Among ADSs, the clinical and imaging manifestations of some diseases overlap, and it is difficult to differentiate them clinically. The clinical phenotypes of ADSs are diverse, including acute disseminated encephalomyelitis (ADEM), transverse myelitis (TM), optic neuritis (ON), neuromyelitis optica spectrum disorder (NMOSD), and multiple sclerosis (MS). In addition to existing treatment plans, additional diagnoses and treatment plans should be developed to reduce recurrence and improve the prognoses of children with MOGAD.Īcquired demyelinating syndromes (ADSs) are a group of idiopathic immune-mediated diseases that originate in the brain (including the optic nerve) and/or spinal cord and are characterized by myelin damage or loss.
The clinical characteristics and imaging features of ADSs differed between MOG-Ab-positive and MOG-Ab-negative children. The initial treatment time correlated with the disease time course, and the prognosis may be affected by the disease time course and serum MOG-Ab titer ( P < 0.05). The average improvement time based on brain MRI was much longer in MOG-Ab-positive than in MOG-Ab-negative children ( P < 0.05). The most common shapes of the lesions were commas, triangles, or patches. Among the MOG-Ab-positive patients, the most affected brain areas detected via magnetic resonance imaging (MRI) were the temporal lobe, cerebellar hemispheres, brainstem, and periventricular lesions. There were significant differences in the MOG titer-related prognosis and disease time course between the disease relapse group and the non-relapse group ( P < 0.01).
There were ten cases of a rebound increase in MOG-Ab titers. Acute disseminated encephalomyelitis was the most common ADSs in both groups. MOG-Ab-positive children experienced more prodromal infections than did MOG-Ab-negative children ( P < 0.05). ResultsĪmong 90 children with ADSs, 30 were MOG-Ab-positive, and 60 were MOG-Ab-negative. The clinical data of children with MOGAD who were treated in the Department of Neurology at Shanghai Children's Hospital from January 2017 to October 2021 were retrospectively collected. Therefore, this study aimed to explore and analyze the clinical characteristics and prognoses of Chinese children with acquired demyelinating syndromes (ADSs) who tested positive or negative for MOG-Ab. In addition, further data on biomarkers are needed to predict the disease course could help guide management.Research on myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated disease (MOGAD) among Chinese children is relatively rare. More data is necessary to confirm risk of relapse in the years following diagnosis. Our cohort showed a higher relapse rate than has been reported previously and all known relapses occurred within one year of diagnosis. There is no consensus on whether maintenance therapy should be started for MOGAD cases with a single clinical event. DISCUSSION/CONCLUSION: Our study confirms prior data regarding the demographics, clinical presentation and radiologic presentation of MOGAD. Our cohort had a higher-than- expected percentage of cases with relapsing disease (56.3%) compared to monophasic (43.8%). The majority of our patients were treated with rituximab and we did not see a significant benefit of yearly relapse reduction for rituximab versus other therapies. Therapies include rituximab, IVIG, ocrelizumab, mycophenolate mofetil and ofatumumab. 83.3% of our patients were treated with chronic therapy at some point during their disease course.
IV methylprednisolone was used in the vast majority of cases for acute treatment. The most common clinical and radiologic presentation was optic neuritis followed by transverse myelitis and brainstem symptoms/lesions. No racial groups were affected disproportionately, and age of symptom onset spanned a large range with a median of 40 years. RESULTS: Our MOGAD cases revealed a slight female to male predominance of 1.5:1. Data was collected on patient demographics, clinical presentation, objective diagnosis with MRI and serum antibody levels, acute and chronic treatment and disease outcomes. METHODS: This retrospective chart review examined characteristics of thirty-three known adult MOGAD cases at a single institute. For clinicians and patients, the primary question is whether relapses will occur and whether to treat with chronic therapy. However, there are many questions that remain unanswered. The most common clinical presentation is optic neuritis, and first line acute treatment is intravenous (IV) steroids. Published version (DOI) Open Access Copy (Duke) Link to itemīACKGROUND/INTRODUCTION: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a chronic demyelinating disorder that has been increasingly recognized since the serum antibody became commercially available in 2017.
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