Cerebral Attacks in MOG Antibody-Associated Disease: Clinical Spectrum and Fulfillment of ADEM criteria
Sara Hooshmand1, Xiaoyang Li1, John Chen2, Vyanka Redenbaugh1, Samantha banks1, laura cacciaguerra1, Cristina Valencia Sanchez3, alfonso lopezchiriboga5, Elia Sechi6, Jan-Mendelt Tillema1, Sean Pittock4, Eoin Flanagan4
1Departments of Neurology and Center for MS and Autoimmune Neurology, 2Departments of Neurology and Center for MS and Autoimmune Neurology, Department of Ophthalmology, 3Department of Neurology, 4Departments of Neurology and Center for MS and Autoimmune Neurology and Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, 5Mayo Clinic, 6Department of Neurology, University of Sassari
Objective:
To characterize cerebral attacks of myelin oligodendrocyte-glycoprotein-antibody-associated disease (MOGAD) and assess for fulfillment of acute-disseminated encephalomyelitis (ADEM) criteria.
Background:
The clinical spectrum of MOGAD cerebral attacks has not been fully elucidated and it is unclear what proportion adhere to ADEM criteria.
Design/Methods:
We retrospectively searched our Mayo Clinic MOGAD database, and inclusion criteria were: 1) Symptomatic cerebral attack; and 2) Abnormal brain MRI; 3) MOG-IgG positivity. We assessed if included children and adults fulfilled current ADEM criteria of: A) First, polyfocal clinical central nervous system demyelinating event; B) encephalopathy (not explained by fever); C) no new clinical/MRI findings beyond 3 months; D) abnormal brain MRI (typically large, poorly-demarcated T2-hyperintensities involving white or deep gray matter, while T1-hypointense lesions are rare).
Results:
We included 90 patients. The median age was 23 (range, 2 -75), and 61% were female. In 22 (24%), recurrent cerebral attacks occurred. Clinical manifestations from the first cerebral attack included: headache, 51 (57%); brainstem symptoms, 41 (46%); encephalopathy, 34 (38%); cerebellar symptoms, 36 (40%), seizures, 17 (19%). In 16 patients (18%), the attack fell within the spectrum of cerebral cortical encephalitis. Concomitant myelitis was noted in 44 and 17 had concomitant optic neuritis. Acute MRI images were available for re-review in 81/90 and abnormalities included: poorly demarcated lesions, 55/81 (68%); deep gray matter lesions, 35/81 (43%); cerebral T1-hypointense lesions 46/81 (57%). In our cohort, 7 (8%) fulfilled ADEM criteria, and 83 (92%) did not due to ≥1 of: subsequent relapse, 58 (64%); absence of encephalopathy, 38 (42%); preceding demyelinating attack, 27 (30%); encephalopathy with fever, 15 (17%), and single lesion 4 (5%).
Conclusions:
Cerebral MOGAD attacks have a wide spectrum and usually do not fulfill ADEM criteria when retrospective applied. Future revisions of ADEM criteria might consider a disease-associated ADEM category to better capture MOGAD cerebral attacks.