Objective:

To describe demographic, clinical, radiologic features and outcomes of pediatric MOGAD presenting with elevated ICP.

Background:

Intracranial hypertension is increasingly recognized in a subset of pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD). Presentations range from mild (idiopathic intracranial hypertension) IIH-like cases to severe cases requiring ICU management.

Design/Methods:

We retrospectively analyzed children ≤18 years of age at our center who met consensus criteria for MOGAD and were found to have elevated opening pressure or signs of increased ICP without a better explanation. Clinical characteristics, CSF findings, neuroimaging features, treatment course, and outcomes were evaluated.

Results:

Fourteen patients met inclusion criteria (9 female). Median age at presentation was 9 years (range 6 – 14 years). Median BMI percentile was 95.1%. Seven patients (50%) had preceding viral symptoms at a median of 1.4 weeks prior to presentation. All patients presented with headache. Six (42.9%) also had nausea/vomiting and seven (50%) had visual changes. Ten patients had elevated opening pressure (OP) documented at the time of lumbar puncture (LP); for those without recorded OP, 1 had tonsillar herniation and 3 had documented optic disc edema. Median opening pressure was 36 cm H₂O. Idiopathic intracranial hypertension (IIH) and migraine were common early diagnoses. There was a median delay of 17 days from headache-onset to corticosteroids. Initial serum MOG titer was positive in all patients (median 1:130). All patients were treated with steroids, 64.2% with Diamox, 50% with IVIG and 28.6% with plasmapheresis. Diamox was continued for average of 2.2 months. Headaches remained at follow-up for a median of 4.9 months and completely resolved in 71% of cases.

Conclusions:

MOGAD may initially present with unexplained intracranial hypertension and in these cases, is often misdiagnosed initially. With appropriate treatment and time, most headaches resolved by 6 months.