Kilgo- Demyelinating Disorders

Question 1

only real primary demyelinating disorder is ____

Answer 1

MS

Question 2

4 principles to diagnose MS:

Answer 2

demyelinating event
dissemination in space
dissemination in time
other explanations ruled out

Question 3

CNS demyelination that is separated in time and space

Answer 3

Multiple Sclerosis

Question 4

= >1 clinical event by history or presence of both active and inactive plaques simultaneously on MRI

Answer 4

MS separation in time

Question 5

=exam deficits referable to >1 CNS lesion, or >1 MRI lesion

Answer 5

MS separation in space

Question 6

MS plaques are active for ___ weeks

Answer 6

6 weeks

Question 7

painful vision loss in young person

Answer 7

optic neuritis

Question 8

Optic neuritis
Transverse myelitis
Brainstem syndromes

Answer 8

common presenting symptoms of MS

Question 9

(MLF attacked and +nystagmus in unaffected eye and the affected eye cannot adduct); heavily myelinated structure in CNS so look out for demyelination

Answer 9

Internuclear opthalmoplegia

Question 10

Cognitive presentations (dementia)
Tumefactive presentations (tumor-like)
Isolated neurogenic bladder/bowel (can’t pee/poop)

Answer 10

less common presentations of MS

Question 11

Inflammation of one or both of the optic nerves
May be the first indication of multiple sclerosis (MS)

Answer 11

optic neuritis

Question 12

mimickers of MS

Answer 12

NMOSD and MOG Ab disease

Question 13

Symptoms may include pain with extraocular movement, visual field loss, flashing lights and loss of red/green color vision

Answer 13

optic neuritis

Question 14

usually in ____ only one optic nerve affected

Answer 14

MS

Question 15

in ____ and ____ both optic nerves are affected and can be at different points in time

Answer 15

NMOSD
MOG Ab disease

Question 16

Peak age of onset is 20-40 years

Answer 16

MS

Question 17

Most common cause of non-traumatic disability in young adults

Answer 17

MS

Question 18

Vision loss (optic neuritis)
Double vision (brainstem lesion)
Paresthesias/weakness (spinal cord lesion)
Appendicular or Gait Ataxia

Answer 18

MS

Question 19

supporting (not diagnostic) paraclinical test for MS

Answer 19

Oligoclonal immunoglobin bands in CSF

Question 20

on section, areas of well-demarcated discoloration seen in white matter

Answer 20

MS

Question 21

Often present in the optic nerves (20% of cases) and at the lateral angles of the lateral ventricles but can be anywhere in white matter

Answer 21

MS

Question 22

Answer 22

MS

Question 23

have myelin and their cell bodies are in the retina
see areas of demyelination with relative axonal sparing

Answer 23

optic nerve

Question 24

on Luxol Fast Blue

Answer 24

MS

Question 25

this MRI has subtracting out T2 and looks like T1 again except any white matter lesion will be accentuated

Answer 25

FLAIR

Question 26

(multiple scars) areas of gliosis that are seen from prior inflammation

Answer 26

MS

Question 27

MRI T2

Answer 27

MS

Question 28

demyelination of periventricular region

Answer 28

MS

Question 29

MRI T2

Answer 29

MS

Question 30

hypercellular with parenchymal and perivascular lymphocytes and macrophages preservation of axons

Answer 30

active plaques

Question 31

hypocellular and gliotic with loss of oligodendrocytes and variable loss of axons

Answer 31

inactive plaques

Question 32

areas of remyelination, often within larger zones of demyelination

Answer 32

shadow plaques

Question 33

Answer 33

active plaques

Question 34

Answer 34

active plaques

Question 35

Answer 35

green: active plaques red: shadow plaques

Question 36

Rare multifocal inflammatory white matter disease viral cause

Answer 36

progressive multifocal leukoencephalopathy (PML)

Question 37

caused by reactivation of JC virus (john cunningham) mainly in immunosuppressed patients

Answer 37

PML

Question 38

being on ______ and _____ are most common causes of PML in multiple sclerosis patients

Answer 38

Natalizumab and Fingolimab

Question 39

Answer 39

PML (progressive multifocal leukoencephalopathy)

Question 40

how to diagnose progressive multifocal leukoencephalopathy

Answer 40

(+) JCV DNA PCR in CSF

Question 41

Answer 41

PML

Question 42

Answer 42

PML

Question 43

Classically associated with rapid overcorrection of hyponatremia, osmotic demyelination

Answer 43

Central Pontine Myelinolysis

Question 44

Central Pontine Myelinolysis can be seen in other conditions with normal sodium level, ___ and ___

Answer 44

AIDS alcohol

Question 45

Older patient who came in hyponatremic; blood Na+ down in the 120s and gets way to rapidly corrected overnight to 140

Answer 45

central pontine myelinolysis or even locked-in syndrome

Question 46

lesion to the pons-----basilar a. stroke affecting the whole pons

Answer 46

locked-in syndrome

Question 47

Answer 47

central pontine myelinolysis

Question 48

myelin gone, but axons remain

Answer 48

central pontine myelinolysis

Question 49

Cardinal symptom: encephalopathy Neurological symptoms may be preceded (days to weeks) by a viral syndrome

Answer 49

acute disseminated encephalomyelitis (ADEM)

Question 50

decreased consciousness most often monophasis event (compared to MS which is multiple events)

Answer 50

ADEM (acute disseminated encephalomyelitis)

Question 51

perivascular inflammation and "sleeve like" demyelination

Answer 51

ADEM

Question 52

Good prognosis in children, worse in adults (+) MOG antibody in serum is seen in high percentage of pediatric cases

Answer 52

ADEM

Question 53

Will look like hypertensive encephalopathy (confused, cant see) Rx: lower bp See this on MRI (affects occipital part of brain) Dialysis patients; patients with eclampsia

Answer 53

posterior reversible encephalopathy syndrome (PRES)

Question 54

inability of posterior circulation to autoregulate

Answer 54

PRES (posterior reversible encephalopathy syndrome)

Question 55

Genetic cause of peripheral neuropathy FOOT DROP PMP-22 involved

Answer 55

Charcot-Marie-Tooth

Question 56

family history important foot drop high foot arch decreased tendon reflex

Answer 56

Charcot-Marie-Tooth

Question 57

"onion bulb"

Answer 57

Charcot-Marie-Tooth

Question 58

Immune system attack on peripheral myelin Usually doesn’t happen again and again (unlike MS in CNS)

Answer 58

guillain-barre syndrome

Question 59

can happen after Infection: campylobacter or mycoplasma

Answer 59

Guillain-Barre syndrome

Question 60

Weakness and tingling or loss of sensation starting in the feet and legs then spreading to the upper body and arms (ascending)

Answer 60

Guillain-Barre syndrome

Question 61

main treatment for guillain-barre syndrome

Answer 61

IVIG

Question 62

peripheral nerve

Answer 62

guillain-barre syndrome