Comparative Analysis Of Clinical Features Of MOG And AQP4 Antibody Related Demyelinating Diseases

Background and objective:Myelin Oligodendrocytes Glycoprotein Ig G（MOGAD）,initially considered a subtype of Neuromyelitis optica spectrum disease（NMOSD）,which has only recently been recognized as a completely separate disease entity.And with the further understanding of MOGAD,the related clinical disease spectrum is also enlarging.The purpose of this study was to further summarize the clinical phenotypes and characteristics of MOGAD by comparing and analyzing the clinical characteristics of MOGAD and AQP4-positive NMOSD,in order to improve the understanding of MOGAD by medical staff.Method:The complete medical records of patients who were admitted to the Department of Neurology,Pediatrics and Ophthalmology of the First Hospital of Jilin University from April 2018 to November 2021 were retrospectively collected.There were 76 cases who met the criteria in this study,including 54 MOGAD patients and 21AQP4-positive NMOSD patients.Patients in the MOGAD group were further classified into the encephalitis group（encephalitis and meningoencephalitis）and the non-encephalitis group（optic neuritis and myelitis）according to the clinical phenotype.The general situations,clinical symptoms and phenotypes,laboratory and imaging examinations of the patients were recorded.SPSS 26.0 was used for statistical analysis.Results:A total of 54 MOGAD patients and 21 AQP4-positive NMOSD patients were collected in this study.The MOGAD group was predominantly male（55.6%）and the age of initial onset was 22.5（14.75,51.0）years,while the AQP4-positive NMOSD group was mainly female（81.0%）and the onset age was 54.0（39.5,58.0）years.There were significant differences in gender ratio and age of first onset between the two groups（P=0.004,P=0.001）.The proportion of patients with onset in spring and summer in the MOGAD group（51.9%）was not remarkably different from that in the AQP4 positive NMOSD group（61.9%）（P>0.05）.In the MOGAD group,18 patients were included in the encephalitis group and 18 cases in the non-encephalitis group.There was no considerable difference in the ratio of male to female in the encephalitis group（1.25:1）and non-encephalitis group（1:1）（P > 0.05）.The onset age of 18.5（13.0,26.3）years in the encephalitis group was significantly lower than that in the non-encephalitis group 51.0（22.5,57.0）years（P=0.003）.There was no statistical difference in prodromal history between the MOGAD group（11.1%）and AQP4-positive NMOSD group（4.8%）（P > 0.05）.The first clinical manifestations of headache and/or fever were more common in 15 patients（27.8%）in the MOGAD group than in 1 patient（4.8%）in the AQP4-positive NMOSD group（P=0.031）.Visual impairment such as blurred vision was more common in 9 AQP4-positive NMOSD patients（42.9%）than in 10 MOGAD patients（18.5%）（P=0.03）.17 patients with Encephalitis（31.5%）and 11 cases of Acute disseminated encephalomyelitis（ADEM）phenotype（20.4%）)were more common in the MOGAD group than in the AQP4-positive NMOSD group（P=0.002,P=0.028）.Because these two phenotypes were not seen in the AQP4-positive NMOSD group.Transverse myelitis（TM）phenotypes were more common in AQP4-positive NMOSD patients（11 cases,52.4%）than in the MOGAD group（11cases,20.5%）（P=0.006）.The initial Expanded Disability Status Scale（EDSS）score of MOGAD group 2.0（2.0,5.0）was significantly lower than that of AQP4-positive NMOSD group 6（3.0,7.5）（P < 0.005）.EDSS score 2.0（1.0,2.0）of the encephalitis group was significantly lower than that of the non-encephalitis group 4.5（2.0,7.0）（P < 0.05）.The cerebrospinal fluid white blood cell count 36.5（11.3,95.6）*10^6/L was higher than that in AQP4-positive NMOSD group 16（4,20）（10^6/L）（P=0.011）.There was no statistical significance in the cerebrospinal fluid protein determination（P > 0.05）.The white blood cell count in the cerebrospinal fluid of the patients with encephalitis in MOGAD group was 111.5（47.5,209.75）*10^6/L,which was significantly higher than that of patients of non-encephalitis group 14（6,24）)*10^6/L（P < 0.001）.Platelet count in MOGAD group was 279.5（227.8,335.0）*10^6/L higher than that in NMOSD group 249.0（220.0,283.5）*10^6/L（P=0.016）.While there were no statistical differences in the number of white blood cells,neutrophils,lymphocytes,monocytes,NLR,PLR,SIRI.（P > 0.05）.In the encephalitis group,the white blood cell count was 11.06（8.84,12.68）*10^9/L,and the neutrophil count was 7.31（5.96,10.29）*10^9/L.Leukocyte count was 7.06（5.09,8.83）*10^9/L,neutrophil count was 4.35（3.08,6.41）*10^9/L,SIRI:1.08（0.57,1.51）*10^9/L（P=0.001,P=0.001,P=0.037）.There were no significant differences in monocyte count,lymphocyte count,platelet volume,NLR and PLR between the two groups（P > 0.05）.There was no statistical difference in the distribution of lesions（cortical/subcortical,brainstem,paraventricular/deep white matter,etc.）between MOGAD group and AQP4-positive NMOSD group on Magnetic Resonance Imaging（MRI）of the head（P > 0.05）.In AQP4-positive NMOSD group,8 cases（38.1%）of longitudinally extensive transverse myelitis（LETM）were more common than 4 cases（10.0%）of MOGAD group,but there was no statistical difference in spinal cord MRI involvement（P > 0.05）.The proportion of abnormal visual evoked potential（VEP）was 55.2%（16 cases）in MOGAD group and 50.0%（8cases）in AQP4-positive NMOSD group（P > 0.05）.In terms of treatment,41 patients（75.9%）in MOGAD group received high dose Intravenous methylprednisolone（IVMP）and/or Intravenous immunoglobulin（IVIG）,which was more common than 4patients（19.0%）in AQP4-positive NMOSD group.Immunosuppressants were more common in 15 AQP4-positive NMOSD patients（75.0%）than in 9 MOGAD patients（16.7%）（P < 0.05）.In the acute stage of MOGAD,11 cases（61.1%）of encephalitis patients were treated with high-dose IVMP and/or IVIG,which was more common than 4 cases（22.2%）of non-encephalitis group（P < 0.05）.The recurrence rate for the MOGAD group and AQP4-positive NMOSD group are respectively11.1%,4.8%.The recurrence rate of 5 cases in the encephalitis group（27.8%）and in the non-encephalitis group（0）.Conclusion:1.Compared with the patients of NMOSD group with positive AQP4,the patients of MOGAD group had younger onset age,more common encephalitis,ADEM phenotype and relatively mild symptoms in the acute phase.2.In the MOGAD subgroup,the inflammatory response in cerebrospinal fluid and peripheral blood of encephalitis patients were more severe than those in the non-encephalitis group,and the EDSS scores were lower.3.In conclusion,MOGAD and AQP4-positive NMOSD are two different diseases due to differences in morbidity,first symptoms and clinical phenotype.