Degenerative Diseases of the CNS

Question 1

what are 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 imaging often normal (atrophy)

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 dementia?

Answer 3

late onset
alzheimers\vascular
lewy body

young onset
alzherimers
vascular
frontotemporal

Question 4

investigations for dementia?

Answer 4

bloods, CT / MRI

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

Question 5

what are the clues to diagnosis of dementia?

Answer 5

Speed of progression
rapid progression (CJD)
stepwise progression (vascular)

Other neurological signs
abnormal movements (Huntington’s)
parkinsonism (Lewy body)
myoclonus (CJD)

Question 6

what are the risk factors for alzheimers disease?

Answer 6

genetic

lifestyle (smoking, exercise, diet, alcohol)

Question 7

what are the symptoms of frontotemporal dementia?

Answer 7

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

Question 8

what are the non pharmocological treatment for dementia?

Answer 8

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

Question 9

what are the pharmmacological symptomatic treatment for dementia?

Answer 9

Insomnia
Behaviour (care with antipsychotics)
Depression

Question 10

what is the specific treatment for alzheimers?

Answer 10

Cholinesterase inhibitors (cholinergic deficit)
Donepezil, rivastigmine, galantamine
Small symptomatic improvement in cognition (wash-out)
No delay in institutionalisation
NMDA antagonist (memantine)

Question 11

symptoms of parkinsonism?

Answer 11

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

Pathology in basal ganglia
predominantly dopamine loss

Question 12

causes of parkinsons?

Answer 12

Idiopathic Parkinson’s disease
Dementia with Lewy bodies

Drug-induced (e.g. dopamine antagonists)

Vascular parkinsonism (lower-half)

Parkinson’s plus syndromes
Multiple system atrophy
Progressive supranuclear palsy / corticobasal degen

Question 13

what is the 2nd commonest neurodegenerative disease?

Answer 13

parkinsons disease

Question 14

diagnosing parkinsons

Answer 14

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

Question 15

treatment of parkinsons?

Answer 15

dopaminergic neuron

Question 16

later complications of dopaminergic neurons?

Answer 16

Drug-induced:
Motor fluctuations - levodopa wears off
Dyskinesias - involuntary movements (levodopa)
Psychiatric - hallucinations, impulse control

Non-drug induced esp non-motor:
Depression (20%) 
Dementia (~50% after 10 yrs) 
Autonomic:  BP, bladder, bowel
Speech, swallow
Balance