Diagnostic delay in patients with myelin oligodendrocyte antibody-associated disease presenting with elevated intracranial pressure.

BACKGROUND

Elevated intracranial pressure (ICP) is a recently recognized complication of myelin oligodendrocyte antibody associated disease (MOGAD).

OBJECTIVE

We describe 5 patients with MOGAD presenting with elevated ICP.

METHODS

Serum MOG antibody testing completed at the University of Vermont Medical Center between January 1, 2017 to May 1, 2025 was reviewed. Patients with MOGAD were included. Elevated ICP was defined as opening pressure > 25 cm H2O, or opening pressure >20 cm H2O with an initial diagnosis of idiopathic intracranial hypertension (IIH).

RESULTS

Of 9 patients with MOGAD who had opening pressures measured, 5 patients (56%) had elevated ICP. Corticosteroid treatment was delayed due to higher clinical suspicion for alternate diagnoses, with idiopathic intracranial hypertension being the most common misdiagnosis. One patient underwent brain biopsy given concern for malignancy.

CONCLUSION

Red flags including rapidly progressive vision loss, lack of improvement on acetazolamide, CSF pleocytosis, and enhancement on neuroimaging may help differentiate MOGAD from IIH. Increased recognition of elevated ICP as a complication of MOGAD is needed to prevent potential morbidity associated with unnecessary diagnostic evaluation and treatment delay.