Frequency of Increased Intracranial Pressure in MOGAD
Nanthaya Tisavipat1, Natthapon Rattanathamsakul2, Abdul-Rahman Salman3, Vyanka Redenbaugh1, Laura Cacciaguerra1, Deena Tajfirouz1, Kevin Chodnicki2, Jan-Mendelt Tillema1, Sean Pittock1, Eoin Flanagan1, John Chen2
1Neurology, 2Ophthalmology, Mayo Clinic, 3George Washington University School of Medicine and Health Sciences
Objective:

To evaluate the frequency of increased intracranial pressure (ICP) in a large cohort of MOGAD.

Background:
There are a few case reports of bilateral optic disc edema and raised opening pressure (OP) on lumbar puncture (LP) in myelin-oligodendrocyte-glycoprotein-antibody-associated-disease (MOGAD), which could cause diagnostic confusion between MOGAD with bilateral optic neuritis (ON) and pseudotumor cerebri.
Design/Methods:

Cross-sectional study of MOGAD patients at a tertiary institution with LP OP within 30 days of attack onset.

Results:

From 321 MOGAD patients, 109 LPs were included from 104 patients. The frequency of increased ICP (OP >250 mmH2O in adults or >280 mmH2O in children) was 26% (28/109) in MOGAD attacks (25% [26/104] in MOGAD patients). Patients with increased ICP were slightly younger (median age at LP 23 [IQR 11-40] vs. 35 [IQR 17-49], p=0.050), more often male (58% vs. 35%, p=0.038) and obese (54% vs. 32%, p=0.043). The median OP was 350 [IQR 296-400] mmH2O during increased-ICP attacks and 172 [IQR 140-210] during normal-ICP attacks (p<0.001). The median CSF white cell count was 33 (IQR 5-134) cells/mm3 in increased-ICP attacks vs. 8 (IQR 2-43) in normal-ICP attacks (p=0.022), with CSF pleocytosis (>5 cells/mm3) in 75% vs. 54% (p=0.063). CSF protein was comparable. Increased ICP was most commonly observed during acute disseminated encephalomyelitis (ADEM: 44%; 10/23), cerebral cortical encephalitis (67%; 2/3), and ON with myelitis attacks (33%; 3/9). Among the 28 increased-ICP attacks, 32% (9/21) had bilateral disc edema (2 papilledema from increased ICP alone; 7 bilateral ON), while 33% (7/21) had no disc edema, and 64% (18/27) had headache. Three (11%) attacks initially treated as pseudotumor cerebri had bilateral ON and CSF pleocytosis.

Conclusions:
Increased ICP can be observed in a quarter of MOGAD attacks, particularly ADEM and cerebral cortical encephalitis. The cause is uncertain but potential etiologies include MOGAD inflammation, coinciding pseudotumor cerebri, and spuriously elevated OP.
10.1212/WNL.0000000000206730