Optic neuritis (45.8%), transverse myelitis (22.8%), and brainstem encephalitis (17.1%) are more common presentations in adults, whereas acute disseminated encephalomyelitis is common in children.2A few patients with unusual presentations have been reported to have MOG-IgG positivity, including encephalitis, leukodystrophy, posterior reversible encephalopathy syndrome, multiple sclerosis, meningoencephalitis, and aseptic meningitis.3-7 In this study, we report a case of a MOG-IgG-positive patient who presented with IH. report presents the first description of a relapsing remitting course presenting each time with only symptoms of raised intracranial pressure, without developing any typical clinical manifestations of MOGAD. Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein found in the myelin sheaths of neurons in the CNS. MOG is an important target for a spectrum of autoimmune disorders, particularly optic neuritis and transverse myelitis. In this study, we report an unusual manifestation of MOG antibody-associated disease (MOGAD) in a young woman who presented with isolated intracranial hypertension (IH). == Case History == A 19-year-old woman developed a severe headache with acute-onset and occasional transient visual obscurations persisting for 1 week. She was evaluated at a local hospital and found to have bilateral papilledema on fundoscopy. The patient had no other neurologic symptoms. MRI of the brain with contrast venogram showed normal results. The results of CSF analysis were normal, but the opening pressure was elevated to 25 cm H2O Pseudoginsenoside-F11 (normal range: 620 cm H2O). The patient was subsequently diagnosed with IH. The patient responded to oral acetazolamide at 750 mg daily for 1 month. After 3 months, the symptoms recurred, with acute-onset severe headache, vomiting, and intermittent blurring of vision, which was greater in the left eye than in the right. The patient was then referred to a tertiary specialist. A detailed history was obtained, and she did not have fever, seizures, or altered sensorium. Fundoscopy revealed persistent bilateral disc edema. Focal neurologic deficits were not observed. The visual field test results and acuity were normal. Visual evoked potentials (VEPs) were normal. Another brain MRI revealed mild meningeal enhancement in the left parietal region on contrast sequences. Minimal hyperintensity without any diffusion restriction or white matter lesions was further observed in the underlying cortex on fluid-attenuated inversion recovery (FLAIR).Figure 1Ashows a postcontrast T1-weighted axial image indicating abnormal unilateral leptomeningeal enhancement along the left temporoparietal cortical sulci, andFigure 1Bshows the minimal hyperintensity of the adjoining cortex on an axial FLAIR image. A repeat CSF tap showed lymphocytic pleocytosis (20 cells) and normal glucose and protein levels. The opening pressure was 35 cm H2O. == Figure 1. MRI Brain FLAIR and Contrast. == (A) Abnormal leptomeningeal enhancement along the left temporoparietal cortical sulci. (B) Fluid-attenuated inversion recovery (FLAIR) image showing minimal hyperintensity of the adjoining cortex. Owing to the presence of minimal cortical FLAIR hyperintensity and leptomeningeal enhancement in the brain MRI associated with CSF pleocytosis, further evaluation was performed. Serum and CSF MOG immunoglobulin (Ig) G antibodies were tested with a cell-based immunoassay using transfected cell lines for qualitative or semiquantitative in vitro determination of human IgG antibodies to the aquaporin-4 receptor and anti-MOG positive in serum and CSF (1:10 dilutions).1Other workup results for vasculitis and infective and autoimmune encephalitis were negative (including antinuclear antibodies, antineutrophil cytoplasmic antibodies, anticardiolipin antibodies, and antibodies to aquaporin-4). The patient’s serum angiotensin-converting enzyme level was normal at 26 nmol/mL/min (normal value, 40 nmol/mL/min). PET imaging of the entire body, including the brain, revealed normal results. The patient was diagnosed with MOGAD. Her headache subsided, and the papilledema was partially resolved with IV methylprednisolone at 1 g/d for 3 days, followed by oral steroids. However, after initial improvement, her symptoms reappeared while steroids were being tapered. Mycophenolate mofetil (MMF) was added to the treatment, but despite this, she experienced 4 relapses in 6 months. All relapses presented with headache and signs of increased intracranial pressure (ICP) with papilledema when steroids were tapered, except at the fourth follow-up. Each episode improved after a CSF tap. The serial CSF reports are presented inTable. == Table. == Results of Serial CSF and Laboratory Studies In view of the relapses with headache despite treatment with MMF and steroids, IV rituximab was initiated, with positive results. Her symptoms, such as headache and papilledema, subsided, and steroids and MMF were tapered. Papilledema resolved over a period Bgn of 2 months. The patient remained asymptomatic even after 1 year (Figure 2). == Figure 2. Pseudoginsenoside-F11 Flowchart of the Clinical Course of Patient Treatment. == Pseudoginsenoside-F11 MMF = mycophenolate mofetil; MOG = myelin oligodendrocyte glycoprotein. == Discussion == Antibodies against MOGs (MOG-IgG) cause a wide spectrum of inflammatory demyelinating CNS conditions. Optic neuritis (45.8%), transverse myelitis (22.8%), and brainstem encephalitis (17.1%) are more common presentations in adults, whereas acute disseminated encephalomyelitis is common in children.2A few patients with unusual presentations have been reported to have MOG-IgG positivity, including encephalitis,.