Tisavipat, Nanthaya

Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating syndrome observed in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) but there are limited data on application of this definition to its cerebral attacks or in adults with this disease. Methods: We analyzed patients from the Mayo Clinic MOGAD cohort ( n = 263) with cerebral attacks. We assessed whether children (<18 years-old) and adults (⩾18 years-old) fulfilled the 2013 ADEM definition. Results: We identified 89 patients (children, 34; adults, 55) and 55/89 (62%) were of female sex. Acute magnetic resonance imaging (MRI) abnormalities included: poorly demarcated T2 lesions (white matter, 85 (96%); deep gray matter, 41 (46%); T1-hypointense lesions (42 (47%)). Forty-two patients did not fulfill the ADEM definition due to: monofocal syndrome, 9; or no encephalopathy, 33. The remaining 47 patients (53%: children, 27/34 (79%); adults, 20/55 (36%)) fulfilled the ADEM definition, and 14 had preceding attacks, and 34 had subsequent attacks relative to ADEM episode. The features in children and adults with ADEM were broadly comparable. Conclusion: Applying the ADEM definition to MOGAD cerebral attacks highlighted that an update of the 2013 ADEM definition would be beneficial and could explicitly state its application to both adults and children and allow it to be applied to attacks at any time during the disease course.

Increased intracranial pressure (ICP) is increasingly recognized in myelin oligodendrocyte glycoprotein antibody-associated disease but has not been systematically explored in other demyelinating conditions. This cross-sectional study examines the features of raised ICP in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). Lumbar puncture opening pressure (OP) done within 30 days of an attack was identified in 41 NMOSD patients and 37 MS patients. Raised OP was present in 7 (17.1%) NMOSD patients and 4 (10.8%) MS patients who were largely asymptomatic, partially explained by obesity, and did not require ICP lowering medications beyond acute treatment with corticosteroids.