CM: MS and Related

Question 1

What is MS?

Answer 1

autoimmune process leading to demyelination and neurodegeneration

Question 2

What are the statistics involving ambulation at 10 yrs?

Answer 2

50% will require cane

15% will require wheelchair

Question 3

What percentage of pts with MS will develop secondarily progressive MS (SPMS) after 10 yrs?

Answer 3

50% of RRMS

Question 4

What are the four characteristics that predict a severe course of MS?

Answer 4

motor and cerebellar symptoms
disability after the first attack
short time interval between attacks
lesion burden on MRI (high load, or change in load w/i first yr)

Question 5

What are the symptoms of RRMS?

Answer 5

relapses comprised of visual blurring (optic neuritis), diplopia, vertigo, numbness, paresthesias, and weakness (myelopathy)
incomplete healing after attack –> slight residual symptoms, cumulative disability in long term

Question 6

What are the symptoms of SPMS?

Answer 6

more pronounced, steady, progressive decline in fxn, w or w/o superimposed relapses
ambulation difficulties, bladder, bowel and sexual dysfunction = myelopathic features
= transition from inflammatory to degenerative

Question 7

What are the symptoms of PPMS?

Answer 7

gradual decline from onset, myelopathic features
progressive relapsing MS is subset - rare relapses on slow progression, paucity of MRI lesions, F:M = 1:1, lack of response to immunomodulatory agents used in RRMS

Question 8

What are the basics of diagnosing MS?

Answer 8

lesions in time and space
McDonald criteria - combo of clinical hx, PE, MRI and paraclinical tests (evoked potentials, CSF)
spinal cord lesions
IV gadolinium enhancement - breakdown of BBB, differentiates active from chronic lesions

Question 9

What is the classic MRI finding in MS?

Answer 9

PERIVENTRICULAR, perpendicular, ovoid lesions

infratentorial lesions = Dawson’s fingers, can find on FLAIR

Question 10

What are evoked potentials?

Answer 10

measuring slowing of electrical signal propagation can indicate demyelination
better w auditory, visual, and sensory neural
somatosensory and brainstem auditory have low sensitivity

Question 11

What is the clinically isolated syndrome of MS?

Answer 11

one attack and no evidence of second relapse
one symptom or abnormality w/o evidence of classic lesions
majority who will develop MS have lesion on MRI at time of first event
present w spinal cord syndrome, optic neuritis, or brainstem symptoms

Question 12

What are three main presenting symptoms of MS?

Answer 12

optic neuritis
transverse myelitis
intranuclear ophthalmoplegia

Question 13

What is optic neuritis?

Answer 13

inflammation and demyelination of optic nerve –> blurred vision, blindness, pain w ocular movement, color desaturation, afferent pupillary defect = Marcus Gunn pupil (affected lacks direct response), disc usually normal but pallor can happen with time

Question 14

What is transverse myelitis?

Answer 14

numbness and paresthesias, weakness, spasticity, pain, bowel bladder and sexual dysfunction
predilection for lesions in posterior columns

Question 15

What is Lhermitte’s sign?

Answer 15

electrical sensation down arms or legs w flexion of neck due to inflammation of cervical spinal cord
common complaint of transverse myelitis

Question 16

What is intranuclear ophthalmoplegia?

Answer 16

lesions in brainstem typically in periventricular pontine tegmentum
lesions of MLF produce classic adductor slowing of contralateral eye (intact abduction)

Question 17

What are some differences between MS and other autoimmune disorders that can present similarly?

Answer 17

lesions typically more peripheral in deep white matter in others
exceptions = sarcoid - lesions can be in periventricular areas, tumors - lesions can be in CC

Question 18

What are the basics of treatment of MS?

Answer 18

IV steroids for relapses and optic neuritis

preventative - immunomodulators (interferon, monoclonal antibodies, IVIG/plasmapharesis) and immunosuppressives

Question 19

What can cause relapse w symptom exacerbation and what is seen on MRI?

Answer 19

heat, fatigue, UTI - inciting factor should be IDed and improvement should be w/i 1-2 days of Rx
lack of new lesions, gadolinium enhancement

Question 20

What is neuromyelitis optica?

Answer 20

bilateral optic neuropathy and myelitis
serum Ab against aquaporin 4 (NMO-IgG)
brain MRI usually has few hyperintensities and myelitis usually multi-segmental

Question 21

What is acute disseminated encephalomyelitis (ADEM)?

Answer 21

multiple enhancing lesions in brain w multifocal symptoms, typically monophasic course
often following vaccination or viral inf
rx = corticosteroids

Question 22

What is central pontine myelinolysis?

Answer 22

noninflammatory demyelinating of pons - rapid overcorrection of hyponatremia
confusion, gaze paralysis, and quadriplegia