Degenerative Diseases of the Central Nervous System

Question 1

What are the common features of neurodegenerative diseases?

Answer 1

Aetiology largely unknown
Usually late onset
Gradual progression
Neuronal loss
Structural imaging often normal

Question 2

What is dementia?

Answer 2

A syndrome consisting of 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 are the causes of late onset dementia?

Answer 3

Alzheimer’s disease
Vascular
Lewy body

Question 4

What are the causes of young onset dementia?

Answer 4

Alzheimer's disease
Vascular
Frontotemporal
Toxic (alcohol)
Genetic (Hungtington's)
Infection
Inflammatory

Question 5

What are the treatable causes of dementia?

Answer 5

Vitamin B12 deficiency
Thyroid disease
HIV
Syphilis

Question 6

What diseases can mimic dementia?

Answer 6

Hydrocephalus
Tumours
Depression

Question 7

How is dementia diagnosed?

Answer 7

History- independent witness, type of deficit, progression, risk factors, FH
Examination- cognitive function, neurological, vascular
Investigations- bloods and CT/MRI are routine, CSF, EEG, functional imaging and genetics can also help

Question 8

How is cognitive function examined?

Answer 8

Screening tests- mini-mental (MMSE), Montreal (MOCA)

Neuropsychological assessment

Question 9

What clues help in a diagnosis of dementia?

Answer 9

Type of cognitive deficit
Speed of progression
Abnormal movements indicative of Hungtington's
Parkinsonism
Myoclonus (CJD)

Question 10

What are the characteristics of frontotemporal dementia?

Answer 10

Early change in personality/behaviour
Often change in eating habits
Early dysphasia
Memory/visuospatial realtively preserved

Question 11

What are the characteristics of vascular dementia?

Answer 11

Mixed picture of symptoms

Stepwise decline

Question 12

What is the non-pharmacological treatment of dementia?

Answer 12

Information and support- dementia services
Occupational therapy
Social work/support/respite/placement
Voluntary organisations

Question 13

What symptoms of dementia can be treated pharmacologically?

Answer 13

Insomnia
Behaviour
Depression

Question 14

What specific treatment can be given for Alzheimer’s disease?

Answer 14

Cholinesterase inhibitors- small symptomatic improvement in cognition
NMDA antagonist

Question 15

How is Parkinsonism defined clinically?

Answer 15

A clinical syndrome with at least two of:
Bradykinesia (slowness of movement)
Rigidity
Tremor
Postural instability

Question 16

What is the pathology behind Parkinsons disease?

Answer 16

Pathology in the basal ganglia, predominantly a dopamine loss

Question 17

What are the causes of Parkinsons disease?

Answer 17

Idiopathic
Drug-induced
Vascular parkinsonism

Question 18

How is Parkinson’s disease diagnosed?

Answer 18

History- Bradykinesia + at least one of tremor, rigidity and postural instability. Slowly progressive.
Suspicion supported by good response to dopamine replacement treatment

Question 19

What drug therapy can be given in early Parkinson’s disease?

Answer 19

COMT inhibitor
Levodopa
MAO-B inhibitor
Dopamine agonists

Question 20

What are the possible complications of Parkinson’s disease?

Answer 20

Drug induced:
-Motor fluctuations
-Dyskinesias
-Psychiatric- hallucinations, impulse control
Non-drug induced:
-Depression
-Dementia
-Blood pressure, bladder and bowel problems
-Speech and swallow
-Balance

Question 21

What treatment can be given in later Parkinson’s disease?

Answer 21

Prolong levodopa half life with MAO-B inhibitors, COMT inhibitor, slow release levodopa
Add oral dopamine agonist
Continuous infusion of apomorphine or Duodopa
Functional neurosurgery