Multiple Sclerosis

Question 1

features suggestive of MS

Answer 1

onset between 15-50, optic neuritis, Lhermitte sign, internuclear ophthalmoplegia, fatigue, Uhthoff phenomenon, sensory sx of numbness and paresthesia, motor symptoms of paraparesis, spasticity, bowel and bladder dysfunction

Question 2

Uhthoff phenomenon

Answer 2

heat sensitivity

Question 3

Lhermitte sign

Answer 3

electrical shocks up the spine

Question 4

Types of MS

Answer 4

relapsing remitting (majority), primary progressive, secondary progressive, progressive relapsing

Question 5

Diagnosis of MS

Answer 5

T2 MRI lesions seen in time and space (periventricular juxtacortical and infratentorial or spinal cord.

Oligoclonal IgG bands on CSF fluid

Question 6

what to see on CSF when diagnosing MS?

Answer 6

oligoclonal IgG bands and elevated IgG index (90%)

Question 7

manifestations of optic neuritis

Answer 7

acute, peaks at 2 weeks, monocular vision loss, eye pain with movement,
washed out color vision
afferent pupillary defect

Question 8

diagnosis of optic neuritis

Answer 8

need MRI of orbits and brain

look for MS

Question 9

treatment of optic neuritis

Answer 9

IV steroids and 35% of cases reoccur

need to rule out MS

Question 10

Who gets optic neuritis

Answer 10

young women and strongly associated with multiple sclerosis and associated with immune mediated demyelination

can also be a clinically isolated syndrome but can also progress to MS.

Question 11

acute vision loss, decreased color perception and pupillary defect, headache and pain with eye movement

Answer 11

optic neuritis

may see swelling and hyperemia of disk on funduscopic exam. But 66% of all pts also haven normal funduscopic exam.

Question 12

If suspecting optic neuritis you need to get a

Answer 12

MRI to rule out signs of demyelinating disease and MS

Optic neuritis may be isolated clinical syndrome and so need to assess risk for progression to MS. High risk for progression is multiple demyelinating plaques and may be started on early disease modifying therapy like interferon or galtiramer acetate for relapse prevention.

Question 13

Surveillance schedule of MRI with optic neuritis

Answer 13

Get MRI on presentation and then 5 months and 1 year and imaging remaining negative no further imaging

normal brain MRI have a 20% risk of conversion to MS.

Question 14

Clinically isolated syndrome in MS is a

Answer 14

defined as a 1st episode of CNS dysfunction caused by demyelination and it may progress to MS. All pts should undergo a MRI to evaluate for demyelination plaques and assess MS risk stratification and abnormal MRI findings need to get dx modifying therapy

Question 15

what is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)?

Answer 15

this is an acquired inflammatory disorder that has demyelination of peripheral nerves and nerve roots that presents as progressive, symmetric and proximal and distal muscle weakness along with a distal sensory loss affecting the arms and legs. This is NOT MS.

Symptoms last over 8 weeks and help differentiate CIDP from acute inflammatory demyelinating polyneuropathy or AIDP

Question 16

what is on CIPD presentation

Answer 16

distal sensory loss with vibratory and proprioception loss is seen.

progressive, symmetric proximal and distal muscle weakness

see diminished or absent deep tendon reflexes are also characteristic.

Question 17

chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) diagnosis

Answer 17

from electrodiagnostic studies which show peripheral nervous system demyelination (reduced conduction velocity and prolonged distal motor latencies and LP can show albuminocytologic disassociations in 90% of pts

nerve biopsy is limited and but it can now be used to help confirm segmental meylin loss when electrodiagnostic studies are indeterminate.

Question 18

what is AIDP also known as?

Answer 18

guillain barre syndrome

Question 19

acute inflammatory demyelinating polyneuropathy or AIDP or Guillain Barre syndrome is caused by

Answer 19

prior antecedent illness Campylobacter infection, HIV, infection or vaccination.

See rapid onset of symptoms within 3-4 weeks.

Question 20

Lambert Eaten myasthenia gravis syndrome

Answer 20

see auto immune PRE synaptic neuromuscular junction disorder associated with malignancy (small cell lung cancer) in 50% of cases

Question 21

presentation of Lambert Eaten myasthenia gravis syndrome

Answer 21

progressive symmetrical proximal muscle weakness that improves with physical activity and see reflexes become diminished or absent but sensory function is typically preserved.

Question 22

What do you see on MRI of brain and orbits with gadolinium if patient has MS and presents with optic neuritis?

Answer 22

see periventicular white matter lesions that are >3mm and optic nerve inflammation

Question 23

relapsing remitting (majority) of MS is defined as

Answer 23

disease relapse after full or partial recovery of initial MS symtpoms

Question 24

acute MS or relapses are treated with

Answer 24

glucocorticoids but immunomodulatory agents are continued indefinitely to decrease relapse rate and slow accumulative lesions on MRI.

Question 25

1st line immunomodulator for treatment is

Answer 25

interferons or glatiramer acetate based on side effect profile and clinical data

Question 26

if MS pts don’t respond to 1st line agent when what are they switched too:

Answer 26

natalizumab = recombinant monoclonal atnibody against alpha 4 integrins that prevent adhesion of inflammatory lymphocytes and monocytes.

Question 27

side effect of natalizumab

Answer 27

can cause progressive multifocal leukocephalopathy (PML) which is a fatal disease caused by the JC virus

Question 28

what must be checked prior to starting natalizumab?

Answer 28

JC virus antibody because potential for fatal PML infection.

Question 29

If JC virus antibodies are positive and MS pt has failed initial interferon therapy with continued relapses, what to start

Answer 29

start fingolimod instead of natalizumab

Question 30

Which virus increases your predisposition for developing MS?

Answer 30

EBV.

Question 31

after starting fingolimod, what is there an increased risk of:

Answer 31

HSV infections.

Question 32

treatment of progressive MS is

Answer 32

limited

mitoxantrone s only FDA approved therapy for secondary progressive MS

ocrelizumab is only FDA approved therapy for primary progressive MS