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Gabriel Schaab, Sara Ferri, Lekha Gopal; Atypical Presentations of Neuromyelitis Optica. Invest. Ophthalmol. Vis. Sci. 2011;52(14):3879.
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© ARVO (1962-2015); The Authors (2016-present)
To report two rare and atypical cases of neuromyelitis optica and illustrate the complexity of diagnosis as well as report long term results of various therapeutic options.
This is a retrospective review of two cases of patients diagnosed with neuromyelitis optica.
First case is a 21 year old Hispanic female with ocular history of recurrent attacks of bilateral alternating optic neuritis and residual visual deficit who presented with new onset left lower extremity weakness. Best corrected visual acuity was 20/30 OD and 20/800 OS. MRI brain/orbits showed increased signal intensity in the right optic nerve sheath without abnormal signal in the brain. MRI spine showed signal hyperintensity from C6 to conus and from T7 through T9. The patient underwent a 5 day course of IV steroids without improvement. The patient then underwent 7 cycles of plasmapheresis with improved lower extremity strength and visual acuity OS. At 5 year follow up her visual acuity was 20/30 OD and 20/250 OS. The second case is an atypical case of a 38 year old African American female with five day history of gradually worsening vision OU. Past ocular history was significant for a previous episode of declining vision OD nine monts prior. Best corrected visual acuity was count fingers at 1 foot OD and 20/80 OS. Optic nerve head examination showed temporal pallor OD. MRI brain/orbits on two separate occasions nine months apart showed bilateral optic nerve sheath enhancement with no enhancing lesions in brain parenchyma. MRI of spine did not show any enhancing lesions. However, NMO-IgG antigen specific assay was strongly positive. The patient received 5 day course of IV steroids and will undergo plasmapheresis in the future.
This case series discusses two rare and different presentations of neuromyelitis optica while showing visual and functional improvement can be achieved long term with plasmapheresis.
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