Degenerative diseases of the CNS

Question 1

Common features of neurodegenerative diseases (3)

Answer 1

usually late onset

aetiology largely unknown

gradual progression

Question 2

Define dementia

Answer 2

Progressive impairment of multiple domains of cognitive function in alert patient leading to loss of acquired skills and interference in occupational and social role

Question 3

Causes of late-onset dementia (over 65)

Answer 3

alzheimers 55%
vascular 20%
Lewy body 20%

Question 4

Causes of early-onset dementia (under 65)

Answer 4

Alzheimer’s 33%
Vascular 15%
Frontotemporal 15%

Other - toxic (alcohol), genetic (Huntington’s), Infection (HIV, CJD), inflammatory (MS)

Question 5

What are some treatable causes of dementia?

Answer 5

vit B12 deficiency

Thyroid disease

HIV, Syphilis

Question 6

What are some mimics that present with dementia (3)

Answer 6

hydrocephalus
tumour
depression -
‘pseudodementia’

Question 7

How is dementia diagnosed?

Answer 7

History - type of deficit, progression, RFs and FH

Examination - cognitive function, neurological, vascular

Investigations - routine = bloods, CT/MRI - others = CSF, EEG, functional imaging, genetics

Question 8

What domains are examined when examining cognitive function (9)

Answer 8

Memory
Attention
Language
Visuospatial
Behaviour
Emotion
Executive function
Apraxias - individual has difficulty with the motor planning to perform everyday tasks or movements when asked
Agnosias - inability to process info

Question 9

Screening tests done to test cognitive functioning (2)

Answer 9

mini-mental MMSE

Montreal MOCA

Question 10

How can you work out what type of cognitive deficit a patient has

Answer 10

1. speed of progression
   - rapid = Creutzfeldt–Jakob disease (CJD)
   - Stepwise = vascular
2. other neurological signs
   - abnormal movements = Huntington’s
   - Parkinsonism = Lewy body
   - Myoclonus (CJD)

Question 11

Risk factors of Alzheimer’s disease

Answer 11

genetic

lifestyle - smoking, exercise, diet, alcohol

Question 12

How does Temporo-parietal dementia present? (3)

Answer 12

Early memory disturbance

Language and visuospatial problems

Personality preserved until later

Question 13

How does Frontotemporal dementia present? (3)

Answer 13

Early change in personality/behaviour

Often change in eating habits

early dysphasia

Memory/visuospatial relatively preserved

Question 14

Define dysphasia

Answer 14

Impaired ability to understand or use the spoken word.

Question 15

Non-pharmacological treatment for symptoms of Dementia (4)

Answer 15

Information & support, dementia services

Occupational therapy

Social work / support / respite / placement

Voluntary organisations

Question 16

Pharmacological treatment can treat which symptoms associated with Dementia

Answer 16

Insomnia
Behaviour
Depression

Question 17

Specific treatment of Alzheimer’s (+/- Lewy body dementia)

Answer 17

Cholinesterase inhibitors (donepezil, rivastigmine)
ACh is lost in dementia, the effect of a cholinesterase inhibitor is to increase the amount of ACh - although this doesn't stop the degeneration of nerves, it does delay progressive disability or entry into a care home

NMDA antagonist (memantine)

Question 18

What are the top 2 commonest neurodegenerative diseases?

Answer 18

1. Alzheimer’s

2. Parkinson’s

Question 19

Later drug-induced complications with Parkinson’s treatment

Answer 19

Motor fluctuations - levodopa wears off

Dyskinesias - involuntary movements (levodopa)

Psychiatric - hallucinations, impulse control

Question 20

What are common features amongst neurodegenerative diseases?

Answer 20

Gradual neuronal loss

Structural imaging is often normal

Question 21

What are the different types of dementia?

Answer 21

Tempero-parietal

Frontotemporal

Vascular

Question 22

Treatment for Frontotemporal dementia?

Answer 22

none