2023NEMOS建议：视神经脊髓炎谱系疾病的诊断与治疗（更新版）—第1部分：诊断及鉴别诊断

Journal of Neurologyhttp5/doi.org/10.1007/s00415-023-11634-0REVIEWUpdate on the diagnosis and treatment of neuromyelits opticaspectrum disorders(NMOSD)-revised recommendationsof the Neuromyelitis Optica Study Group(NEMOS).Part l:Diagnosisand differential diagnosisSven Jarius'.Orhan Aktas2.llya Ayzenberg3.Judith Bellmann-Strob,5.6.17.Achim Berthele7.Katrin Giglhuber7.Vivien HauBlerJoachim Havla.Kerstin Hellwig3.Martin W.Hummert.Ingo Kleiter32.Luisa Klotz3.Markus Krumbholz,15,16.Tania KumpfelFriedemann Pau5617.Marius Ringelstein218.Klemens Ruprecht.Makbule Senel19.Jan-Patrick Stellmann,2021.Florian Then Bergh22.Hayrettin Tumani19.Brigitte Wildemann1.Corinna Trebst11.Neuromyelitis Optica Study Group(NEMOS)Received:21 October 2022/Revised:17 February 2023/Accepted:18 February 2023©The Author(s)2023AbstractThe term'neuromyelitis optica spectrum disorders'(NMOSD)is used as an umbrella term that refers to aquaporin-4 immu-noglobulin G(AQP4-IgG)-positive neuromyelitis optica(NMO)and its formes frustes and to a number of closely relatedclinical syndromes without AQP4-IgG.NMOSD were originally considered subvariants of multiple sclerosis (MS)but arenow widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis,clinicalpresentation,optimum treatment,and prognosis.In part 1 of this two-part article series,which ties in with our 2014 recom-mendations,the neuromyelitis optica study group(NEMOS)gives updated recommendations on the diagnosis and differentialdiagnosis of NMOSD.A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoproteinantibody-associated encephalomyelitis (MOG-EM;also termed MOG antibody-associated disease,MOGAD),which sharessignificant similarity with NMOSD with regard to clinical and,partly,radiological presentation,but is a pathogeneticallydistinct disease.In part 2,we provide updated recommendations on the treatment of NMOSD,covering all newly approveddrugs as well as established treatment options.Keywords Neuromyelitis optica spectrum disorders(NMOSD).Neuromyelitis optica(NMO)Optic neuritis.MyelitisDiagnostic criteria.Diagnosis.Differential diagnosis.MOG antibody-associated encephalomyelitis(MOG-EM).MOGantibody-associated disease(MOGAD).Magnetic resonance imaging (MRD.Serology.Cerebrospinal fluid(CSF).Optic coherence tomography (OCT).Clinical presentation.Aquaporin-4(AQP4).Myelin oligodendrocyte glycoprotein(MOG).AutoantibodiesAbbreviationsAOP4Aquaporin-4ACeAngiotensin-converting enzymeCADASILCerebral autosomal dominant arte-ADEMAcute disseminated encephalomyelitisriopathy with subcortical infarcts andAPSArea postrema syndromeleukoencephalopathyCBACell-based assayCHⅡ3LIChitinase 3-like 1 proteinThe members of the Neuromyelitis Optica Study Group (NEMOS)CLIPPERSChronic lymphocytic inflammation withare listed in acknowledgements section.pontine perivascular enhancementCNSCentral nervous system☒Sven JariusCSFsven.jarius@med.uni-heidelberg.deCerebrospinal fluidCT