Demyelination and autoimmunity / paraneoplastic Flashcards

0
Q

natural course of eye issues in optic neuritis

A

2/3 w/ 20/20 vision after recovered

relative APD persists in 90%

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1
Q

Optic neuritis:

Risk of MS if normal brain MRI vs lesions on MRI

A

25% –> 75%

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2
Q

most common site of spinal cord lesions in MS

A

1-2 spinal cord levels, usually on edge near posterior columns, usually partial

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3
Q

Pts w/ Transverse myelitis have what % risk of developing MS depending on MRI

A

nl MRI: 10%

lesions on MRI: 85% chance in next 10-14 yrs

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4
Q

4 characteristic lesion areas in MS

2 bonus

A

juxtacortical (U fibers)
Periventricular
Infratentorial
spinal cord

*4th vent (near pial surface) and near middle cerebellar peduncle AND Corpus callosum**

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5
Q

MRI location clues supporting MS vs vascular lesions.

A
MS: deep white vs periph white
Juxta cortical involving U fibers at grey/white junction
Corpus callosum lesions
Near pia of 4th vent/aqueduct
Spinal cord

Vascular loves BG and deep pons/MB, no spinal cord or T1 lesions, rarely clalosal

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6
Q

typical CSF in MS?

A

normal WBC and protein (slight elevation)

May have _ OCB or incr IgG index

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7
Q

How can OCB in CSF be useful for prognosis?

A

in pts w/ clinically isolated syndrome, OCBs show incr risk of developing MS

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8
Q

CSF findings that should give you a double take if you suspect MS?

A

Protein >100
WBC >50
PMNs **rethink dx

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9
Q

3 medications that may delay onset of MS in pts presenting with clinically isolated syndrome

A

Disease modifying treatments:

  1. Interferon B1a
  2. Interferon B1b
  3. Glatiramer acetate (Copaxone)
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10
Q

% of pts with primary progressive MS

A

10-15%

No good treatment, never get better

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11
Q

MS Medication most likely to cause depression

A

Interferons

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12
Q

Treatment of acute optic neuritis

A

Just give IV steroids for 3d, then oral taper over 11 days, then 14 days 1mg/kg oral
(but lousy trials)

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13
Q

Copaxone s/e?

A

post-injection reaction / chest pain
Injection site reaction
CAtegory B pregnancy!

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14
Q

MS drug w/ CBC, LFT, thyroid abnormalities and Depression, and flu like sx

A

Interferons

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15
Q

MS drug with most important risk of infections, particularly which?

A

Tysabri/Natalizumab: esp PML/JC virus

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16
Q

Mitoxantrone MS drug need what additional work up

A

if exposed: regular echo and bloodwork even when off the drug
(CHF and leukemia ris)

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17
Q

classic PML doesn’t enhance except in? what %

A

MS, 50%

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18
Q

Test to look for JC virus?

A

CSF JC virus PCR (viral load may be undetectable though)

Ab testing is predictive not diagnostic

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19
Q

Risk of PML if JC virus ab positive after 24 Natalizumab tx and after prior immunosuppression

A

1:200, 1:100 if other drugs first

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20
Q

Recommended screening for MS patient on Natalizumab?

A

q6mos ab treatment

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21
Q

MS drug that binds to S1P1 receptors to internalize them thereby preventing S1P1 from permit lymphocytes to exit lymph nodes

A

Fingolimod/Gilenya

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22
Q

Why do you need to observe for first dose of Fingolimid/Gilenya
What other major s/e?

A

bradycardia / AV conduction block w infusion

also causes macular edema

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23
Q

avoid Fingolimod in what patients?

A

those with serious heart disease

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24
Q

MS drug that inhibits dihydro-orotate dehydrogenase necessary for pyrimidine synthesis /DNA+RNA in rapidly dividing lymphocytes

A

Teriflunomide / Aubagio

25
Q

Major risk / s/e issues wtih Teriflunomide / Aubagio?

A

transient hair loss, myelosuppression and LFTs, infx, TERATOGEN CATEGORY X for M/F!

26
Q

New MS med oral anti-inflammatory that creates Th1-Th2 shift and apoptosis of active lymphocytes and has GI distress, flushing, HA as major s/es

A

Dimethyl fumarate, BG-12

27
Q

weakness and hiccups, think what dz?

A

NMO

28
Q

CSF in NMO vs MS?

A

WBC higher in NMO and often with PMN predominance during acute attacks
20-30% w/ OCB which usu aren’t persistent as they are in MS

29
Q

antibody test in NMO and how sensitive /specific

What does it indicate

A

NMO IgG aquaporin 4 is 75% sens and 90% specific for NMO vs MS
Higher rate of relapse within 1 yr if positive

30
Q

What do you follow in patient treated with Rituximab?

A

CD-19

31
Q

Typical MRI appearance in ADEM

A

large uniformly enhancing lesions, fluffy
involves grey and white, including deep thal and BG
spinal cord can be large and swollen lesions

32
Q

seasons for ADEM

A

kid in winter or spring

33
Q

expected lab findings in ADEM

A

CSF with elev WBC esp lymphocytic, ESR and CRP increased
Normal to elevated protein in CSF
No OCBs

34
Q

treatment for ADEM

A

usually great response to steroids, with full recovery often by 6 mos (high dose, then taper 4-6 wks)
Often usu covered for infx too for r/o

35
Q

What is Weston Hurst disease and its distinguishing feature and prognosis

A

acute hemorrhagic leukoencephalitis: most fulminant demyelinating disease in young adults > children
Look for punctate hemorrhages in what otherwise looks like ADEM and TREAT QUICK, can be fatal

36
Q

vaccine with larger concern for post-vaccine encephalomyelitis

A

rabies, may have high mortality rate

37
Q

Dx: cranial neuropathies, endocrinopathy, aseptic meningitis, MRI with diffuse meningeal enhancement and noncaseating granulomas

A

neurosarcoidosis

38
Q

MRI findings in Behcet (oral ulcers, genital ulcers, anterior uveitis)

A

brainstem, diencephalon, GB involvement

39
Q

temporal pole involvement of white matter changes suggests what?

A

CADASIL

40
Q

Drug that works by reducing proliferation of T cells and TNF alpha proliferation, altering cytokines to make more TH2, increase IL 10, and decrease immune cells across BBB via adhesion molecules?

A

IFN-B 1a

41
Q

Drug that works by promoting proliferation of TH2 cytokines, competing with MBP for presentation on MHC II molecules, altering function of macrophages, and inducing antigen-specific suppressor T cells

A

Glatiramer acetate (copaxone)

42
Q

the presence of macrophages and myelin degradation products suggests what in MS

A

active plaque (hypercellular and patchy infiltrates of T cells and monocytes)

43
Q

what is a shadow plaque

A

circumscribed regions w/ uniformly thin myelin on axons

represent areas of remyelination / self-repair

44
Q

well demarcated areas of hypocellularity with myelin pallor or loss

A

chronic MS plaque

45
Q

In MS accumulation of APP suggests what

A

in active lesions and border of chronic active lesions, suggests axonal loss
Axonal Ovoids

46
Q

What is the significance of the P300 in MS?

A

latency of P300 is increased in MS, correlated w/ cognitive impairment/total white matter involvement. Thought to indicate info processing speed

47
Q

Ab in limbic / brainstem encephalitis and testicular cancer

A

anti-Ma

48
Q

Ab in subacute sensory neuropathy / limbic encepalopathy/encephalomyelitis and in SCLC

A

anti-Hu

49
Q

Ab w/ paraneoplatic cerebellar degen in SCLC, hodgkins, BR/Ov, female genital tract

A

Anti-Purkinje / anti-Yo Abs

50
Q

Ab in paraneoplastic opsoclonus

A

anti-Ri

51
Q

Ab in SCLC w/ subacute sensory neuropathy w/o rigidity

A

anti-amphiphysin

52
Q

antibody w/ peripheral neuropathy in Waldenstrom macroglobulinemia

A

Anti-MAG

53
Q

Ab in paraneoplastic stiff man syndrome in what Ca

Ab also found in what?

A

anti amphiphysin in BRCA

this Ab also in SCLC w/ sensory neuropathy/no rigidity

54
Q

breathing / undine’s curse: this found in what paraneoplastic syndrome?

A

Anti-Hu in SCLC limbic encephalitis / encephalomyelitis

55
Q

slowly progressive paraneoplastic sensory demyelinating neuropathy w/ what Ab and what Dx

A

anti-MAG w/ Waldenstrom’s macroglobulinemia

56
Q

Anti CV2 causes what

A

sensory neuropathy or encephalomyelitis in thymoma or SCLC

57
Q

Anti Tr in what

A

paraneoplastic cerebellar degen w/ Hodkins

58
Q

anti Yo most commonly with what cancer

causes what

A

ovarian

causes cerebellar degen

59
Q

what drug works by binding to integrins on leukocytes and by blocking interaction of Ab w/ VCAMs to prevent migration across BBB

A

Natalizumab (Tysabri)