Multiple sclerosis Flashcards

(84 cards)

1
Q

what are the three defining features of MS

A

inflammatory demyelinating disease
which affects central nervous system
and is separated in time and space

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2
Q

lumpers

A

look at commonalities between clinical and patient issues

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3
Q

splitters

A

look at differences in:
- clinical course
- pathological types (Lucchinetti, 2000)
- response to treatment
- ethnic groups

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4
Q

what are isolated monophasic inflammatory demyelinating diseases

A

optic neuritis myelitis
balo’s concentric sclerosis (monophasic or progressive)

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5
Q

what are diffuse inflammatory demyelinating diseases

A

ADEM/ MDEM
AHLE

ADEM: acute demyelinating encepahlomyelitis; MDEM: monophasic demyelinating encephalomyeltis; AHLE; acute haemorrhagic leukoencephalitis

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6
Q

what are the three main types of MS

A
  1. relapsing-remitting
  2. secondary progressive
  3. primary progressive
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7
Q

primary and seconday progressive MS types can be further classified as which two types

A

non relapsing and relapsing type

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8
Q

what are Lucchinetti’s four (I-IV) pathological patterns of MS

A

I inflammation and remyelination
II inflammation and antibody/ complement
III oligodentrocyte apoptosis
IV oligodendrocyte damage including normal appearing tissue

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9
Q

what is the first (I) pathological pattern of MS

A

inflammation and remyelination

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10
Q

what is the second (II) pathological pattern of MS

A

inflammation and antiody/ complement

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11
Q

what is the third (III) pathological pattern of MS

A

oligodentrocyte apoptosis

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12
Q

what is the fourth pathological pattern of MS

A

oligodendrocyte damage including normal appearing tissue

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13
Q

what patterns of axonal loss have been researched in MS

A

greater axonal loss in areas of active demyelination
axonal loss in normal appearing white matter

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14
Q

what techniques are used to diagnose MS

A

MRI
immunology
evoked potentials

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15
Q

what are the three main principles of diagnosis

A

dissemination in time (multiple attacks) and space (lesions in more than one area)
independent immune attack in the CNS
exclude mimics

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16
Q

what is the imaging diagnostic marker of MS

A

inflammatory lesions

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17
Q

what does MRI with contrast (gadolinium) plus new inflammatory lesions show

A

new activity

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18
Q

what is an imaging marker of disability for MS

A

appearance of black holes and atrophy on T1 weighted MRI

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19
Q

what are the clinical diagnostic criteria for MS

A

disseminations in time and space

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20
Q

what are the imaging diagnostic criteria for dissemination in time

A

simultaneous asymptomatic contrast and non contrast lesions at any time
or
a new T2 weighted and/or MRI with contrast inflammatory lesions
or
another clinical attack

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21
Q

what are the imaging diagnostic criteria for disseminations in space

A

one or more lesions on T2 weighted MRI in at least two MS CNS regions

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22
Q

which CNS regions are typically associated with MS

A

periventricular
justacortical
infratentorial
spinal cord

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23
Q

what is a key pathological finding of MS

A

CSF oligoclonal bands

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24
Q

in what percentage of patients are CSF oligoclonal bands found

A

97%

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25
descibe the sensitivity and specificity of CSF oligoclonal bands as a marker for MS
CSF OCB are highly sensitive for MS but not very specific
26
what evidence is there from visual evoked potentials
delayed conduction in visual pathways for VEP P100
27
what is the genetic risk of MS
monozygotic twins with an individual gene have 30% risk compared to 3% risk in dizygotic 2-3% risk in first degree relatives 1% risk in second degree relatives
28
what is the relationship between genetics and MS risk
risk for MS increases with greater genetic similarity
29
risk of MS is greater for which familial relationships
monozygotic twins siblings dizygotic twins parent-child maternal half siblings paternal half siblings aunt/ uncle - neice/nephew cousins grandparent-grandchild adopted children | highest to lowest
30
what is the gender preponderance for MS
2:1 female preponderance
31
what is the relationship between geographical region and MS risk
geographical regions with lower UV light exposure have a higher prevalence of MS geographical regions further away from the equator (north-south) have an increased risk
32
what is the relationship between MS and migration
children of people who have migrated from low to high risk countries have an increased risk of MS compared to their parents
33
which factors may be implicated in the relationship between migration and MS
UV levels vitamin D
34
which facotor mediates the relationship between migration and MS risk
migration during early childhood
35
which modifiable risk factors are associated with MS risk
smoking obesity infection (herpes) hygiene
36
what are the characteristics of the epstein-barr virus (herpes virus) ?
causes infectious mono/ glandularr fever 95% of adults have been exposed
37
what is the hygiene hypothesis
a lack of early exposure to common pathogens in early childhood due to high levels of sanitation and hygiene increases the risk for abberant immune responses and autoimmune disease if exposed to infectious triggers in later life
38
which group level factors are related to good prognosis
young female optic neuritis/ sensory type few attacks good recovery long intervals between attacks
39
which group level factors are related to poor prognosis
older male motor/ cerebellar type frequent attacks littel recovery between attacks higher lesion load on MRI progressive types
40
what scale is used to measure disbility in MS
expanded disability status scale (edss)
41
how many grades (stages) are there on the explanded disability status scale (edss)
10
42
what is the range of the EDSS
0 (no disability) to 10 (death from MS)
43
EDSS 1-3.5
combination of functional systems (FSS) graded as minimal, mild or moderate
44
EDSS grade 4.0
full ambulatory without aid for up to 12 hours a day without rest or aid up to 500 metres
45
EDSS grade 5.5
ambulatory without aid or rest for 100 metres severity of diability limits all ADLs
46
EDSS grade 6.0
intermittent unilateral assistance to walk around 100 metres with or without resting
47
EDSS grade 6.5
bilateral assistance to walk 20 metres without resting
48
EDSS grade 7.0
unable to walk five metres; can independently self transfer with a wheelchair
49
EDSS grade 8.0
restricted to bed and/or wheelchair; arms can be used effectively
50
EDS grade 9.0
confined to bed; able to eat and communicate
51
EDSS grade 9.5
unable to communicate effectively or eat/ swallow
52
EDSS grade 10
death
53
what are the eight functional systems of the FSS
(ambulatory) pyramidal cerebellar brainstem sensory bowel and bladder visual cerebral other (mood)
54
which disease type is characterised by slower progression
secondary progressive relapsing type (compared to primary progressive)
55
what factor predicts long term disability
number of attacks early on from the onset of disease
56
what is the rio and modified rio score
a measure of response to treatment in patients with relapsing remitting MS on interferon therapy
57
what are the components of the rio/modified rio score
clinical relapse disease progression (EDSS) and active MRI lesions (T2 weighted)
58
how many relapses does the avergae patient with relapsing-remitting MS have in a year
< 1
59
what classes of drugs are used to manage MS
the "-zumab" / "zimab" drugs
60
what is the 'choice of therapy' triad
RIO score + funding/ NICE guidelines + patient attitude to ris/ lifestyle/ pregnancy
61
what are the treatments for MS
first line injectables orals monoclonal antibodies HCST (haematopoietic stem cell transplantation) statins
61
what are the three overall therapeutic targets for MS treatment
blood blood-brain barrier CNS
62
How effective are first line injectables at reducing relapses?
reduce relapses by around 30%
63
what are the two first line injectables used to treat MS
beta interferon glatyrimer acetate
64
what is the role of interferons in treatment for MS
they modulate the immune response
65
what are the side effects of beta interferons
flu like symptoms
66
what are the two modes of delivery of beta interferons
subcutaneous or intramuscular injection
67
which beta interferon medications are administered via subcutaneous injection
betaseron and rebif
68
which beta interferon medications are administed via intramuscular injection
avones and plegridy
69
what is glatyrimer acetate
a polymer of myelin
70
what is an advantage of glatyrimer acetate
you don't need to monitor blood
71
what is an advantage of both beta interferons and glatyrimer acetate
they are both safe in preganancy
72
what is the most widely prescribed treatment for MS and via which route is it administered
oral dimethyl fumerate
73
which oral medications are used to treat MS
teriflunomide dimethyl fumerate fingolimod and other "-mod" oral drugs | the "-mod" drugs are S1P inhibitors sphingosine-1-phosphate
74
what is a disadvantage of oral treatments for MS
a lot of adverse side effects on multiple systems increased risk of infection unsafe in preganancy
75
what is the mechanism of natalizumab
natalizumab blocks migration by blocking integron
76
what are potential cellualr treatment targets for MS
B cells
77
what is HSCT for MS
Autologous hematopoietic stem cell transplantation involves taking stem cells from a patient, giving them chemotherapy to temporarily "wipe out" their immune system and then reintroducing their stem cells to repopulate a healthy immune system that won't attack their myelin
78
what are the two main causes of depression in MS
reaction to illness and biological role of brain dysfunction, disability and frontal/ temporal lesions
79
cortical dementia
the loss of specific functions due to cortical lesions
80
subcortical dementia
characterised by general slowing, apathy, depression, difficulty accessing functions
81
what are the two general categories of pain aetiology in MS
neuropathic and other
82
what are the neuropathic causes of pain in MS
trigeminal neuralgia limb pain tricyclic anitdepressants (e.g. duloxetine) AEDs (carbamezepine, pregabalin)
83
what other complications cause pain in MS
spasticity musculoskeletal pain psychogenic component constitutional symptoms immobility e.g. bedsores