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.
LucasJ.An account of uncommon symptoms succeeding the measles; with some additional remarks on the infection of measles and small pox. Lond Med J1790; 11(Pt 4): 325–331.
2.
DaleRCde SousaCChongWK, et al. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain2000; 123 Pt 12: 2407–2422.
3.
SchwarzSMohrAKnauthM, et al. Acute disseminated encephalomyelitis: A follow-up study of 40 adult patients. Neurology2001; 56: 1313–1318.
4.
KruppLBBanwellBTenembaumS.Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology2007; 68: S7–S12.
5.
KruppLBTardieuMAmatoMP, et al. International pediatric multiple sclerosis study group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: Revisions to the 2007 definitions. Mult Scler2013; 19(10): 1261–1267.
6.
O’ConnorKCMcLaughlinKADe JagerPL, et al. Self-antigen tetramers discriminate between myelin autoantibodies to native or denatured protein. Nat Med2007; 13: 211–217.
7.
GaudiosoCMMarSCasperTC, et al. MOG and AQP4 antibodies among children with multiple sclerosis and controls. Ann Neurol2023; 93(2): 271–284.
8.
BruijstensALLechnerCFlet-BerliacL, et al. E.U. paediatric MOG consortium consensus: Part 1—classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders. Eur J Paediatr Neurol2020; 29: 2–13.
9.
Lopez-ChiribogaASMajedMFryerJ, et al. Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol2018; 75: 1355–1363.
10.
BanwellBBennettJLMarignierR, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD panel proposed criteria. Lancet Neurol2023; 22(3): 268–282.
11.
KoelmanDLChahinSMarSS, et al. Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study. Neurology2016; 86: 2085–2093.
12.
Valencia-SanchezCGuoYKreckeKN, et al. Cerebral cortical encephalitis in myelin oligodendrocyte glycoprotein antibody-associated disease. Ann Neurol2023; 93: 297–302.
13.
SechiEBuciucMPittockSJ, et al. Positive predictive value of myelin oligodendrocyte glycoprotein autoantibody testing. JAMA Neurol2021; 78: 741–746.
14.
RedenbaughVFryerJPCacciaguerraL, et al. Diagnostic utility of MOG antibody testing in cerebrospinal fluid. Ann Neurol2024; 96(1): 34–45.
15.
ReindlMSchandaKWoodhallM, et al. International multicenter examination of MOG antibody assays. Neurol Neuroimmunol Neuroinflamm2020; 7: e674.
16.
CartaSCobo CalvoÁArmanguéT, et al. Significance of myelin oligodendrocyte glycoprotein antibodies in CSF: A retrospective multicenter study. Neurology2023; 100: e1095–e1108.
17.
SechiEKreckeKNMessinaSA, et al. Comparison of MRI lesion evolution in different central nervous system demyelinating disorders. Neurology2021; 97: e1097–e1109.
18.
CacciaguerraLRedenbaughVChenJJ, et al. Timing and predictors of T2-lesion resolution in patients with myelin oligodendrocyte glycoprotein antibody-associated disease. Neurology2023; 101: e1376–e1381.
19.
GrausFTitulaerMJBaluR, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol2016; 15: 391–404.
20.
ArmangueTOlivé-CireraGMartínez-HernandezE, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: A multicentre observational study. Lancet Neurol2020; 19(3): 234–246.
21.
WendelEMThonkeHSBertoliniA, et al. Temporal dynamics of MOG antibodies in children with acquired demyelinating syndrome. Neurol Neuroimmunol Neuroinflamm2022; 9(6): e200035.
22.
HacohenYWongYYLechnerC, et al. Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease. JAMA Neurol2018; 75: 478–487.
23.
RamanathanSMohammadSTantsisE, et al. Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry2018; 89(2): 127–137.
24.
PetzoldAFraserCLAbeggM, et al. Diagnosis and classification of optic neuritis. Lancet Neurol2022; 21: 1120–1134.
25.
Transverse Myelitis Consortium Working G. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology2002; 59: 499–505.
26.
Correction to “Do early relapses predict the risk of long-term relapsing disease in an adult paediatric cohort with MOGAD?”Ann Neurol2024; 95: 623.
27.
SpatolaMPetit-PedrolMSimabukuroMM, et al. Investigations in GABA(A) receptor antibody-associated encephalitis. Neurology2017; 88: 1012–1020.
28.
ThompsonAJBanwellBLBarkhofF, et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol2018; 17: 162–173.
29.
WingerchukDMBanwellBBennettJL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology2015; 85: 177–189.
30.
LamprosADe BrouckerTBonnanM.Fever is a common onset feature of MOG-IgG associated disorders (MOGAD). Mult Scler Relat Disord2021; 49: 102748.
31.
MontalvoMKhattakJFRedenbaughV, et al. Acute symptomatic seizures secondary to myelin oligodendrocyte glycoprotein antibody-associated disease. Epilepsia2022; 63(12): 3180–3191.
32.
HamidSHMWhittamDSaviourM, et al. Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease. JAMA Neurol2018; 75: 65–71.
33.
CacciaguerraLAbdel-MannanOChampsasD, et al. Radiologic lag and brain MRI lesion dynamics during attacks in MOG antibody-associated disease. Neurology2024; 102: e209303.
