| Objective:To analyze the clinical features of inflammatory demyelinating diseases mediated by myelin oligodendrocyte glycoprotein(MOG)antibody,so as to improve clinical understanding of inflammatory demyelinating diseases with positive MOG antibody.Methods:We retrospectively analyzed the gender,age of onset,clinical symptoms,laboratory and imaging results,treatment and prognosis of 11 patients with MOG antibody-positive inflammatory demyelinating diseases who were hospitalized in Fujian Provincial Hospital and Fujian Medical University Union Hospital from October 2016to January 2021.Results:(1)This study included 8 males and 3 females,with a ratio of 2.67:1.The age of onset was 15-58 years old,with a median of 28 years old,and young people were in the majority.(2)Encephalopathy was the most common initial symptom,and the main clinical manifestations were encephalopathy,brainstem symptoms,myelitis,and ON.6 patients were accompanied by fever during the course.(3)All patients were positive for MOG antibody in sreum,with titer ranging from 1:10-1:100.3 patients were positive for MOG antibody in CSF,with titer ranging from 1:1-1:3.2.Intracranial hypertension occurred in 2 cases,the CSF leukocyte count increased in 10 cases,of which 7 cases were less than 100×10~6/L,and the CSF protein increased in 3 cases.10cases of intracranial abnormal signals were all involved in the white matter of the brain,including corpus callosum,as well as in the brainstem,basal ganglia,thalamus,and cerebral cortex.Widening of the ventricle system occurred in 1 case.Abnormal signals of the spinal cord were found in 4 cases,involving the cervical and thoracic segments,2cases of continuous long-segment lesions and 1 case of discontinuous short-segment lesions.(4)7 cases were positive for other autoantibodies,while only 1 case had other autoimmune disease.(5)6 cases had an ADEM-like clinical phenotype,including clinical diagnosis of ADEM 3 cases,2 cases of encephalitis,encephalomyelitis in 1 case.5 cases had an NMOSD-like clinical phenotype,including clinical diagnosis of NMOSD 1 case,1 case of ON,2 cases of myelitis,brainstem encephalitis in 1 case.(6)All patients were treated with low-dose or high-dose steroidhormone in the acute phase,some were treated with IVIG,and 1 case was treated with oral mycophenolate mofetil in the remission stage.The follow-up was 2-51 months,with a median of 17 months.During the follow-up period,4 patients had recurrences,with a total of 2-3 attacks.The interval between the initial relapse and the first onset was 3-16 months,with a median of 6 months.During the follow-up period,the mean titer of serum MOG antibody was1:12.14 in patients with monophasic course,while the mean titer of serum MOG antibody was 1:50.24 in patients with recurrent courses.Most of them had a good prognosis,while one had neurological disability.Conclusion:(1)Inflammatory demyelinating diseases mediated by MOG antibody are more common in young people,with clinical manifestations including ON,myelitis,meningoencephalitis,brainstem encephalitis,etc.Encephalitis is an important clinical phenotype of this disease,often accompanied by epilepsy.Fever may be a clinical feature of this disease,which is distinct from other CNS demyelinating diseases.Most patients may be positive for other autoantibodies.(2)Intracranial lesions can be multifocally distributed in the supratentorial or infratentorial white and gray matter.Spinal cord lesions can be continuous long-segment lesions or discontinuous short-segment lesions.(3)Serum MOG antibody is the first choice for diagnosis and monitoring in clinical practice,and serum MOG antibody titer may be related to recurrence.(4)High-dose steroidhormone and IVIG are effective for the recovery of neurological function in the acute phase,and immunosuppressive therapy can be given to patients with recurrent steroid-dependent disease courses.The majority of patients have a good prognosis,but there is a risk of recurrence and disability. |
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