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A 26-year-old woman presented with a week of fatigue, unsteady gait, slowed speech, right-eye blurriness, and painful eye movement, along with two days of worsening left-sided numbness and weakness. Initial examination notable for right afferent pupillary defect, internuclear ophthalmoplegia, and left upper and lower facial weakness, dysarthria, left-sided weakness, and a T4 sensory level. Visual acuity was normal with subjective blurriness. Fundoscopic exam showed blurry margins of the right optic nerve. MRI brain revealed a “salt and pepper” radiophenotype with a curvilinear pattern of enhancement in the pons, concerning for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). On the next day, her condition worsened, with new urinary retention, excessive drowsiness, and worsening right-eye vision. MRI spine revealed multiple segments of longitudinal extensive transverse myelitis (LETM) including conus involvement. CSF showed lymphocytic predominant pleocytosis (216 WBC, 87% lymphocytes) and elevated protein 92. After ruling out CNS infection, patient was started on high dose steroids. At the end of her steroid course, vision, speech and mentation returned to normal. Due to persistent left-sided weakness, urinary retention, and unchanged sensory level, she was escalated to plasmapheresis. CSF myelin basic protein was elevated >128; oligoclonal bands were negative. Angiotensin-converting enzyme levels were normal; ANA screening and Aquaporin-4 antibodies were negative. Two weeks after admission, her Anti-myelin-oligodendrocyte glycoprotein (MOG) IgG antibodies was positive with titer >1:2560. At one-month follow up, symptoms had resolved apart from mild left-sided weakness.

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This case of subacute onset of neurologic deficits with "salt and pepper" radiophenotype found on brainstem MRI was suggestive of CLIPPERS. Further work-up revealed LETM and positive anti-MOG antibody titer, suggesting that MOGAD can present with a CLIPPERS radiophenotype and should be considered in the differential.