Degenerative diseases of the CNS

Question 1

What is dementia?

Answer 1

a syndrome consisting of progressive impairment of multiple domains of cortical function in alert patients leading to loss of acquired skills and interference with occupation and social role

Question 2

what are the main causes of late onset dementia?

Answer 2

alzheimers
lewy body parkinsons
vascular (mini strokes)

Question 3

what are the causes on early onset dementia?

Answer 3

alzheimers (genetic)
vascular 
frontotemporal
toxic (alcohol)
genetic (huntington's)
infection (HIV, CJD)
inflammatory (MS)

Question 4

what are the treatable causes of dementia?

Answer 4

vitamin B12
hypothyroidism
HIV
syphillis

Question 5

what can mimic the presentation of dementia?

Answer 5

hydrocephalus
tumour
depression (pseudo dementia)

Question 6

what do you have to find out when taking a history form a patient and their family when assessing if they have dementia?

Answer 6

type of deficits 
progression
pattern of progression
risk factors 
family history

Question 7

what are the sub types of dementia?

Answer 7

temporoparietal (alzheimerS)
frontotemporal
vascular

Question 8

what investigations would you carry out if someone was suspected of having dementia?

Answer 8

bloods
CT/MRI
CSF (LP)
EEG
functional imaging 
genetics

Question 9

what screening tests can be carried out to examine a patients cognitive function?

Answer 9

mini mental (MMSE)
montreal (MOCA)

Question 10

what are the main clues for identifying dementia and distinguishing it from other syndromes?

Answer 10

spread of progression
- if rapid = CJD
- if stepwise = vascular 
abnormal movements = huntington's 
parkinsonism signs = lewy body

Question 11

what is agnosia?

Answer 11

no cortical association

can feel what is inside their pocket but cannot make the association as to what it is

Question 12

what are the main characteristic of alzheeimers dementia?

Answer 12

early memory disturbance
personality preserved until later
language problems
visuospatial problems

Question 13

what are the main characteristics of front-temporal dementia?

Answer 13

early personality/behaviour disturbance
change in eating habits
early dysphagia
memory / visuospatial spared until late

Question 14

what are the characteristics of vascular dementia?

Answer 14

mixed picture depending on where the vascular hit is

stepwise decline

Question 15

what are the treatments for alzheimer’s dementia?

Answer 15

cholinesterase inhibitors e.g. donepezil

NMDA antagonist e.g. memantine

Question 16

why do most patients with alzheimers present with memory loss and what type of amnesia is this termed as?

Answer 16

because the hippocampus is usually damaged first which is required for searching for memories - therefore they often present with anterograde amnesia
they can remember past memories which are held deep within the brain but can’t remember what they did this morning

Question 17

what is the criteria for diagnosing parkinson’s?

Answer 17

a clinical syndrome of >2 of;
bradykinesia
tremor
rigidity 
postural instability

Question 18

where is the pathology of parkinson’s within th brain?

Answer 18

basal ganglia

Question 19

what is the pathology of parkinson’s?

Answer 19

dopaminergic neurone loss in the basal ganglia (dopamine loss)

Question 20

what are the causes of parkinsonism?

Answer 20

lewy body
drug induced e.g. dopaminergic antagonists
vascular parkinsonism

Question 21

what are the early medical treatments of parkinson’s?

Answer 21

dopamine agonists

levodopa

Question 22

what are the late medical treatments you can give to patients who have progressive late stage parkinson’s?

Answer 22

prolong levodopa half life with MAO-B inhibitor or COMT inhibitor 
add oral dopamine agonist
functional neurosurgery (brain stem stimulation)

Question 23

what is the average progression of parkinson’s?

Answer 23

> 5-10 yrs

Question 24

what is the gait like of someone with parkinson’s?

Answer 24

narrow stance
loss of arm swing
slowness to start
shuffling

Question 25

what imaging could you use for diagnosing / assessing parkinson’s?

Answer 25

dopamine transporter SPECT

Question 26

what are the consequences of long term / too high dose of levodopa?

Answer 26

can cause chorea - Levodopa induced dyskinesia

neurological jerky involuntary movements affecting shoulder, face and hips

Question 27

what are treatable causes of dementia?

Answer 27

HIV
hypothyroidism
syphillis
vitamin B12