Lecture 36 Degenerative Diseases of the CNS

Question 1

Common features of neurodegenerative diseases

Answer 1

• Aetiology largely unknown (mendelian genetic cases rare, often younger onset)
• Usually late onset
• Gradual progression
• Neuronal Loss (specific neuropathology)
• Structural imagine often normal (but there may be atrophy that is not visible)

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

Name causes of late onset dementia

Answer 3

Alzheimer’s, Vascular, Lewy Body, Others- common causes

Question 4

Name causes of young onset of dementia

Answer 4

Alzheimer’s, Vascular, Frontotemporal, Alcohol, Huntington’s, HIV, CJD, MS)

Question 5

Name treatable causes of dementia

Answer 5

• Vitamin deficiency -B12
• Endocrine - thyroid disease
• Infective - HIV, syphilis

Question 6

Name mimics of dementia

Answer 6

o Hydrocephalus
o Tumour
o Depression: Pseudodementia (apathetic, slow thinking, confused)

Question 7

How do you diagnose dementia

Answer 7

History
Examination
Investigations

Question 8

What history would you take for diagnosing dementia

Answer 8

o Type of deficit
o Progression
o Risk Factors
o FH

Question 9

What type of examination would you carry out to diagnose dementia

Answer 9

o Cognitive function
o Neurological
o Vascular

Question 10

What investigations would you carry out to investigate dementia

Answer 10

o	Routine bloods: B12, Thyroid function
o	CT/MRI- rule out any causes that mimic dementia
o	CSF- MS, CJD
o	EEG
o	Functional imaging
o	Genetics (biopsy)

Question 11

Name the various domains involved in the examination of cognitive function

Answer 11

• Memory, attention, language, visuospatial,

* Behaviour, emotion, executive function

Question 12

What is apraxia

Answer 12

inability copy certain movements and are unable to, inability to dress- cognitive ability to create a motor plan

Question 13

What is agnosia

Answer 13

does not recognizes faces, can’t tell its face- unable to link sensory to cognitive processing

Question 14

What screening tests can be used to examine cognitive function

Answer 14

• Mini-mental (MMSE)- memory

* Montreal (MOCA)- executive function and memory

Question 15

What is the commonest neurodegenerative condition

Answer 15

Alzheimer’s

Question 16

What are the risk factors for Alzheimer’s

Answer 16

Genetic
Smoking
Exercise
Diet
Alcohol

Question 17

Name features of temporal-parietal dementia

Answer 17

– Early memory disturbance
– Language and visuospatial problems
– Personality preserved until later

Question 18

Name the features of Fronts-temporal dementia

Answer 18

• Early change in personality / behavior
• Often change in eating habits
• Early dysphasia
• Memory / visuospatial relatively preserved

Question 19

Name features of Vascular dementia

Answer 19

Mixed picture

Stepwise decline

Question 20

Non-pharmacological treatment of Alzheimer’s

Answer 20

– Information & support, dementia services
– Occupational therapy
– Social work / support / respite / placement
– Voluntary organisations

Question 21

Specific treatment for Alzheimer’s

Answer 21

– Cholinesterase inhibitors (cholinergic deficit)
– NMDA antagonist (memantine)
No treatment for fronto-temporal
Decrease vascular risk factors for vascular dementia

Question 22

What are the clinical features of Parkinson’s

Answer 22

– Bradykinesia (slowness of movement)
– Rigidity (stiffness)
– Tremor (shakiness)
– Postural instability (unsteadiness / falls)

Question 23

What is the pathology of the basal ganglia in Parkinson’s

Answer 23

Predominantly dopamine loss

Question 24

What are the causes of Parkinson’s

Answer 24

Dopamine antagonists
Vascular parkinsons
Dementia with Lewy bodies 
Multiple system atrophy
Progressive supra nuclear palsy

Question 25

How is Parkinson’s Diagnosed

Answer 25

• Bradykinesia + ≥1 tremor, rigidity, postural instability
• No other cause / atypical features
• Slowly progressive (> 5-10 yrs)

Question 26

Name the early treatment for Parkinson’s diseases

Answer 26

• Levodopa
• Metabolised by liver so given with COMT enzyme inhibitor
• MAO-B inhibitor- dopamine recycling
• Dopamine agonists

Question 27

Name the Late treatment for Parkinson’s Disease

Answer 27

• Prolong levodopa half-life:
• MAO-B inhibitors
• COMT inhibitor
• slow release levodopa
• Add oral dopamine agonist
• Continuous infusion (apomorphine, Duodopa)
• Functional neurosurgery (deep brain stimulation)
• Allied health professionals +/- care package

Question 28

Wha are the drug induced complications of Parkinson’s treatment

Answer 28

o Motor fluctuations - levodopa wears off
o Dyskinesias - involuntary movements (levodopa)
o Psychiatric - hallucinations, impulse control- overproduction of dopamine

Question 29

What are the non-drug induced

Answer 29

o	Depression (20%) 
o	Dementia (~50% after 10 yrs) 
o	Autonomic:  BP, bladder, bowel
o	Speech, swallow
o	Balance