Degenerative disease of the CNS

Question 1

What causes neurodegenerative disease?

Answer 1

The aetiology of Neurodegenerative diseases such as dementia and parkinsonism is largely unknown

Rarely linked to mendelian genetic cases

Question 2

Describe the general onset of neurodegenerative diseases as a whole

Answer 2

Onset is usually late - ie typically in older people

Onset and progression of Neurodegenerative diseases is gradual

Question 3

What is dementia?

Answer 3

A syndrome consisting of progressive impairment of multiple domains of cognitive function

Leads to loss of acquired skills and interference in occupational and social aspects

Question 4

What are the causes of dementia?

Note - causes are also used as ‘types’ of dementia

Answer 4

Alzheimer’s - most common

Vascular

Lewy body

Frontotemporal

Question 5

Complete the diagram of causes of dementia in old and young people

Answer 5

Alzheimer’s is by far the most common cause

Question 6

What ‘other’ causes of dementia are there?

Answer 6

Alcohol abuse

Huntington’s disease

Infections:

• HIV
• CJD

Multiple sclerosis (inflammation aspect)

These are more common causes of dementia in under 65’s (young people)

Question 7

What are the risk factors for dementia

Answer 7

Vitamin B12 deficiency

HIV, syphilis

Thyroid disease (endocrine problems)

Huntington’s, MS

Alcohol abuse

Old age

Question 8

What conditions can mimic dementia (differentials)?

Answer 8

Hydrocephalus

Tumours

Depression - ‘pseudodementia’

Question 9

What is the approach to diagnosing dementia?

Answer 9

History (with independent witness):

• type of deficit, progression, risk factors, FH

Examination:

• cognitive function, neurological, vascular

Investigations:

• Bloods + CT / MRI

others - CSF, EEG, functional imaging, genetics (biopsy)

Question 10

Describe how you would assess a patient (with potential dementia)’s cognitive function?

Answer 10

Assess the various domains of cognitive function:

• memory, attention, language, behaviour, visuospatial etc

Screening tests:

• Mini-mental test (MMSE)
• Montreal test (MOCA)

Neuropsychological assessment

Question 11

What type of onset is specifically associated with Vascular dementia?

Answer 11

Stepwise progression - is indicative of Vascular dementia

Question 12

A rapid progression of dementia symptoms is associated with what cause of dementia?

Answer 12

CJD (Creutzfeldt–Jakob disease)

Fuck ever spelling that

Question 13

For each cause of dementia below, state any specific neurological signs that may indicate that cause…

A) Huntington’s

B) Lewy body

C) CJD

Answer 13

A) Huntington’s:

• Abnormal movements

B) Lewy body:

• Parkinsonism

C) CJD:

• Rapid onset/progression
• Myoclonus

Question 14

What are the risk factors for Alzheimer’s disease?

Answer 14

Being old

Some Genetic risks

Lifestyle:

• Smoking
• Exercise
• Diet
• Alcohol

Question 15

What neurological signs are indicative of Temperoparietal dementia (which btw is a type of Alzheimer’s)

Answer 15

Early memory disturbance

Visuospatial problems:

• falling over
• bruises from bumping into things etc

Personality preserved until later stages so this will likely be normal at time of presentation

Question 16

What neurological signs are specific to frontotemporal dementia?

Answer 16

Early change in personality/behaviour

Change in eating habits

Early dysphasia - communication problems

However - memory & visuospatial function remains okay at early stages

Question 17

Describe the symptomatic treatment for dementia

Answer 17

Non-pharma:

• Information & support, dementia services
• Occupational therapy
• Social work / respite / placement (care homes?)
• Voluntary organisations

Pharma:

• Insomnia medication
• Antipsychotics - for extreme behavioural problems
• Anti-depressants

Question 18

What treatments are available for Alzheimer’s?

Answer 18

Cholinesterase inhibitors (cholinergic deficit)

• Donepezil, rivastigmine, galantamine
• Pretty shit though

NMDA antagonist (memantine)

Question 19

What is the treatment route for Lewy body dementia?

Answer 19

Same as alzheimer’s

Cholinesterase inhibitors - Donepezil, rivastigmine, galantamine

NMDA antagonists - memantine

Question 20

How is frontotemporal dementia specifically treated?

Answer 20

No available treatments exist for FT dementia

Treatment is purely symptomatic

Question 21

What specific treatments exist for vascular dementia?

Answer 21

Nothing really exists

Decreasing vascular risk factors has no robust evidence to support it

Question 22

What is Parkinsonism?

Answer 22

A clinical syndrome with 2 or more of:

• Bradykinesia - slow movement. Pretty much always seen.
• Rigidity - stiffness
• Tremor
• Postural instability - falls, unsteadiness

Question 23

What causes parkinsonism?

Answer 23

Pathology in the basal ganglia - predominantly dopamine loss

Question 24

What are the types of parkinson’s disease?

Answer 24

Idiopathic Parkinson’s disease:

• Dementia with Lewy bodies

Drug-induced:

• Dopamine antagonists

Vascular parkinsonism

Parkinson’s plus syndromes:

• Multiple system atrophy
• Progressive supranuclear palsy / corticobasal degeneration
  • Group of conditions which feature classic parkinsonism but with extra distinct features

Question 25

What is the prognosis for Parkinson’s disease?

Answer 25

60-90% dead or disabled at 10 yrs

Because of a lack of effective specific treatments

Question 26

Describe the typical presentation for a patient with Parkinson’s disease

Answer 26

Bradykinesia + 1/more of:

• Tremor - affects one side (hand), at rest, ‘pill-rolling tremor’
• Rigidity
• Postural instability

Slow progression (>5-10 years)

± Dementia symptoms/signs, neurological features

Question 27

Why is a trial of dopamine replacement treatment a good investigation for Parkinsonism?

Answer 27

Good response to treatment would support a diagnosis of Parkinsonism