Increased Intracranial Pressure in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease

Abstract

Background and ObjectivesElevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP.MethodsIn this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm H2O. We also present a case series of patients with or without OP measurement who required medical and/or surgical management of increased ICP.ResultsOf 86 children with MOGAD, 43 (50.0%) had an OP recorded and 7 (8.1%) required ICP management. In those with OP recorded, the median (interquartile range) OP for the different MOGAD phenotypes were: 30.0 (22.8–41.6) (acute disseminated encephalomyelitis, ADEM), 20.5 (16.1–23.6) (optic neuritis), 17.0 (17.0–22.5) (myelitis), and 19.5 (16.5–29.3) (other) cm H20. Overall, 20.9% had increased ICP based on an OP > 28 cm H2O, of whom 77.8% presented with ADEM. In a subgroup analysis of those presenting with ADEM, those with an elevated ICP had longer hospital stay (p= 0.007) and neurologic disability (defined as modified Rankin Scale >1) (p= 0.049). In those with or without OP recorded, 7 (6 with ADEM, one with cerebral cortical encephalitis) required ICP-directed therapies. Findings on brain MRI in these 7 children revealed extensive disease burden with bilateral cerebral involvement and evidence of restricted diffusion. While neuropsychological data in this small subset revealed significant variability, all sustained identifiable deficits after discharge, including attention-deficit hyperactivity disorders and language and learning disorders.DiscussionIn pediatric MOGAD, increased OP and ADEM at initial presentation were associated with longer hospital stays and greater long-term morbidity. Although invasive ICP monitoring has not been specifically advocated in the management of MOGAD, it is important to recognize signs and symptoms of increased ICP in these patients and consider ICP monitoring and management strategies based on clinical and radiologic findings, especially in those presenting with ADEM and with OP > 28 cm H2O.

Pediatric Autoimmune Neurologic Disorders

“…Rarely, MOGAD attacks in children may be severe enough to require mechanical ventilation from an inability to protect the airway in the setting of severe encephalopathy or status epilepticus 51 . In some MOGAD attacks, increased intracranial pressure has been reported, and in one study this occurred in 9 of 43 pediatric patients (7 requiring treatment for raised intracranial pressure) and was associated with worse outcomes with longer hospital stays and higher modified Rankin scale scores at last follow-up 52 . There are case reports of fulminant presentations that required emergent CSF diversion or hemicraniectomy, and some children have died from massive cerebral edema and brain herniation 53-56 .…”

“…51 In some MOGAD attacks, increased intracranial pressure has been reported, and in one study this occurred in 9 of 43 pediatric patients (7 requiring treatment for raised intracranial pressure) and was associated with worse outcomes with longer hospital stays and higher modified Rankin scale scores at last follow-up. 52 There are case reports of fulminant presentations that required emergent CSF diversion or hemicraniectomy, and some children have died from massive cerebral edema and brain herniation. [53][54][55][56] This risk highlights the importance of emergency management with severe MOGAD attacks, including monitoring the airway, assessing for increased intracranial pressure, and managing these complications accordingly.…”