Degenerative diseases of the NS

Question 1

what are common characteristics of the NS degenerative diseases

Answer 1

• Late onset
• Gradual progression
• Neuronal loss
• Imaging useful
• aetiology unknown

Question 2

What is dementia

Answer 2

• patient alert
• Progressive impairment of multiple domains of cognitive function
• Loss of skills in social and occupational roles

Question 3

What are the late onset causes of dementia?

Answer 3

In over 65s:

• Alzheimers
• Lewy body
• Vascular

Question 4

What is Lewy body demential associated with

Answer 4

Clumps of proteins which aggregate in neurones

Question 5

What are the young onset causes of dementia?

Answer 5

Under 65s
Alzheimers 
-Vascular 
-Fronto-temporal 
-Others 
Alcohol 
Genetic e.g. Huntingtons 
Infection e.g. HIV, CJD
Inflammation e.g. MS

Question 6

What are the treatable causes of dementia?

Answer 6

• B12 deficiency
• Infections e.g. HIV
• Endocrine e.g. associated with thyroid disease

Question 7

Differential diagnosis for dementia

Answer 7

• Depression
• Hydrocephalus
• Tumours- particularly frontal tumours

Question 8

Why is dementia such a big issue?

Answer 8

• It impacts 2/3rds of families
• It is very expensive for both the NHS and families
• Common and increasing
• NO cure

Question 9

History of some one with dementia

Answer 9

FROM AN INDEPENDENT WITNESS 
Type of deficit - behavioural changes 
Progression 
Family history 
Risk factors

Question 10

Examination of a patient with dementia

Answer 10

Cognitive :

• Test memory, attention, behaviour, language, emotion, agnosias, apraxias
• Screening tests e.g. Mini mental test, Montreal

Neurological
Vascular

Question 11

Investigations of a patient with dementia

Answer 11

• CT/MRI
• Bloods
• CSF
• Genetics
• EEG
• Functional imaging
• Brain biopsy

Question 12

clues to help differentiate between different types of dementia

Answer 12

-Type of deficit
-Progression of disease
-other signs :
Myoclonus
Parkinsonism
Abnormal movement

Question 13

Progression of alzheimers

Answer 13

TEMPO-PARIETAL DEMENTIA :
Affects memory
Visual spatial and language disturbances
Personality affected later on

Question 14

Progression of fronto-temporal dementia

Answer 14

Early changes to personality
Dysphagia
Changes in eating habits
Memory and visuospatial problems present later on

Question 15

Characters of. vascular dementia

Answer 15

• Mixed problems

- Stepwise decline

Question 16

How to treat dementia

• Non-pharmalogical
• Pharmalogical

Answer 16

Non -pharmalogical

• OT/social carers
• Voluntary work
• Information services

Pharmalogical

• Problems sleeping
• Behaviour e.g. eating
• Depression

Question 17

How to treat dementia

-specific

Answer 17

Alzheimers/lewy body dementia
-Cholinesterase inhibitors e.g. DONEPEZIL
-NMDA (N-methyl, D-aspartatate) antagonist/ MEMANTINE:
In Alzheimers too much glutamate is produced
Glutamate is an NT which is taken into cells via NDMA

Question 18

How to diagnose parkinsonism

Answer 18

-Bradykinesia + 1 of rigidity, tremor, postural instability
-Tremor is:
At rest
Asymmetric
Responds to dopamine treatment
-No other causes
-Slow progression: 5-10 years

Question 19

What does imaging in parkinsonism reveal

Answer 19

Reduced dopamine transporter at the basal ganglia

Question 20

Early treatment of parkinsonism

Answer 20

• Dopamine agonists
• Levadopa
• COMT inhibitor: breaks down levodopa into product which is not dopamine
• MAO-B inhibitor: prevents dopamine in pre-synaptic terminal from being broken down

Question 21

Examples of dopamine agonists

Answer 21

Ropinirole
Rotigotine
Pramipexole

Question 22

Example of COMT inhibitor

Answer 22

entecapone

Question 23

Later onset symptoms of Parkinson’s

Answer 23

Drug induced:

• Motor fluctuations as levodopa wears off
• Dyskinesia
• Psychiatric problems e.g. hallcinations

Non-drug induced

• Depression
• Dementia
• Autonomic problems e.g. BP, bladder
• Speech and swallowing problems
• Balance

Question 24

Later treatment for Parkinson’s

Answer 24

-Prolong half-life of levodopa:
Slow release levodopa
COMT inhibitors
MAO-B inhibitor
Add oral levodopa

• Functional neurosurgery for brain stimulation
• Add in help from other health care professionals

Question 25

causes of Parkinson’s

Answer 25

• Idiopathic
• Drug induced: dopamine antagonists
• Vascular Parkinsonism - affects lower half of limbs
• Parkinson’s plus syndrome: multi-system atrophy, progressive supra nuclear palsy, corticospinal degeneration