Neurodegenerative Diseases

Question 1

What are the common features of neurodegenerative diseases?

Answer 1

Aetiology largely unknown
(mendelian genetic causes rare)

Usually late onset

Gradual progression

Neuronal loss (specific neuropathology)

Structural imaging often normal (atrophy)

Question 2

Dementia is a syndrome consisting of….

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

What is the epidaemiology of dementia?

Answer 3

Incidence = 200 per 100,000
Prevalence 1,500 per 100,000
>750,000 patients in UK

Question 4

What is the cost of dementia?

Answer 4

Devastating impact on patient and family

£20 billion per year (£6 billion by carers)
-66% of those in care homes have dementia

Question 5

What are the causes of late onset (65+ years) dementia?

Answer 5

Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)

Question 6

What are the causes of young onset (

Answer 6

Alzeimer's (33%)
Vascular (15%)
Frontotemporal (15%)
Other (33%)
-Huntington's, CJD
-Infection (HIV)
-Inflammatory (MS)
-Toxic (Alcohol)

Question 7

What are the treatable causes of dementia like conditions?

Answer 7

Vitamin deficiency (B12)
Endocrine (Thyroid disease)
Infective (HIV, Syphilis)

Question 8

What are mimics of dementia?

Answer 8

Hydrocephalus
Tumour
Depression “pseudodementia”

Question 9

How do you diagnose dementia?

Answer 9

History (independent witness)

• Type of deficit
• Progression
• Risk factors
• Family history

Examination:

• Cognitive function
• Neurological
• Vascular

Investigations:

• Routine (bloods, CT/ MRI)
• Others (CSF, EEG, Functional imaging, genetics)

-Investigations to exclude other causes

Question 10

What are the various domains for examining someones cognitive function?

Answer 10

Memory
Attention
Language
Visuospatial
Behavior
Emotion
Executive function
Apraxias
Agnosias

Question 11

What screening tests can you use for examination of cognitive function?

Answer 11

Screening tests:

• Mini mental (MMSE)
• Montreal (MOCA)

Mini mental misses alot so montreal may be better

NEUROPSYCHOLOGICAL ASSESSMENT

Question 12

What is apraxia?

Answer 12

Cannot plan complex motor tasks.

Wants to put a t-shirt on but cannot plan out all the sequences of movement to do so

Question 13

What is agnosia?

Answer 13

Inability to interparate sensations and therefore recognise things.

Can be handed a coin with their eyes closed. Will be able to feel it and describe it in detail but not actually recognise its a coin

Question 14

What are the clues to diagnosis in neurodegenerative diseases?

Answer 14

Type of cognitive deficit

Speed of progression

• Rapid (CJD, tumour, infection)
• Stepwise progression (vascular)

Other neurological signs:

• Abnormal movements (Huntington’s)
• Parkinsonism (Lewy body)
• Myoclonus (CJD)

Question 15

What are the signs of temporo-parietal dementia?

Answer 15

Early memory disturbance

Language and visuospatial problems

Personality preserved until later

Question 16

What are the signs of frontotemporal dementia?

Answer 16

Early change in personality/ behaviour

Often change in eating habits

Early dysphagia

Memory/ visuospatial relatively preserved

Question 17

What is the non-pharmacological symptomatic treatment for dementia?

Answer 17

Information and support, dementia services

Occupational therapy

Social work/ support/ respite/ placement

Voluntary organisations

Question 18

What is the pharmacological treatment of dementia?

Answer 18

Insomnia

Behaviour (care with antipsychotics, consider behavioural therapy)

Depression

Question 19

What is the specific treatment for Alzheimers?

Answer 19

(+/- Lewy body dementia)

Colinesterase inhibitors (cholinergic deficit)

• Donepezil, rivastigmine, galantamine
• Small symptomatic improvement in cognition (wash out)
• No delay in institutionalisation

NMDA antagonist (memantine)
-Basically same as above

Question 20

What is parkinsonism?

Answer 20

A clinical syndrome with 2 or more of:

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

Pathology in basal ganglia:
-Predominantly dopamine loss

Question 21

How do you diagnose Parkinson’s disease?

Answer 21

1. Bradykinesia + >/= 1 tremor, rigidity, postural instability
2. No other cause / atypical features
3. Slowly progressive (>5-10 years)

Supported by asymmetrical onset, rest tumor, response to dopamine replacement treatment

Less likely if repid progression, symmetrical, lack of rest tremor, poor response to treatment, early falls, early dementia, other abnormal neurological signs

Question 22

What are the drug induced complications in parkinsons?

Answer 22

Motor fluctuations: drugs wear off

Dyskinesias: involuntary movements

Psychiatric: hallucinations, impulse control

Question 23

What are the later complications of parkinson’s that are non-drug induced?

Answer 23

Depression (20%)
Dementia (about 50% after 10 years)
Autonomic: BP, Bladder, bowel
Sensory and sleep
Poor swallowing
Falls

Question 24

What can be done in the late treatment of parkinson’s disease?

Answer 24

Prolong levodopa half life

• MAO-B inhibitors
• COMT inhibitor
• Slow release levodopa

Add oral dopamine agonist
Continuous infusion (apomorphone, duodopa)

Functional neurosurgery = deep brain stimulation

Allied health professionals +/- care package