Tumefictive MS (Video)

A 26-year-old woman with history of diabetes insipidus and hypothyroidism presented for evaluation of painless vision loss in her right eye. She was evaluated by a local neurologist and multiple sclerosis (MS) specialist who diagnosed acute optic neuritis. MRI brain showed an enhancing T2 hyperintense lesion of the body of the corpus callosum on the right. The pituitary and infundibulum were not enlarged or abnormally enhancing. A lumbar puncture demonstrated 8 WBC, 1 RBC, protein 29 mg/dL, glucose 84 mg/dL, IgG index 0.99 (normal < 0.70), and 5 CSF specific oligoclonal bands. CSF bacterial and fungal cultures, cytology, Lyme and VDRL were negative. She was diagnosed with tumefactive multiple sclerosis (MS) and was treated with 5 days of high dose oral corticosteroids and glatiramer acetate was initiated. She never recovered vision. She discontinued glatiramer acetate 6 months later because of site reactions. Fingolimod was recommended, but never initiated. Fourteen months after her initial presentation, she noted declining visual acuity OS. A neurologist diagnosed acute optic neuritis OS and started 5 days of high dose oral corticosteroids. She was referred to our clinic for further evaluation. Best-corrected visual acuity was 20/100 OD and 20/80 OS. There was a right APD and both optic discs were pale. The differential diagnosis included neoplasm and atypical inflammatory processes. Antiaquaporin 4 and anti-myelin oligodendrocyte glycoprotein antibodies were negative. Repeat MRI brain/orbits demonstrated a heterogeneously enhancing T2 hyperintense lesion of the subcortical right frontal lobe, involving the corpus callosum, hypothalamus, pituitary stalk and optic chiasm. CT of the chest, abdomen, and pelvis were negative. A diagnostic procedure was performed