Neuromyelitis Optica-Associated Acute Intermediate Uveitis

Abstract

Optic neuritis is one of the diagnostic criteria for Neuromyelitis Optica Spectrum Disorder (NMOSD). However, the presentation of other ophthalmic manifestations in a patient with NMOSD is less common. A case of intermediate uveitis in a patient with anti-aquaporin-4 antibody (AQP4) positivity is detailed in this report. We present here a case of a 17-year-old Southeast Asian woman who presented with segmental fullness of the optic disc margin, small hypopigmented lesion along inferior temporal arcade, trace vitreous cells and inferior snowballs consistent with unilateral intermediate uveitis. The patient subsequently presented with an episode of optic neuritis of the same eye, resulting in diagnostic workup conforming NMOSD including neuroimaging and AQP4 antibody. Our case report highlights that NMOSD should be considered in the differential for intermediate uveitis. Furthermore, the sequence of both intermediate uveitis and optic neuritis in our patient demonstrates the importance of continued ophthalmic exams in patients with NMOSD.

Keywords: aquaporin-4 antibody; intermediate uveitis; neuromyelitis optica spectrum disorder; optic neuritis.

Figure 1

Fundus imaging of right eye. Fundus photos of right eye with hypopigmented lesion along inferior temporal arcade (A) and inferior snowballs (black arrows) (B).

Figure 2

Fundus autofluorescence imaging of right eye. Fundus autofluorescence of right eye re-demonstrating the hypopigmented lesion (white arrow) with hypo-autofluorescence.

Figure 3

Neuroimaging with evidence of NMOSD. Fat suppressed contrast-enhanced T1 weighted magnetic resonance imaging (MRI) of brain and orbits demonstrated right optic nerve enhancement (A) and white matter lesions (white arrows) in the right pericallosal region, left posterior frontal lobe and juxtacortical region on FLAIR sequences (B).

Figure 4

Fundus imaging of right eye. Fundus photos of right eye with resolving hypopigmented lesion along inferior temporal arcade without macular or optic nerve edema.