12 - Demyelinating Diseases, MS Flashcards

1
Q

What is multiple sclerosis (MS)?

A
  • Inflammatory
  • Demyelinating
  • Symptoms depend on area affected
  • Relapsing-remitting or Progressive
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2
Q

MS relapse (overview)

A
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3
Q

common optical defects in MS

A

no relation between top and bottom images

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4
Q

MS relapse - duration

A
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5
Q

MS findings on imaging -
Periventricular, juxtacortical, cortical, infratentorial or spinal cord - with or without enhancement
*central vessel sign
*spinal cord lesions (C or T)

A
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6
Q

MS - mechanism

A
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7
Q

MS - Disease course

A
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8
Q

MS - clinical criteria

A

Paraclinical evidence:
the hot bath test, evoked response studies, tissue
imaging procedures, and reliable, expert urological assessment

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9
Q

McDonald Criteria

A
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10
Q

MS - differential diagnosis

A
  • Migraine with aura
  • Inflammatory disorders:
    –Neuromyelitis optica spectrum disorder (NMOSD)
    –Sarcoid
    –Lupus
    –Sjogren’s
    –Medication related
    →TNF inhibitors (Infliximab, Adalimumab, Etanercept)
  • Infectious:
    –Syphilis
    –HIV
    –Tuberculosis
    –VZV; CMV; HTLV-1 (transverse myelitis)
    –Progressive multifocal leukoencephalopathy
  • Neoplastic
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11
Q

MS - treatment

A
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12
Q

MS - disease modifying therapy

A
  • Decrease relapse rate
  • Slow accumulation of brain lesions
  • Main goal: decrease disability

3 things to consider: safety, convenience, and efficacy

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13
Q

MS - Choice of therapy

A
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14
Q

MS - injectables for treatment

A
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15
Q

MS - oral treatments

A
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16
Q

MS - infusion treatments

A
17
Q

How to decide aggressiveness of treatment?

A
  • Poor prognosis associated with:
    – Location of lesions (brainstem and spinal cord)
    – Lesion burden
    – Disease activity
    – Gender – male
    – Black ethnicity
18
Q

Family Planning in MS - Pregnancy
considerations

A
19
Q

MS - considerations before Conception

A
  • DMT discontinuation - risk of rebound
  • Natalizumab and S1Ps
  • DMT initiation
  • Goal of continuing through pregnancy versus disease
    stability then stopping prior to conception?
20
Q

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - What, where and how?

A
21
Q

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - Aquaporin 4 antibody

A
22
Q

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - Aquaporin 4 antibody pathway (diagram)

A
23
Q

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - Pathology

A
  • Astrocytopathy → Necrosis and infiltration with neutrophils and eosinophils
  • AQP4 immunoreactivity
  • Perivascular complement activation
  • NO RECOMMENDATION FOR BIOPSY
24
Q

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - diagnostic criteria for
adult patients

A
25
Q

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Core clinical characteristics

A
  1. Optic neuritis
  2. Acute myelitis
  3. Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting
  4. Acute brainstem syndrome
  5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions
  6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions
26
Q

Additional MRI requirements for Neuromyelitis Optica Spectrum Disorders (NMOSD) without AQP4-IgG and NMOSD with unknown AQP4-IgG status

A
27
Q

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Spinal Cord MRI

A
  • Increased T2 signal extending over 3 or more complete vertebral segments
  • Central cord predominance
  • Gad enhancement
  • Cervical Rostral extension of the lesion into the brainstem
  • Cord expansion/swelling
  • Decreased signal on T1-weighted sequences corresponding to region of increased T2-weighted signal
  • Atrophy when chronic
28
Q

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Brainstem Imaging

A
  • Dorsal medulla (especially the area postrema) -
    contiguous with an upper cervical spinal cord lesion
  • Periependymal surfaces of the fourth ventricle
  • Lesions extending to the hypothalamus, thalamus, or periependymal surfaces of the third ventricle
29
Q

MS - Diencephalic and cerebral lesions

A