Clinical Comparative Analysis Of 76 Children And Adults With Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease

Background and ObjectiveMyelin oligodendrocyte glycoprotein(MOG)is specifically expressed in the outermost layer of the myelin sheath of the central nervous system(CNS)and has high immunogenicity.With the popularization of CNS demyelination-related antibody detection in clinical practice,myelin oligodendrocyte glycoprotein antibody(MOG-IgG)has been detected in patients with idiopathic inflammatory demyelinating disease in recent years.Later studies found that the immunopathology and clinical manifestations of this disease had its own particularity,so the disease spectrum was gradually separated and named myelin oligodendrocyte glycoprotein antibody-associated disease(MOGAD).There were few reports about MOGAD in China and many deficiencies in the understanding of the disease,but the clinical decision-making for MOGAD is still in the process of exploration.Therefore,this study analyzed and summarized the clinical characteristics and prognosis of MOGAD patients in our hospital,and compared children with adult patients to deepen the understanding of MOGAD and provide reference for disease diagnosis,treatment,prognosis evaluation and specific management of different age groups.Materials and MethodsThe clinical data of 76 patients with MOGAD treated in the Department of Neurology,Pediatrics and Ophthalmology of the First Affiliated Hospital of Zhengzhou University between August 2019 and August 2021 were analyzed retrospectively,including sex,age,history of prodromal infection,first symptom,clinical phenotype,auxiliary examination(serum and cerebrospinal fluid autoantibody detection,cerebrospinal fluid detection,head magnetic resonance imaging(MRI),spinal MRI,optic nerve MRI,neuroelectrophysiological examination),immunotherapy,etc.The prognosis was followed up by outpatient clinic or telephone.The patients were scored with expanded disability status scale(EDSS)at the first acute attack without regular drug treatment and at the last follow-up.The patients with MOGAD were divided into children group and adult group according to age,and the differences in general information,initial symptoms,clinical manifestations,auxiliary examination,treatment and prognosis between the two groups were discussed.Results1.76 patients with MOGAD had a male/female ratio of 1.2:1 with a median age of 17(8～33)years.Children accounted for 51.3%(39/76),and adults accounted for 48.7%(37/76).There was no significant difference in gender distribution between the two groups(P>0.05).Fever,vision loss and headache were the most common symptoms of the first attack,in which the first symptoms of epilepsy and dysarthria were significantly more in children than in adults,while sensory and defecation disorders were more common in adult patients(P<0.05).Among all the phenotypes,encephalitis has the highest proportion.Compared with adult patients,encephalitis and ADEM phenotype were more in children(P<0.05).2.Among the 76 MOGAD patients,60.5%(46/76)had intracranial lesions at the first attack,and the intracranial lesions in children were more likely to involve subcortical and cerebellum than adults(P<0.05).Spinal MRI showed that 86.7%(26/30)of patients with myelitis had long segmental lesions with the highest proportion of cervicothoracic spinal cord damage,and some patients could show multiple discontinuous lesions.In MOGAD-ON patients with abnormal optic nerve MRI,88.9%(8/9)had involvement of the anterior segment of the optic nerve,especially in the intraorbital segment.3.Among MOGAD patients completing other autoimmune antibodies detection,8.0%(3/38)were GFAP-IgG positive in cerebrospinal fluid,19.4%(6/31)were NMDAR-IgG positive,3.2%(1/31)were Caspr2-IgG positive,16.9%(11/65)had abnormal thyroid antibodies,and 34.4%(11/32)had abnormal systemic autoimmune antibodies.Two MNOS patients showed typical symptoms of NMDAR encephalitis and secondary demyelination and the remaining 4 MNOS patients only had demyelinating manifestations.No tumor was found in all MNOS patients.The 3 MOGAD patients with GFAP-IgG positive were all childhood-onset,and manifested as hydrocephalus and demyelinating lesions.One case of MOGAD with Caspr2-IgG positive presented with limbic encephalitis.4.All patients with MOGAD improved the routine examination of cerebrospinal fluid during the acute attack.22.4%(17/76)of the protein content increased,and 61.8%(47/76)of the white blood cell count increased,among which the frequency of neutrophils in children was higher than that in adult patients(P<0.05).71 patients underwent cerebrospinal fluid protein electrophoresis.31.0%(22/71)of cerebrospinal fluid/serum albumin quotient(QAlb)were elevated,15.5%(11/71)of cerebrospinal fluid had oligoclonal band.There was no significant difference in the increase of QAlb and the positive rate of oligoclonal band between children and adults.5.The EDSS score of 76 patients with MOGAD in acute phase was 3.0(2.0～3.5),which was not correlated with the titer of contemporaneous MOG-IgG,and it is also not correlated between the two different age groups(P>0.05).However,the EDSS score in children was lower than that in adult(P<0.05).All patients received immunotherapy.The proportion of combined treatment of hormone and intravenous immune globulin in children in acute stage was higher than that in adults,but the proportion of adult patients treated with hormone was more common than that in children(P<0.05).During the remission period,more children were treated with low-dose steroids,but the proportion of adults who received low-dose steroids combined with immunosuppressive therapy was significantly higher than that of children(P<0.05).The median follow-up time of the 73 effective cases was 11(9～18)months,The EDSS score at the last follow-up was 0.0(0.0～1.0),which was significantly lower than that in the acute phase/P<0.05).The prognosis of children was relatively better,and the proportion of patients with EDSS and persistent disability at the last follow-up were lower than those of adults(P<0.05).23.3%(17/73)patients had recurrence,including ON,myelitis and ADEM.There was no significant difference in the proportion and phenotypic distribution of recurrence between children and adults(P>0.05).Conclusion1.Among the first symptoms of MOGAD,epileptic seizures and dysarthria were more common in children,while sensory disturbances and urination and defecation disorders were more common in adults.2.Compared with adult patients,children with MOGAD showed more encephalitic phenotype and ADEM phenotype and intracranial lesions were more likely to involve subcortical and cerebellum.3.The increase of white blood cell count in cerebrospinal fluid was common in patients with MOGAD,in which the frequency of neutrophils was higher in children.4.The prognosis of MOGAD patients was good.The neurological damage degree of children in the acute stage was relatively milder,and the prognosis is better.