Neurodegenerative Diseases

Question 1

Define Neurodegenerative disease

Answer 1

Progressive, irreversible condition leading to neuronal loss

Question 2

What is the end stage of neurodegenerative disease?

Answer 2

Dementia

Question 3

Define dementia

Answer 3

A global impairment of cognitive function and personality without impairement of consciousness.
This impairement goes beyond what might be expected from normal ageing

Includes memory impairment and at least 1 cognitive disturbances (aphasia, agnosia, apraxia) or a disturbance in executive function A global impairment of cognitive function and personality without impairement of consciousness.
This impairement goes beyond what might be expected from normal ageing

Includes memory impairment and at least 1 cognitive disturbances (aphasia, agnosia, apraxia) or a disturbance in executive function

Question 4

Define aphasia

Answer 4

A language disorder. May be expressive or receptive

Question 5

Define apraxia

Answer 5

Loss of ability to carry out learned purposeful tasks

Question 6

Define agnosia

Answer 6

Loss of ability to recognise objects and people

Question 7

What is the pathogenic mechanism causing neurodegenerative disease?

Answer 7

Accumulation of misfolded proteins which may be intra- or extracellular

Question 8

What pathological protein(s) (misfolded) is associated with alzheimers disease?

Answer 8

Tau
Beta-amyloidTau
Beta-amyloid

Question 9

What pathological protein(s) (misfolded) is associated with dementia with lewy bodies?

Answer 9

Alpha-synuclein
UbiquitinAlpha-synuclein
Ubiquitin

Question 10

What pathological protein(s) (misfolded) is associated with Corticobasal degeneration?

Answer 10

Tau

Question 11

What pathological protein(s) (misfolded) is associated with Picks disease?

Answer 11

Tau

Question 12

What pathological protein(s) (misfolded) is associated withfrontotemporal dementia linked to Chr 17?

Answer 12

Tau

Question 13

Which lobes are most marked in Alzheimer’s?

Answer 13

Temporal and frontal with loss of cholinergic neurones

Question 14

What is characteristic of Alzheimer’s?

Answer 14

Generalised atrophy of the brain, widened sulci, narrowed gyri and enlarged ventricles

Question 15

What is the management of Alzheimer’s?

Answer 15

Diagnosis is clinical, although PET and MRI may help
Senile plaques of beta-amyloid protein and neurofibrillary tangles of tau protein

Treatment is symptomatic: anti-cholinesterases, nAChR agonists, glutamate antagonistsDiagnosis is clinical, although PET and MRI may help
Senile plaques of beta-amyloid protein and neurofibrillary tangles of tau protein

Treatment is symptomatic: anti-cholinesterases, nAChR agonists, glutamate antagonists

Question 16

What are the symptoms of dementia with lewy bodies?

Answer 16

Psychological disturbances occur early
Day-to-day fluctuations in cognitive performance
Visual hallucinations
Spontaneous motor signs of Parkinsonism
Recurrent falls and syncope

This is pathologically indistinguishable from PDPsychological disturbances occur early
Day-to-day fluctuations in cognitive performance
Visual hallucinations
Spontaneous motor signs of Parkinsonism
Recurrent falls and syncope

This is pathologically indistinguishable from PD

Question 17

Define multiple sclerosis

Answer 17

Autoimmune demyelinating disease

Question 18

How is MS classified?

Answer 18

Primary progressive (10% - get continually worse)
Relapsing remitting (better between episodes but progresses over years)

Question 19

How does MS usually present?

Answer 19

With focal symptoms i.e. optic neuritis, poor coordination.

Question 20

MS Buzzwords are:

Answer 20

Myelin basic protein

Proteo-lipid protein

Question 21

What is the pathology of MS

Answer 21

MS plaques showing sharp margins of myelin loss

Question 22

Define idiopathic parkinsons disease

Answer 22

Decreased stimulation of the motor cortex by the basal ganglia (caused by death of dopaminergic neurons in the substantia nigra)

Question 23

What are the symptoms of PD?

Answer 23

TRAP

Tremor
Rigidity
Akinesia
Postural instability

Some develop psychiatric features later in the disease e.g. PD dementia, hallucinations, anxiety

Question 24

What can pathologically be seen in PD?

Answer 24

Lewy bodies are present in affected neurones
Alpha synuclein protein is the main component
Mutations are reported in famililal PD

Question 25

Define Multiple System Atrophy

Answer 25

Degenerative neurological disorder that can present in a very similar manor to Parkinsons but shows a porr response to Parkinsons medication

Question 26

Multiple system atrophy is characterised by:

Answer 26

Shy Drager: Autonomic dysfunction
Striatonigral: Difficulty with movement
Olivopontocerebellar: Difficulty with balance and co-ordination