Demyelinating Diseases

Question 1

Name three characteristic MS syndromes

Answer 1

1. optic neuritis
2. transverse myelitis
3. intranuclear ophthalmoplegia

Question 2

How can optic neuritis present?

Answer 2

loss of acuity
red desaturation (decreased ability to distinguish color)
optic disc atrophy/pallor
RAPD

Question 3

How can transverse myelitis present?

Answer 3

PATCHY inflammatory demyelination in spinal cord…partial lesion does not mimic complete cord transection

can present with unilateral/bilateral weakness or sensory loss below lesion. Can have bowel/bladder incontinence. Tingling/pain around torso at level of lesion.

Question 4

How does INO present?

Answer 4

inability to adduct eye during contralateral gaze with nystagmus of abducting eye; convergence is SPARED

Question 5

tingling electric sensation down the spine when patient flexes neck

Answer 5

Lhermitte’s sign

Question 6

worsening of MS symptoms in the heat

Answer 6

Uhthoff’s phenomenon

Question 7

discrete episodes of neurologic dysfuction that resolve after a period of time

Answer 7

relapsing remitting MS

relapses = “flares”

Question 8

recovery from each MS relapse is incomplete and baseline function deteriorates

Answer 8

secondary progressive

Question 9

relentlessly progressive course from onset of MS with superimposed relapses

Answer 9

progressive relapsing

Question 10

relentlessly course from MS onset with NO relapses

Answer 10

primary progressive

Question 11

features predicting good prognosis for MS

Answer 11

young age of onset
female
primarily sensory symptoms
mild relapses with little/no residual deficits
optic neuritis WITHOUT MOTOR SYMPTOMS

Question 12

what imaging modality good for picking up new MS lesions

Answer 12

T2 MRI
FLAIR

t2 hyperintesne ovoid lesions are classic

Question 13

Classic CSF finding MS

Answer 13

oligoclonal bands

Question 14

mechanism of oligoclonal bands on CSF

Answer 14

intrathecal production of IgG antibodies by plasma cell clones

Question 15

Besides oligoclonal bands what else can MS CSF show?

Answer 15

moderate pleiocytosis and elevated protein

Question 16

what can visual evoked potentials be used for?

Answer 16

document evidence of old optic neuritis

Question 17

chronic treatment options for MS

Answer 17

interferon beta 1a/1b
glatiramer acetate
natalizumab
fingolimod

Question 18

natalizumab increases risk of?

Answer 18

progressive multifocal leukoencephalopathy

Question 19

What labs to get before starting interferon?

Answer 19

CBC and LFTs

can cause leukopenia and transaminitis

Question 20

MOA natalizumab

Answer 20

monoclonal antibody against alpha 4 integrins that prevents lymphocytes and monocytes from crossing BBB

Question 21

MOA fingolimod

Answer 21

mixed agonist/antagonist of sphingosine 1p1 receptor (first oral MS med; all others are injectable)

Question 22

what must be monitored when patient is on fingolimod

Answer 22

ECG watch out for bradycardia and heart block

Question 23

symptomatic treatment options for MS spasticity

Answer 23

baclofen, tizanidine, diazepam

Question 24

symptomatic treatment for MS bladder dysfunction

Answer 24

anticholinergic agents and intermittent self cath

Question 25

presents similarly to MS except usually after viral antecedent or vaccine

Answer 25

acute disseminated encephalomyelitis

Question 26

acute disseminated encephalomyelitis vs. MS

Answer 26

Unlike MS, ADEM patients will... - only have one phase (monophasic) and will usually recover well - rarely show oligoclonal bands - have more cells with lymphocytic pleocytosis

Question 27

neuromyelitis optica (NMO) = ? + ?

Answer 27

development of optic neuritis and transverse myelitis

Question 28

MS vs. NMO

Answer 28

NMO patients unlike MS, will have - greater pain component in ON/transverse myelitis - CSF pleocytosis sometimes neutrophilic predominance

Question 29

How to confirm diagnosis of NMO

Answer 29

antibodies to the aquaporin 4 channel

Question 30

dementia, focal cortical dysfunction, cerebellar abnormaliteis

Answer 30

progressive multifocal leukoencephalopathy (PML)

Question 31

What conditions associated with PML?

Answer 31

immunosuppressed states, AIDS, leukemia/lymphoma

Question 32

causative agents PML

Answer 32

JC virus! (leads to demyelination by infecting oligodendrocytes)

Question 33

patient presents with aucte confusion and cortical vision loss (blindness with preserved pupil reactivity) in the setting of rapid HTN or on transplant immunosuppressants?

Answer 33

posterior reversible encephalopathy syndrome | PRES