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Peds neuro boards, V2 > Demyelinating D/O > Flashcards

Demyelinating D/O Flashcards

1
Q

Pathogenesis of Myasthenia Gravis

A

Type V (II-B) hypersensitivity reaction:

Abnormal Myoid tissue originating from Thymus recognized as foreign by B/T cells

These antibodies bind to Achetylecholine receptors

(AchR modulating, blocking, binding, anti-MUSK, etc)

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2
Q

Myasthenia Gravis:
MUSK (+) versus MUSK (-) patients

A

Significantly more:

Bulbar Weakness

Respiratory crises

Electrodiagnostic findings may be localized

Resistant to treatment (Plasma Exchange best option)

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3
Q

Phases (3) and Pathogenesis (5) of MS

A

Phases

  1. inflammatory initiating event
  2. recovery
  3. chronic progression

Pathogenesis

  1. Immune cells activated in peripheral lymphoid tissue
  2. Egress of activated T(?B) cells into circulation
  3. Adherance to surface molecules on vascular endothelium
  4. entry through BBB
  5. Immune-mediated damage within CNS
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4
Q

Risk of patient of Optic Neuritis will Develop MS

A
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5
Q

Pathologic Subtypes of MS
Pattern I

Pattern II

Pattern III

Pattern IV

A
  1. Pattern I:
    1. T-Cell and macrophage infiltrates centered around vessels
    2. Oligodendrocytes = Spared
    3. NO compliment activation
  2. Pattern II (more common) :
    1. T-cells and macrophage infiltrates centered around vessels
    2. Oligodendrocytes = Spared
    3. YES compliment activation / immunoglobulin deposition
  3. Pattern III:
    1. Apoptosis of Oligodendrocytes
    2. Sparing of perivascular spaces
  4. Pattern IV:
    1. non-apoptotic oligodendrocyte death
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6
Q

Interferon Beta:

  1. Mechanism of Action
  2. Adverse effects
A
  1. Transcriptional regulator of >1000 genes, but exact effect on MS is unknown
  2. Adverse effects:
    1. Common:
      1. Skin reactions
      2. Fever
      3. Myalgia
      4. Headache
    2. Severe
      1. Liver toxicity
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7
Q

Glatiramer Acetate

  1. Proposed MoA’s (5)
  2. Adverse effects
A
  1. Proposed MoA
    1. Competitive binding with APCs
    2. Preferred bindng of GA-MHC complex compared to MBC-MHC complex to T-cells
    3. Induction of tolerance in T-cells
    4. Expression of neurotropic factors
    5. Stimulation of neurogenesis
  2. Adverse effects
    1. Injection site reaction
    2. Allergic Reaction
    3. flushing/palpitations (scary but transient)
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8
Q

Natalizumab

  1. MoA
  2. Adverse Effects
A

MoA

  1. alpha 4 integrin antagonist
  2. Possible suppresssion of existing inflammation within lesion
    1. inhibits microglial activation
    2. induces apoptosis of alpha4 integrin expression

Adverse Effects

  1. Fatigue
  2. Allergic reaction
  3. infusion reaction
  4. PML - especially if JC virus (+)
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9
Q

Risk for PML(flow-chart)

A
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10
Q

Fingolimod

  1. MoA
  2. Adverse Effects - not dose dependent (6)
  3. Adverse Effects - dose dependent (3)
A
  1. sphingosine 1-phosphate receptor modulator
    1. impedes egress of lymphocytes from lymph nodes (particularyl during recirculation)
  2. Adverse Effeccts
    1. Bradycardia (usually transient but possibly fatal)
    2. diarrhea
    3. back pain
    4. elevation of liver enzymes
    5. cough
    6. chest pain
  3. Adverse effects (at high doses)
    1. seizure
    2. headache
    3. lymphopenia
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11
Q

Teriflunomide:

  1. Mechanism of action
  2. Adverse Effects
A
  1. Inhibits pyrimidine synthesis (arrests proliferation of activated lymphocytes)
  2. Adverse effects
    1. Alopecia
    2. Elevated ALT
    3. Back pain
    4. nausea
    5. nasopharyngitis
    6. Leukocytosis (unclear significance)
    7. Pregancny category X
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12
Q

Dimethyl Fumarate

  1. Mechanism of Action
  2. Adverse Effects
A
  1. Unknown, proposed:
    1. Affects Krebs cycle
    2. upregulation of anti-inflammatory cytokines
    3. cytoprotective effect
  2. Adverse Effects
    1. Flushing
    2. GI upset
    3. Lymphopenia
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13
Q

Mitoxantrone

  1. MoA
  2. Adverse effects (7)
A
  1. Topoisomerase inhibitor (inhibits DNA synthesis)
    1. Only FDA approved med for primary progressive MS
  2. Adverse Effects
    1. Nausea
    2. Hair loss
    3. Hypotension
    4. Rashes
    5. Urinary tract infection
    6. Menstrual disorder
    7. CARDIOTOXICITY - requires close observation
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14
Q

Risk factors (5) and triggers (2) for multiple sclerosis

A

Risk factors

  1. Northern lattitude environmental exposure before age 15
  2. Vitamin D deficiency
  3. Caucasian population
  4. Females (2:1 M:F)
  5. family history of MS (5-10x)
    1. Identical twins = 25% chance

Triggers

  1. Viral exposures
    1. EBV (90-95% of MS patients are EBV positive)
    2. canine distemper virus
    3. faroe island
      2.
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15
Q

Genes that can increase your risk for MS (3)

A

HLA-DR2

IL-2R

IL7Ralpha

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16
Q

Test to order in Teenage girl with eye pain worsening with movement and lack of color vision

A

MRI with Fat-suppression of orbits (for Optic neuritis)

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17
Q

Spinal cord symptoms that should raise suspicion for MS (6+1)

A
  1. L’hermitte’s sign” (barber’s chair phenomenon)
  2. Numbness / sensory level
  3. “GBS” like presentation with genitourinary signs (incontinence, urgency, erectile dysfunction)
  4. progressive, asymmetric paraplegia
  5. Deafferentated hand
  6. partial myelopathy

if patient is numb from neckline to navel, it’s SC lesion until proven otherwise

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18
Q

Charactersistic MS lesions of spine

A

Small

love to hug up against periphery

love the dorsal column

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19
Q

MRI shows this lesion:

Risk of MS with:

No CNS lesions
1+ CNS lesion

A

No CNS lesions: 25%

1+ CNS lesions: 72%

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20
Q

Treatment options for clearly evident primary progressive MS

A

(trick question)

Nothing: AAN

  • interferon-beta and glatieramir do not prevent development of permanent disability n progressive forms of MS
  • These medicaiton sincrease costs and have frequent side effects that may adversely affect quality of life
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21
Q

MRI lesions suggestive of MS

(picture)

A
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22
Q

What are these suggestive of?

A

Multiple Sclerosis

23
Q

MRI buzzwords to suggest MS (5)

A
  • Corpus callosum lesion
  • Optic radiation lesion
  • Brainstem lesion abutting 4th ventricle
  • incomplete / partial enhancement
  • T1 “black holes”
24
Q

Known MS patient has Oligoclonal bands on CSF

what does this tell you?

A

Higher rate of relapse

25
Q

Diseases which can show oligoclonal bands other than MS

(10)

A
  1. Viral infections (HIV/SSPE)
  2. NMO
  3. SLE
  4. ADEM
  5. Vasculitis
  6. Adrenaleukodystrophy
  7. Syphillis
  8. Lyme
  9. CADASIL (+/-)
  10. Sarcoid (+/-)
26
Q

If Transverse myelitis patient fails to respond after course of high-dose steroids, what should you consider for acute treatment?

A

PLEX

“may be considered in pateints with TM who fail to improve after corticosteroids)

27
Q

Pre-treatment studies for Fingolimod

Surveilance studies for Fingolimod
(2)

Precautions for fingolimod
(2)

(5)

A

CBC (due to lymphopenia)
VZV titer (due to immunosuppression)
LFT (due to risk of transamitis)
EKG (due to 1st dose bradycardia / conduction block)
ophtho exam (due to risk of macular edema)

Ophthomalogic exam every 3-4 months (more if DM or uveitits)
LFTs

6 hour observation after first dose (for conduction block)
avoid live viruses (immunosuppression)

28
Q

Fingolimod

MoA (2)
Adverse Effects (6)
A

MoA

  1. Sphingosine-1-phosphate moderator
  2. Prevents egress of immune cells into circulation

Adverse Effects

  1. 1st dose bradycardia / sinus block
    1. CI in patients with someheart diseases
  2. Decreased WBC
  3. Macular Edema
  4. Decreased PFTs
  5. Increased LFT’s
  6. Teratogen
29
Q

Teriflunomide:

MoA (2)

Adverse Effects (4)

A

MoA

  1. Dihydroorotate dehydrogenase inhibitor
    1. Decreased immune cell replication

Adverse effects

  1. Immunosuppresssion (will need to ensure no TB)
  2. Hair loss
  3. hair loss
  4. Teratogen
30
Q

Dimethyl Fumarate

MoA
(2)

Adverse effects
(3)

A

MoA

  1. immunomodulator, induces apoptosis and reduces expression of IL-4, IL-2, and TNF-alpha
  2. May also decreased adhesion molecules

Adverese Effects

  1. GI
  2. flushing
  3. Headaces
31
Q

Mitoxantrone:

MoA

Adverse Effects
(2)

A

MoA

  1. Topoisomerase Inhibitor (inhibits DNA synthesis)

Adverese effects

  1. Systolic dysfunction
  2. Dose-dependent cardiotoxicity
    1. will need yearly cardiac function tests, even after finishing treatment
32
Q

Big adverse effects you should worry about for Natalizumab (1) and relative risk (2)

A

PML!

After 24 doses and (+) JC virus: 1:200

After 24 doses and (+) JC virus, and prior immunosppressive treatment: 1:100

33
Q

Pregnancy categories for MS drugs

  1. Glatiramer Acetate
  2. Interferons
  3. Fingolimod
  4. Natalizumab
  5. Mitoxantrone
  6. Teriflunomide
A
  1. Glatiramer: category B
  2. Interferons: Category C
  3. Fingolimod: Category C
  4. Natalizumab: Category C
  5. Mitoxantrone: Category D
  6. Teriflunamide: Category X
34
Q

Patient with MS with personality changes comes in with this MRI

What medication is she likely taking?

A

Natalizumab

(PML)

35
Q

Optic neuritis / Transverse Myelitis in MS versus NMO

How are they different?

(4)

A

Compared to MS, NMO attacks are:

  1. More intense
  2. have more radicular signs
  3. More readicular symptoms
  4. (transverse myelitis) more likely to show longitudinally extensive lesions
36
Q

Ab helpful in differentiating NMO from MS (1)

Sensitivity / specificity

How often is it present?
related prognostic note

A

Aquaporin-4 (anti-NMO)

Sensitivty: 73%
specificity: 90%

Present in:
38% with first-ever attack of Longitudinally extensive lesion
55% of these will have a relaps wof either TM or ON during the next year

37
Q

Diagnostic criteria for NMO

(5)

A
  1. Optic neuritis
  2. Transverse Myelitis
  3. 2 of the following 3
    1. MRI lesion not suggestive of MS
    2. Longitudinally extensive transverse myelitis (3+ spinal vertebral segments)
    3. (+) NMO serology
38
Q

Treatment for NMO

Acute (1+2)

Chronic (2)

A
  1. Steroids, Plasmapharesis if doesn’t respond
    1. Better response if:
      1. optic neuritis (opposed to TM)
      2. Onset of treatment <20 days from symptoms
  2. Chronic
    1. Azathioprine + steroids
    2. Rituximab
39
Q

ADEM

Typical population

Features (5)

A

Usually in young childrens / adolescences (peak age 3-10 years)

Features

  1. typically 7-14 days after infection (very rarely after vaccine)
  2. Headache / Vomiting
  3. Meningisumus
  4. Altered mental status
  5. multifocal neurologic signs
    1. ataxia
    2. tremors
    3. dysarthria
    4. hemiparesis, etc
  6. seizures (10-30%)
40
Q

Child comes in with altered metnal status and multifocal neurologic findings following illness.

This is his MRI

How do you treat?
what is his prognosis?

A

Acute disseminated encephalomyelitis (ADEM)

  1. Treatment
    1. Steroids (high-dose IV methylprednisolone) for 3-5 days
    2. afterwards oral prednisolone tapered over 4-6 weeks
  2. Prognosis
    1. long-term follow up is good
    2. many fully recover by 1-6 months
41
Q

Patient comes in with ataxia, interstitial keratitis, and hearing loss:

What is your highest item on differential?
what should you exclude (2)?
what should you look for (2)
how do you treat (2)?

A

likely diagnosis: Cogan syndrome

Rule out:
syphillis
Sarcoid

Look for:
vascular diseases
(vasculitis, especially aortic insufficiency)

Treatment:
keratitis: topical steroids / atropine
hearing loss: oral steroids

42
Q

Features of Neurosarcoidosis

Clinical (4)
CSF (1)
MRI (3)

A

Clinical

  1. multiple cranial nerves affected
    1. MC facial palsy (VII), occulomotor palsy (III, IV)
    2. hearing loss

CSF

  1. “asceptic meningitis” (elevated WBC and protein)

MRI

  1. give-away = non-caseating granulomas
  2. diffuse minengial enchancement
  3. possibly hydrocephalus
43
Q

man comes in with oral / genital ulcers and anterior uveitis

What is his most likely race?
What test should you order and what should it show?

A

Behcet disease

  1. Typically japanese ar mediterranean men
  2. MRI features
    1. restricted diffuseion in:
      1. Brainstem
      2. diencephalon
      3. basal ganglia
44
Q

Young adult comes in with “ADEM” like picture

What similarly presenting but potentially fatal condition should you rule out?
How?
If present, how do you treat it?

A

Acute hemorrhagic leukoencephalitis

  1. MRI Shows
    1. Asymemtric, large, edematous white matter lesion
    2. Punctate hemorrhages
  2. Treatment
    1. steroids
    2. PLEX
    3. IVIG
45
Q

Alemtuzumab

What does it target?

why is it not commonly used?

A

Targets CD52 cells

due to adverse effects:
mainly that it can produce secondary autoimmune disorders (currently approved only for those who failed 2+ disorders)

46
Q

Tremor most commonly seen in MS

A

Intention tremor

47
Q

Patient with MS drug presents with seizure

What med is likely to blame?
What is the mechanism of action?
What is it used for?
What could increase their risk?

A

Dalfampridine
increases walking speeds in MS patients
Thought to work by facilitating conduction of action potentials via potassium channel blockade
Increases with renal impairment.

48
Q

Risk of MS in patient’s with Clinically isolated syndrome (CIS)

A

30%

49
Q

Alemtuzumab

Mechanism of action

Adverse effects

A

MOA: monoclonal antibody to CD52

Adverse effects (Cancer, cancer, cancer, kidney, clotting, and…can you stop the infusion because there’s a reaction)

  1. Melanoma
  2. thyroid malignancies
  3. blood malignancies
  4. thrombocytopenia
  5. anti-GBM disease
  6. life-threatening infusion reactions
50
Q

Drug used to treat pendular nystagmus

A

Memantine

51
Q

MS drug to avoid in:

Depression
Heart block
Heart Failure
+ JC virus Ab
Beta Blockers
Liver disease
Pregnancy

A

Depression: Interferons
Heart block: Fingolimod
Heart Failure: Mitoxantrone
+ JCV: natalizumab
Beta Blocker: Fingolimod
Liver disease: Fingolimod, Interferon, teriflunomide
Pregnancy: everything but Copaxone,

52
Q

Patient presents with PML

Test to suggest related to MS therapy

A

MRI is of cerebellar peduncles is enhancing (in about 50% of patients)

53
Q

Dissemination-in-space criteria for MS (4)

A
  1. Periventricular
  2. juxtacortical/cortical
  3. infratentorial
  4. Spinal cord

Need 2 of 4

54
Q

Antibody associated with miller-fisher varient of GBS

A

GQ1b

Peds neuro boards, V2 (29 decks)

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