12 - Demyelinating Diseases, MS

Question 1

What is multiple sclerosis (MS)?

Answer 1

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

Question 2

MS relapse (overview)

Answer 2

Question 3

common optical defects in MS

Answer 3

no relation between top and bottom images

Question 4

MS relapse - duration

Answer 4

Question 5

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

Answer 5

Question 6

MS - mechanism

Answer 6

Question 7

MS - Disease course

Answer 7

Question 8

MS - clinical criteria

Answer 8

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

Question 9

McDonald Criteria

Answer 9

Question 10

MS - differential diagnosis

Answer 10

• 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

Question 11

MS - treatment

Answer 11

Question 12

MS - disease modifying therapy

Answer 12

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

3 things to consider: safety, convenience, and efficacy

Question 13

MS - Choice of therapy

Answer 13

Question 14

MS - injectables for treatment

Answer 14

Question 15

MS - oral treatments

Answer 15

Question 16

MS - infusion treatments

Answer 16

Question 17

How to decide aggressiveness of treatment?

Answer 17

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

Question 18

Family Planning in MS - Pregnancy
considerations

Answer 18

Question 19

MS - considerations before Conception

Answer 19

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

Question 20

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

Answer 20

Question 21

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - Aquaporin 4 antibody

Answer 21

Question 22

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

Answer 22

Question 23

Neuromyelitis Optica Spectrum
Disorders (NMOSD) - Pathology

Answer 23

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

Question 24

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

Answer 24

Question 25

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Core clinical characteristics

Answer 25

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

Question 26

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

Answer 26

Question 27

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Spinal Cord MRI

Answer 27

* 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

Question 28

Neuromyelitis Optica Spectrum Disorders (NMOSD) - Brainstem Imaging

Answer 28

* 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

Question 29

MS - Diencephalic and cerebral lesions

Answer 29