Clinical Analysis Of Myelin Oligodendrocyte Glycoprotein Antibody-associated Diseases

Background:Myelin oligodendrocyte glycoprotein antibody-associated diseases(MOGAD)is a rare,acquired inflammatory demyelinating disease of the central nervous system that has been proposed in recent years.However,with advances in detection technology,its incidence is increasing each year.The lesions can involve the optic nerve,brain tissue and spinal cord,which in turn can lead to visual impairment and paralysis.Previous reports have shown that MOGAD is present in all age groups,with differences between children and adults,and may be age-dependent.In addition,long-term follow-up has revealed that MOGAD has recurrent manifestations,and failure to receive timely and effective treatment during the acute phase may lead to neurological deficits and consequently severe disability.At the same time,the clinical manifestations overlap with neuromyelitis optica spectrum disease(NMOSD)and multiple sclerosis(MS),making the diagnosis more difficult clinically.Therefore,a comprehensive and adequate understanding of the clinical features of the disease is particularly important.Objective:1.To compare the clinical characteristics of first-onset MOGAD patients with different characteristics(age,severity,etc.),to explore the relevant factors influencing disease severity,and to construct models that can assess disease severity.2.To compare the clinical data of MOGAD,aquaporin-4(AQP-4)antibody positive NMOSD and MS patients with myelitis manifestations,and to explore the similarities and differences among patients with different myelitis phenotypes,in order to provide reference for clinical diagnosis and treatment.Materials and Methods:1.Clinical characteristics of MOG antibody-associated diseases and factors influencing disease severity.This retrospective study comprised 74 individuals with newly diagnosed MOGAD who was admitted to the First Affiliated Hospital of Zhengzhou University from January 2018 to June 2021.Patients were divided into two groups based on age:juvenile(<18 years)and adult(≥18 years)groups;the severity at first onset was assessed using the extended disability status scale(EDSS),and the patients were divided into the mild-to-moderate group(EDSS≤3)and severe group(EDSS>3),and the clinical data of two groups were compared separately.Multivariate logistic regression analysis was used to explore the correlate factors of disease severity.Plotting the Receiver Operating Characteristic curve(ROC)to evaluate the predictive power of the model.2.Comparison of clinical characteristics and prognosis of patients with MOGAD myelitis and other demyelinating related myelitis.Patients diagnosed as MOGAD,AQP-4 antibody-positive NMOSD,and MS in the First Affiliated Hospital of Zhengzhou University from January 2018 to June 2021 with the first onset was selected,and patients with myelitis symptoms were screened for inclusion in the study.There were 44 cases in the MOGAD group,77 cases in the NMOSD group,and 59 cases in the MS group.The three groups were analyzed and compared.Results:1.Clinical characteristics of MOG antibody related diseases and factors influencing disease severity.(1)Among the 74 patients with MOGAD included,the juvenile group has 34 cases and the adult group has 40 cases.The most common phenotype in the adult group was myelitis and the most common phenotype in the juvenile group was encephalitis,with statistically significant differences between the two groups(41.2%vs 75.0%,P=0.003;61.8%vs 22.5%,P=0.001).Conus medullas and longitudinally extensive myelitis were more common in the adult group than in the juvenile group,and the difference was statistically significant(22.5%vs 2.9%,P=0.035;55.3%vs 19.4%,P=0.001).The juvenile group had a higher positive rate of OCB in cerebrospinal fluid than the adult group,and the difference was statistically significant(26.5%vs 7.5%,P=0.027).(2)Among the 74 enrolled patients with MOGAD,36 were in the mild-to-moderate group(EDSS≤3)and 38 were in the severe group(EDSS>3).There were statistically significant differences in weakness,paresthesia,headache,bowel and bladder dysfunction between the two groups(13.9%vs 39.5%,P=0.013;5.6%vs 42.1%,P<0.001;13.9%vs 34.2%,P=0.042;0 vs 23.7%,P=0.006).Longitudinally extensive myelitis were more common in the severe group(55.3%vs 19.4%,P=0.001).Elevated albumin quotient was more common in the severe group than that in the mild-tomoderate group(57.9%vs 16.7%,P<0.001).(3)Multivariate logistic regression analysis showed that age(OR=1.069,95%CI:1.018-1.121,P=0.007),accompanying headache(OR=10.033,95%CI:2.100-47.944,P=0.004),longitudinally extensive myelitis(OR=14.005,95%CI:2.218-88.430,P=0.005)and elevated QALB(OR=9.302,95%CI:1.642-52.695,P=0.012)are independent risk factors for the severity of neurological impairment in MOGAD patients during the first course of the disease.(4)The area under the ROC for predicting disease severity in MOGAD patients by logistic regression model was 0.906,95%confidence interval was 0.839-0.972,P<0.001,sensitivity was 0.90,and specificity was 0.81.2.Comparison of clinical characteristics and prognosis of patients with MOGAD myelitis and other demyelinating related myelitis.(1)There were 33 cases of women(55.9%)in the MS group with a mean age of onset of 36.8±14.8 years,67 cases of women(87.0%)in the NMOSD group with a mean age of onset of 46.1±16.4 years,and 25 cases of women(56.8%)in the MOGAD group with a median age of onset of 27.7±16.1 years.Gender composition and age differences across the three groups were statistically significant(all P<0.001).(2)There were no manifestations of painful tonic spasm or zonesthesia in the MOGAD group,and there were more longitudinally extensive myelitis in NMOSD and MOGAD group than in the MS group,the difference was statistically significant(P<0.001,P=0.003).Elevated WBC counts in CSF of the MOGAD group were more common than that of the NMOSD group and MS group(all P<0.001).(3)The severity of nerve damage in the MOGAD group was closer to NMOSD than that in the MS group at first onset(P=0.010).After treatment,MOGAD patients recovered better than NMOSD patients(P<0.001).(4)The number and annual recurrence rate of the MS group were higher than those of the NMOSD group and MOGAD group(all P<0.05).Conclusions:1.MOGAD is age-dependent,with adults often starting with myelitis,and juvenile often starting with encephalitis.2.Age at first onset,concomitant headache,longitudinally extensive myelitis and increased albumin quotient(QALB)are independent risk factors for the severity of neurological impairment during the first course of MOGAD patients.3.The neurological impairment at the onset of MOGAD with myelitis is severe,but recovery is better when prompt treatment is given during the acute phase.4.MOGAD with myelitis is highly sensitive to hormones,but there is a long-term risk of recurrence.