Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

Li, Ziyan; Sun, Hong; Fan, Xiaoxi; Yuan, Ping; Jiang, Yan; Wu, Peng; Zhong, Min; Ma, Jianjun; Jiang, Li; Li, Xiujuan

doi:10.3389/fneur.2021.642664

Cited by 7 publications

(17 citation statements)

References 38 publications

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“…We observed that the children with seropositive AQP4 antibody tended to be older than those with seropositive MOG antibody or seronegative AQP4-/MOG antibody, and a higher female:male ratio in AQP4 antibodypositive pediatric patients was not found in the children with positive MOG antibody or negative AQP4-/MOG antibody. A similar difference in the age and female:male ratio was observed in previous studies as well (6,28,29). We also found a difference in the spatial distribution of onset lesions in the children with different antibody types.…”

Section: Discussionsupporting

confidence: 91%

Risk Factors and Nomogram for Predicting Relapse Risk in Pediatric Neuromyelitis Optica Spectrum Disorders

Zhang

Qiao

et al. 2022

Front. Immunol.

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BackgroundNeuromyelitis optica spectrum disorders (NMOSDs) are attack-relapsing autoimmune inflammatory diseases of the central nervous system, which are characterized by the presence of serological aquaporin-4 (AQP4) antibody. However, this disorder is uncommon in children, and AQP4 antibody was often found to be seronegative. However, some pediatric patients diagnosed with NMOSDs were tested to be positive for myelin oligodendrocyte glycoprotein (MOG) antibody. The previous investigations of pediatric NMOSDs were usually focused on the clinical presentation, treatment responses, and long-term prognoses, but little is known about the risk factors predicting NMOSD relapse attacks in a shorter time, especially, for Chinese children.MethodsWe retrospectively identified 64 Chinese pediatric patients, including 39 positive for AQP4 antibody, 12 positive for MOG antibody, and the rest negative for AQP4 and MOG antibodies. Independent risk factors predicting relapse in 1-year follow-up were extracted by multivariate regression analysis to establish a risk score model, its performance evaluation was analyzed using receiver operating characteristic (ROC) curve, and the independent risk factors related to relapse manifestation were also explored through multivariate logistic analysis. A nomogram was generated to assess relapse attacks in 1-year follow-up. Thirty-five patients from 3 other centers formed an external cohort to validate this nomogram.ResultsFour independent relapsed factors included discharge Expanded Disability Status Scale (EDSS) (p = 0.017), mixed-lesion onset (p = 0.010), counts (≧1) of concomitant autoantibodies (p = 0.015), and maintenance therapy (tapering steroid with mycophenolate mofetil (MMF), p = 0.009; tapering steroid with acetazolamide (AZA), p = 0.045; and tapering steroid only, p = 0.025). The risk score modeled with these four factors was correlated with the likelihood of relapse in the primary cohort (AUC of 0.912) and the validation cohort (AUC of 0.846). Also, our nomogram exhibited accurate relapse estimate in the primary cohort, the validation cohort, and the whole cohort, but also in the cohorts with positive/negative AQP4 antibody, and noticeably, it performed predictive risk improvement better than other factors in the concordance index (C-index), net reclassification improvement (NRI), and integrated discrimination improvement (IDI).ConclusionsThe risk score and nomogram could facilitate accurate prognosis of relapse risk in 1-year follow-up for pediatric NMOSDs and help clinicians provide personalized treatment to decrease the chance of relapse.

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Section: Discussionsupporting

confidence: 91%

Risk Factors and Nomogram for Predicting Relapse Risk in Pediatric Neuromyelitis Optica Spectrum Disorders

Zhang

Qiao

et al. 2022

Front. Immunol.

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“…This general distribution of MOGAD phenotypes was also observed when assessing all identified studies (Table 1). Overall, 39 identified articles (including the six aforementioned incident cohort studies) reported the phenotype at initial presentation for 1,686 pediatric MOGAD patients 4–6,8,11–45 . Most of these studies were limited by the potential for selection bias in patient identification (e.g., patients with stored serum available for retrospective MOG‐Ab testing, relapsing disease course, etc.).…”

Section: Resultsmentioning

confidence: 99%

“…Approximately two-thirds of the identified studies not included in Table 2 (i.e., not inception cohorts) reported the proportion of pediatric MOGAD patients with a relapsing course to be greater than 30%; however, this is likely a result of selection bias. For example, in one study the proportion of pediatric and adult MOGAD patients with a relapsing course was 50% among the entire identified study cohort (i.e., a convenience sample) but only 25% when restricted to patients with an incident diagnosis who were identified prospectively (42). With regards to relapses, it is important to note that number and frequency of relapses reported is highly dependent upon the duration of the follow-up and the effectiveness of any chronic treatments received, and these factors varied substantially across studies making definitive interpretation difficult.…”

Section: Discussionmentioning

confidence: 99%

Attack phenotypes and disease course in pediatric MOGAD

Santoro

Beukelman²,

Hemingway

et al. 2023

Ann Clin Transl Neurol

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Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating condition that affects children differently than adults. We performed a literature review to assess the presentation and clinical course of pediatric MOGAD. The most common initial phenotype is acute disseminated encephalomyelitis, especially among children younger than five years, followed by optic neuritis (ON) and/or transverse myelitis. Approximately onequarter of children with MOGAD have at least one relapse that typically occurs within three years of disease onset and often includes ON, even if ON was not present at onset. Clinical risk factors for a relapsing course have not been elucidated.

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“…Clinical and radiological heterogeneity has age‐related differences in MOGAD. Younger children are more likely to have severe brain pathology and imaging involvement than older children (Fernandez‐Carbonell et al, 2016; Hacohen, Wong, et al, 2018; Li et al, 2021). In our study, the age at disease onset was under 6 years old, consistent with previous reports that typically children younger than 7 years are more prone to have leukodystrophy‐like brain lesions (Hacohen, Rossor, et al, 2018).…”

Section: Discussionmentioning

confidence: 99%

Clinical features of the first attack with leukodystrophy‐like phenotype in children with myelin oligodendrocyte glycoprotein antibody‐associated disorders

Jiang

Tan

et al. 2023

Intl J of Devlp Neuroscience

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Background Myelin oligodendrocyte glycoprotein antibody‐associated disorders (MOGAD) is identified autoimmune disorder with a predominance in paediatric patients, and the disease spectrum has expanded with clinical and radiological patterns. The aim of the study was to describe the clinical characteristics of the first attack with leukodystrophy‐like phenotype with MOGAD in children. Methods Patients hospitalized at the Children's Hospital of Chongqing Medical University from June 2017 to October 2021 with positive MOG antibodies and phenotype of leukodystrophy‐like (symmetric white matter lesions) were retrospectively analyzed. Cell‐based assays (CBAs) were used to test MOG antibodies. Results Four cases from 143 MOGAD patients were recruited, with two females and two males. The age of onset is all under 6 years old. At the last follow‐up, four cases exhibited a monophasic course, including ADEM in three patients and encephalitis in one patient. The mean EDSS score at onset was 4.62 ± 2.93, and the modified Rankin score (mRS) was 3.00 ± 1.82. First‐attack symptoms include fever, headache, vomiting, seizure, loss of consciousness, emotional and behavioural disorder, and ataxia. The brain MRI showed prominent extensive and essentially symmetric distribution lesions in the white matter. All patients showed clinical and partial radiological improvement after intravenous immunoglobulin and/or glucocorticoid treatment. Conclusion The first attack with MOGAD onset of leukodystrophy‐like phenotype was more frequently seen in younger children than other phenotype patients. The patients may show impressive neurologic disorders, but most patients who receive immunotherapy have a good prognosis.

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Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China

Cited by 7 publications

References 38 publications

Risk Factors and Nomogram for Predicting Relapse Risk in Pediatric Neuromyelitis Optica Spectrum Disorders

Risk Factors and Nomogram for Predicting Relapse Risk in Pediatric Neuromyelitis Optica Spectrum Disorders

Attack phenotypes and disease course in pediatric MOGAD

Clinical features of the first attack with leukodystrophy‐like phenotype in children with myelin oligodendrocyte glycoprotein antibody‐associated disorders

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