Lab work and a lumbar puncture were performed for various infectious and inflammatory causes of atypical optic neuritis
MRI of the cervical and thoracic spine to detect any myelitis given the high suspicion for NMO
Lab Testing Results
RPR and FTA-ABS-ve
Antinuclear antibody (ANA)-ve
SS-A, SS-B Ab-ve
Glucose, 106mg/ml (mildly elevated)
Protein, 22mg/ml (normal)
Two nucleated cells (equivocal)
No growth on culture
MRI C-spine w/wo contrast
No intrinsic cord signal abnormality or abnormal post contrast enhancement
MRI T-spine w/wo contrast
No evidence of abnormal signal
Neuromyelitis Optica (NMO) spectrum disorder
NMO (Devic’s disease) is an autoimmune disease in which auto-antibodies attack myelin. Specifically IgG antibodies against aquaporin 4 protein channels present in the cell membrane of astrocytes has been implicated in the pathophysiology of NMO.
Aquaporin 4 channels are found on astrocytes in the blood brain barrier. Therefore, auto-antibodies in NMO are believed to lead to a breakdown in the blood brain barrier.
A second subset of patients is NMO IgG negative for aquaporin 4. Some of these patients may test positive for another antibody, anti-MOG, which is thought to primarily attack myelin followed by degeneration of axons and astrocytes.
The patient was treated with five days of IV 1G Solu-Medrol
Given the suspicion of an aggressive inflammatory optic neuropathy as the patient presented with acute NLP vision, plasmapheresis was started at the same time as the IV Solu-Medrol. The patient received five doses of plasmapheresis (2on-1off-2on-1off-1on schedule).
She was then transitioned to 60mg PO prednisone for two weeks
Prednisone was tapered to 40mg PO prednisone for the subsequent two weeks
Prognosis and Future Directions
The prognosis for typical demyelinating optic neuritis is very good, with more than 90 percent of patients having improved visual acuity in the first two weeks. Patients presenting typically have an excellent prognosis (~95 percent have vision at six months better than 20/30).
At 15 years, approximately 50 percent of these patients will have developed MS, and 75 percent will have developed MS if they demonstrated at least one typical demyelinating lesion on MRI at presentation.
IV steroids may be associated with more rapid improvement in vision, but no change in final visual prognosis or long-term protection in the development of MS.
If a patient presents with the following typical features, further workup beyond MRI brain and orbits is not recommended (i.e. Lumbar puncture, extensive serological testing)
Ages 18 to 50 years old
Pain with eye movements
No to minimal intraocular inflammation
No hemorrhages or exudates in posterior pole
Demonstrate improvement in vision several weeks after onset
Based on Optic Neuritis Treatment Trial findings, an optic neuritis is considered “atypical” for the classical MS-associated form when any of the above features are absent, precipitating further workup.
For NMO-associated optic neuritis, the prognosis is less favorable than typical demyelinating optic neuritis. Approximately 85 percent of patients have relapses of either optic neuritis or transverse myelitis.
In patients classified as NMO spectrum disease (i.e. either optic neuritis or transverse myelitis but do not meet the full diagnostic criteria for NMO), prognosis has been shown to vary depending on whether patients are anti-AQP4-Ab positive or anti-MOG-Ab (myelin-oligodendrocyte glycoprotein antibody) positive. Patients who are anti-AQP4 are more likely to have optic neuritis that progresses to significant optic atrophy with worse visual prognosis compared to anti-MOG-Ab. Patients who are anti-MOG-Ab are more likely to present with bilateral optic neuritis, respond better to treatment with steroids and are less likely to have extensive optic atrophy.
While azathioprine and rituximab are commonly used immunosuppressive therapies in patients with NMO, there are no randomized controlled trials comparing the effectiveness of various immunosuppressive therapies. Future randomized controlled trials may help guide immunosuppressive treatment in patients with NMO or NMO spectrum disease.
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Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Director, Neuro-Ophthalmology Service, firstname.lastname@example.org