12/18/2019 1 Autoimmune Diseases of the CNS: Optic Neuritis and Myelitis Michael J. Bradshaw, MD Rosalind Franklin University of Medicine and Science Billings Clinic Neurology Disclosures/Off-Label Use No financial conflicts of interest Off-label treatment of neuromyelitis optica spectrum disorders is discussed Goals Recognize and understand diagnostic evaluation of two common presentations of autoimmune CNS disease: Optic Neuritis Myelitis Review the neuromyelitis optica spectrum disorders Case 1 46 year old woman with no medical history reports two days progressive vision loss in the left eye. Her vision started out “cloudy” and has been getting progressively worse. She noticed that while driving, it looked like cars were swerving toward her. She also has mild retro-orbital discomfort. Optic Neuritis: Typical Symptoms Subacute, progressive monocular vision loss Decreased visual acuity Dyschromatopsia Variably accompanied by retro-orbital pain Mayoclinic.org Optic Neuritis: Unusual Symptoms Phosphenes: light flashes with eye mvmt Photisms: light induced by noise, taste or touch Pulfrich effect: false perception of depth in lateral motion “What is out of the common is usually a guide rather than a hindrance.” 1 2 3 4 5 6
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12/18/2019
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Autoimmune Diseases of the CNS: Optic Neuritis and Myelitis Michael J. Bradshaw, MD
Rosalind Franklin University of Medicine and Science
Billings Clinic Neurology
Disclosures/Off-Label Use
No financial conflicts of interest
Off-label treatment of neuromyelitis optica spectrum disorders is discussed
Goals
Recognize and understand diagnostic evaluation of two common presentations of autoimmune CNS disease:
Optic Neuritis
Myelitis
Review the neuromyelitis optica spectrum disorders
Case 1
46 year old woman with no medical history reports two days progressive vision loss in the left eye. Her vision started out “cloudy” and has been getting progressively worse. She noticed that while driving, it looked like cars were swerving toward her. She also has mild retro-orbital discomfort.
Optic Neuritis: Typical Symptoms
Subacute, progressive monocular vision loss
Decreased visual acuity
Dyschromatopsia
Variably accompanied by retro-orbital pain
Mayoclinic.org
Optic Neuritis: Unusual Symptoms
Phosphenes: light flashes with eye mvmt
Photisms: light induced by noise, taste or touch
Pulfrich effect: false perception of depth in lateral motion
“What is out of the common is usually
a guide rather than a hindrance.”
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5 6
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Pulfrich Effect
https://www.youtube.com/watch?v=1mnWI_u_zBg
Watch with dark sunglasses blocking the left eye only and the image will appear three-dimensional due to the Pulfricheffect
Patient’s view
Neurology July 19, 2016, 87:3 338-339.
Localization: surface of the eye to the optic chiasm (on the left)
Baehr M, Frotscher M. Duus’ topical diagnosis in neurology. Thieme.
Mayoclinic.org
Case 1
Physical exam: visual acuity 20/80 OS, 20/20 OD, left eye red desaturation, and a
relative afferent pupillary defect in the left eye. No disc edema.
aapos.org
Optic Nerve Lesion:Physical exam
Decreased visual acuity (especially to low contrast chart)
Color desaturation
Relative afferent pupillary defect
May not have optic disc edema
Lancet Neurol 2014; 13: 83–99.
Relative Afferent Pupillary Defect
https://www.youtube.com/watch?v=soiKbngQxgw
Normal: 1:20 min
Right eye relative afferent pupillary defect: 2:10 min
2006;67:968–72. Mult Scler. 2011;17:312–8. Neurology. 2008; 70:1079–83.
Case 2
A three year old girl is brought to the emergency department for vision changes.
Four days ago, she reported “seeing water” on the floor, then over the next few days seemed to have difficulty seeing objects, such as a toy held out to her. Today, she started walking into walls, saying she couldn’t see them.
Physical exam: no central vision in either eye (light perception only), but some preserved peripheral vision. There is no relative afferent pupillary defect. Left Babinski.
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Optic Neuritis: RED FLAG Features
Severe visual deficits
Bilateral optic neuritis
Involvement of the chiasm on MRI
Longitudinally extensive optic neuritis (>1/2 length of the ON)
Concerning for neuromyelitis optica spectrum disorders
Poor recovery from attacks; emergent
treatment indicated
CSF: 0 WBC, normal glucose, normal protein, no oligoclonal bands, elevated IgG index
Treated with emergent IVMP and PLEX- vision fully recovered
AQP4 is a water channel heavily expressed on optic nerves, brainstem, spinal cord
Aquaporin-4 IgG are pathogenic
Fix complement: kills the astrocyte Neuronal death
J Exp Med 2005;202(4):473-477. Neurology. 2013 Oct;81(14):1197-204.
NMOSD Acute Treatment
All disability accumulates during relapses
Relapses should be treated aggressively
IVMP and early plasma exchange
Area postrema syndrome generally responds to steroids alone
NMOSD Maintenance Treatment
Eculizumab (C5 complement inhibitor; FDA approved)
Rituximab (CD20 Ab)
Mycophenolate mofetil, azathioprine
Clinical trials:
Tocilizumab/satralizumab (IL-6R antagonist)
Inebilizumab (CD19 Ab)
Case 427 yoF presents to her PCP after she developed paresthesias in the hands and feet over the course of a few days. Several days later, she noted sensory loss/paresthesias from the nipple line down.
She had an episode of optic neuritis 12 years ago.
Next step?
Case 2: Next steps?
Ascending sensory
pathways
Descending motor
pathways
Fine touch, vibration, proprioception
(ipsilateral)ARM LEG
Motor control
extremities(ispilateral)
Motor control
trunk (bilateral)
Crude touch,
temperature, pinprick(contralateral)
VENTRAL
DORSAL
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Myelopathy
Spinal cord dysfunction from any cause
Compression, immune-mediated,
infectious, spinal cord infarct,
toxic/metabolic
Myelitis is spinal cord dysfunction with evidence of inflammation
MRI or CSF
A Systematic Approach to MyelopathyClinical evidence of spinal cord