Clinical Evaluation Of AQP4-IgG For Neuromyelitis Optica Spectrum Disorders

Background:Neuromyelitis optica is an immune-mediated inflammatory demyelinating disease of the central nervous system(CNS)with optic nerve and spinal cord involvement.In 2004,Lennon et al.found the specific NMO-IgG in patients with NMO,and the following year AQP4 was proved to be a specific target by immunofluorescence histochemistry.Later,In order to define some of the diseases that are not completely consistent with the NMO diagnostic criteria,but the AQP4 antibody is positive,the concept of the neuromyelitis optica spectrum disorders is proposed.The traditional concept of NMO lesions is limited to the optic nerve and spinal cord.At present,there are many researches on the traditional concept of NMO in China,But there is less research on the new definition of NMOSD in 2015,This study is to meet the latest diagnostic criteria for patients with NMOSD in 2015.Objective:In this paper,we analyzed and explored the clinical symptoms?imaging features and prognosis after treatment in patients with AQP4-IgG positive NMOSD and AQP4-IgG negative patients with NMOSD,to achieve early diagnosis,early treatment,prognosis analysis,reduce the purpose of recurrence.Methods:Retrospective analysis the 67 patients with NMOSD who visit to the Henan Province People'sHospital from January 2016 to January 2017.Each patient was examined in detail by the internal medicine department,and the history of the patients was collected,the serological specimens were collected and AQP4-IgG was detected by indirect immunofluorescence assay.According to the test results,they were divided into AQP4-IgG positive group and AQP4-IgG negative group.The clinical symptoms,imaging features and prognosis after treatment were compared between the two groups.Results:(1)67 cases of NMOSD patients,AQP4-IgG positive patients with a total of 49 cases,AQP4-IgG negative patients with a total of 18 cases,male in 11 cases,female in 56 cases,male:female=1:5.09.The positive rate of AQP4-IgG in female patients was significantly higher than that in male patients,the difference was statistically significant(?~2=14.09,P=0.00).(2)The mean age of onset of AQP4-IgG positive group was(40.5±16.7)years old,and the average age of onset of AQP4-Ig G negative group was(38.5±18.6)years old.The statistical analysis showed that there was no significant difference between the two groups(t=0.42,P=0.68).(3)In the 67 cases of NMOSD patients,the onset of optic neuritis in 34 cases(50.8%),with simple myelitis as the first symptom in 18 patients(26.9%),for the first time in the pathogenesis of optic nerve and spinal cord were affected in 15 cases(22.4%).The statistical analysis showed that there was no significant difference between the two groups of AQP4-IgG positive group and AQP4-IgG negative group(?~2=0.00,P=1.00).There was significant difference between the two groups of AQP4-IgG positive group and AQP4-IgG negative group in the first symptom of myelitis(?~2=11.44,P=0.00).There were significant differences between the two groups of AQP4-IgG positive group and AQP4-IgG negative group in the first symptom of optic neuritis and myelitis(?~2=13.08,P=0.00).(4)The mean duration of AQP4-IgG positive group was(3.1±3.6)years,and the mean duration of AQP4-IgG negative group was(1.2±2.9)years,the difference was statistically significant between the two groups(t=2.08,P=0.04).(5)67 cases of NMOSD patients with autoimmune disease in 18 cases(26.9%).There was significant difference between AQP4-IgG positive group and AQP4-IgG negative group(?~2=6.94,P=0.01).(6)In the AQP4-IgG positive group,the number of segments of spinal cord involvement was (5.30±5.96),and the number of spinal cord involvement in AQP4-IgG negative group was(5.28±3.63).There was no significant difference between the two groups(t=0.02,P=0.99).There were 6 cases of medullary involvement in AQP4-IgG positive group and 3 cases of medullary involvement in AQP4-IgG negative group,There was no significant difference between the two groups(?~2=0.00,P=0.97).There were16 cases of thoracic spinal cord involvement in AQP4-IgG positive group and 8 cases of thoracic spinal cord involvement in AQP4-IgG negative group.There was no significant difference between the two groups(?~2=0.80,P=0.37).There were 14 cases of abnormal intracranial lesions in AQP4-IgG positive group and 8 cases of abnormal intracranial lesions in AQP4-IgG negative group.There was no significant difference between the two groups(?~2=1.50,P=0.22).(7)Two groups of patients after treatment EDSS score,AQP4-Ig G positive group(2.2±1.3)points and AQP4-IgG negative group(1.6±0.6)points,the difference between the two groups was statistically significant(t=2.02,P=0.04).Conclusion:In patients with NMOSD who met the 2015 IPND diagnostic criteria:(1)the onset of optic neuritis and myelitis were more common in patients with AQP4-IgG negative;(2)AQP4-IgG positive patients with NMOSD are more likely to be associated with autoimmune diseases;(3)There was no difference in the number and location of spinal cord involvement in patients with AQP4-IgG positive NMOSD and AQP4-IgG negative NMOSD.(4)AQP4-IgG positive indicate severe clinical attack and worse prognosis.