Neurology -6.06 Multiple Sclerosis And Vision Loss

Front 1

What is MS?

Back 1

disorder of CNS
immune system attacks myelin
episodes of inflammation or chronic condition
results: multiple scars on myelin sheath -> loss of proper nerve function

Front 2

What are some epidemiological facts about MS?

Back 2

genetic susceptibility
latitude has an effect
higher sun exposure during childhood and adolescence = reduce rx
W>M
associated with high socio-economic class

Front 3

When is the usual age of onset for MS?

Back 3

20-40years

Front 4

How does migration from a high prevalence area from a low prevalence area affect risk of MS?

Back 4

take high risk with them if migrate after puberty

Front 5

What HLA has the strongest association with MS?

Back 5

Class II allele DR15 (2x risk)

Front 6

What is MS characterised by pathologically?

Back 6

Periventricular cuffing (demyelination around ventricles)
Multifocal plaque like demyelination
Reactive glial scar formation
Predominantly T cells and macrophages
BBB disrupted

Front 7

What are T cells usually reactive to?

Back 7

myelin basic protein (MBP

Front 8

Are MAC in blood characteristic of MS?

Back 8

Yes

Front 9

What are autoantibodies reactive to in MS?

Back 9

myelin oligodendrocyte glycoprotein (MOG)

Front 10

How might weakness in MS manifest as?

Back 10

loss of strength or dexterity
fatigue
disturbance of gait
exercise induced ror heat weakness (characteristic)
UMN type
Pyramidal signs e.g. spasticity, hyperreflexia, babinski sign

Front 11

How might sensory symptoms of MS manifest as?

Back 11

paresthesia (e.g. pins and needles or painful burning)

hypesthesia (e.g. reduced sensation, numbness)

Front 12

What is spasticity due to?

Back 12

basal ganglia problem

Front 13

What type of eye complaints may and MS patient present with?

Back 13

Optic neuritis may present as diminished visual acuity, dimness or decreased colour perception in the central field of vision

Symptoms usually monocular

Periorbital pain (increased with eye movement) often precedes visual loss

May have papillitis + optic atrophy

Front 14

What might diplopia in MS be caused by?

Back 14

internuclear opthalmoplegia or CN6 palsy (rarely CN3 or 4)

INO = impaired adduction due to lesion in MLF + nytagmus in abducting eye

Front 15

Is unilateral or bilateral INO suggestive of MS?

Back 15

bilateral

Front 16

What are some bladder and bowel problems a patient with MS might have?

Back 16

Detrusor hyperreflexia -urinary frequency, urgency, nocturia, loss of control

Detrusor sphincter dyssynergia = loss of sync between detrusor and sphincter muscle -> difficulty initiating/stopping = urinary retention, increase residual volume, recurrent infection

Front 17

How common is constipation in MS?

Back 17

>30%

can have urgency/incontinence, but rarer (15%)

Front 18

How is facial weakness in MS different from idiopathic Bell's palsy?

Back 18

facial weakness is generally not associated with ipsilateral loss of taste sensation or retroauricular pain

Front 19

Describe the clinical course of RRMS

Back 19

85% of MS
discrete attacks over days-weeks
complete recovery over weeks-months
neurologically stable between attacks

if ambulation is severely impaired during an attack approx 50% fail to improve

Front 20

Describe the clinical course of SPMS

Back 20

always beings as RRMS (approx 50% will progress to SPMS in 15years)
steady deterioration unassociated with acute attacks
greater amount of fixed neurologic disability

Front 21

Describe the clinical course of PPMS

Back 21

15% of cases
no attacks but steady decline
sex distribution more even
mean age 40 years for onset
disability occurs faster

Front 22

Describe the clinical course of PRMS

Back 22

overlaps PPMS and SPMS
5% cases
steady deterioration
experience occasional attacks
early stages are indistinguishable from PPMS

Front 23

How would you definitely diagnose MS?

Back 23

No definitive diagnosis

Front 24

What is the diagnostic criteria for MS?

Back 24

documentation of two or more episodes of symptoms and two or more signs that reflex pathology in anatomically noncontiguous white after tracts of CNS

Symtoms last for >24h & occur as distinct episodes separated by a month or more

Front 25

If you sent your patient with MS for an MRI what would you expect to see?

Back 25

increase in vascular permeability from a breakdown of BBB = leakage of IV Gd into parenchyma

Happens early in MS
useful marker of inflammation

Front 26

For an MS patient sent for a spin-echo (t2 weighted) and proton density images what would you see?

Back 26

residual MS plaques (focal areas of hypersensitivity)

Front 27

What distribution would you expect to see lesions of MS in the brain, BS and SC

Back 27

Dawson's fingers

Perpendicular to ventricular surface, corresponding to pathologic pattern of perivenous demyelination

Front 28

What MRI feature would help you distinguish between MS and cerebrovascular disease?

Back 28

lesions in the anterior corpus callosum

usually spared in cerebrovascular disease

Front 29

What does evoke potential test?

Back 29

assess function of afferent or effect CNS pathways

Provide most info when pathways are not involved

Front 30

How are evoked potential used in the diagnosis of MS?

Back 30

Abnormalities on more or more modalities occur in 80-90% of MS patients

not specific to MS

But marked delay suggestive of demyelination

Front 31

What are some CSF abnormalities found in MS?

Back 31

mononuclear pleocytosis
increase intrathecal synthesis of IgG

Front 32

What would the result of a measurement of oligoclonal banding in a MS patient be?

Back 32

assesses intrathecal IgG2
2 or more bands in MS patient (75-90%)
may not be there at onset but number of bands increases with time

Front 33

When should another diagnosis be considered in MS?

Back 33

1) symptoms localised exclusively to posterior fossa, craniocervical junctionor SC

2) patient <15 or >60yo

3) progressive from onset

4) never experienced visual, sensory or bladder symptoms

5) investigation results are abnormal

Front 34

How would you manage first attacks or acute exacerbation of MS?

Back 34

glucocorticoids

reduces severity and shortens duration of attacks

Front 35

What would you do if a MS patient does not respond to glucocorticoids?

Back 35

plasma exchange

7 changes every other day for 14 days may benefit fulminant attacks

Front 36

What are some disease modifying drugs for MS?

Back 36

IFN beta -1b
IFN beta -1a
Copaxone -copolymer-l
Natalizumab

Front 37

How does IFN beta modify MS?

Back 37

reduce attack rate
improves disease severity

Front 38

How does copaxone modify MS?

Back 38

reduced attack rate in RRMS
may reduce disease severity

Front 39

What type of drug is Glatiramer acetate?

Back 39

Copaxone

Front 40

How does Natalizumab work in MS?

Back 40

binds to alpha 4-integrin on lymphocytes
inhibit T cells to endothelial cells thus migration across BBB

approx. twice as effective in preventing MS attacks

infusion every 4 weeks

Front 41

What type of drug is Novantrone?

Back 41

Mitoxantrone Hydrochloride

Front 42

How does Mitoxantrone modify MS?

Back 42

an anthracenedione = antineoplastic actions

1) intercalate to DNA = strand breaks and inter-strand cross-link
2) interfere with RNA synthesis
3) inhibit topoisomerase 2 (inhibit DNA repair)

Front 43

In what type of MS is mitoxantrone used in?

Back 43

SPMS
PRMS

but not used as first line treatment due to SE (CHF, reduced ejection fraction, amenorrhea, risk of acute leukaemia)

Front 44

What is the prognosis of MS?

Back 44

50% of patients need help walking within 15 years
Avg survival = 30years

Front 45

What do MS patients commonly die from?

Back 45

pyelonephritis from recurrent UTI

Front 46

Where is demyelination more prominent in MS?

Back 46

Optic nerves
Periventricular white matter
BS
Cerebellum
SC