19

Define Parkinsonism

Answer 19

A clinical syndrome with two or more of the following symptoms:
Bradykinesia
Rigidity
Tremor
Postural instability

Question 20

What is the general pathology of Parkinson’s disease?

Answer 20

Basal ganglia affected, predominantly dopamine loss

Question 21

What is the most common cause of Parkinson’s disease

Answer 21

Idiopathic Alzheimer’s - dementia with Lewy bodies

Drug-induced e.g. dopamine antagonists

Question 22

What is the clinical diagnosis for Parkinson’s disease?

Answer 22

Bradykineseia + at lest one of tremor, rigidity postural instability
Slowly progressive over 5 to 10 years

Question 23

What can be the early medical treatment of PD?

Answer 23

Levadopa with carbidopa or similar

Question 24

What are some drug-induced later complications of PD?

Answer 24

Motor fluctuations as drugs eventually wear off
Dyskinesias - involuntary movements
Psychiatric - hallucinations, impulse control

Question 25

What are some non drug-induced later complications of PD?

Answer 25

Depression
Dementia
Autonomic dysfunction e.g. BP, bladder, bowel
Sensory and sleep

Question 26

What drugs can be used to prolong the half-life of dementia?

Answer 26

MAO-B inhibitors
COMT inhibitors
Slow release levadopa