Abstract
Background/Objective: The aim of the study was to estimate the rate of evolution or for multiple sclerosis (MS), after a first acute demyelinating event (ADE) in pediatric patients, and to investigate the variables that predict this evolution.Methods: We retrospectively evaluated the clinical and neuroradiological features of children who presented a first ADE between January 2005 and April 2017. All patients included underwent a baseline MRI, a cerebrospinal fluid and blood analysis, including virological examinations. The evolution into MS was determined by the 2013 International Pediatric Multiple Sclerosis Study Group (IPMSSG) criteria. Clinical and radiological features predictive of MS were determined using multivariate analyses.Results: Ninety-one patients were selected (mean age at onset: 10.11 ± 4.6). After a mean follow-up of 5.6 ± 2.3 years, 35% of patients' conditions evolved to MS. In the logistic multivariate analysis of clinical and laboratory data, the best predictors of evolution into MS were: the presence of oligoclonal bands in CSF (p < 0.001), past infection with EBV (p < 0.001), periventricular lesions (p < 0.001), hypointense lesions on T1 (p < 0.001), and lesions of the corpus callosum (p < 0.001) including Dawson fingers (p < 0.001).Conclusion: Our findings suggest that a pattern of neuroimaging and laboratory findings may help to distinguish between, at clinical onset, children with a monophasic syndrome (clinically isolated syndrome or acute disseminated encephalomyelitis) from those who will develop MS.
Highlights
The term “acquired demyelinating syndrome” (ADS) is used to indicate the first clinical episode of acute central nervous system (CNS) demyelination, which can either represent the sentinel attack of an underlying chronic demyelinating disorder or remain monophasic
Studies have focused on defining criteria for early diagnosis in children and adolescents, as well as focusing on searching for predictive markers of progression into the recurrent disease (2, 5, 8–10). These studies have some limits: (1) the incidence of multiple sclerosis varies in different cohorts (15–46%), because different inclusion criteria are applied, even based on MRI findings alone, without considering clinical symptoms/signs (2, 4–6, 15); (2) some studies include patients aged between 16 and 18 years, leaving childhood unexplored (5, 7); (3) it is difficult to distinguish between patients with ADEM from MS patients with ADEM-like onset, at the time of the first attack (7, 8, 10, 16); and (4) predictors with high specificity and positive predictive value show low sensitivity (5, 7, 17)
The inclusion criteria were: (1) a history of an acute neurological event suggestive of central nervous system (CNS) inflammatory demyelinating disease not attributable to other conditions; (2) a clinical follow-up of at least 1 year; (3) age at onset ≤16 years; (4) available laboratory and clinical data at onset (clinical symptoms according to EDSS classification, serum, and cerebrospinal fluid (CSF) examinations, serology for Epstein Barr virus (EBV)); and (5) brain and spinal MRI at onset and during follow-up
Summary
The term “acquired demyelinating syndrome” (ADS) is used to indicate the first clinical episode of acute CNS demyelination, which can either represent the sentinel attack of an underlying chronic demyelinating disorder or remain monophasic. Studies have focused on defining criteria for early diagnosis in children and adolescents, as well as focusing on searching for predictive markers of progression into the recurrent disease (2, 5, 8–10) These studies have some limits: (1) the incidence of multiple sclerosis varies in different cohorts (15–46%), because different inclusion criteria are applied, even based on MRI findings alone, without considering clinical symptoms/signs (2, 4–6, 15); (2) some studies include patients aged between 16 and 18 years, leaving childhood unexplored (5, 7); (3) it is difficult to distinguish between patients with ADEM from MS patients with ADEM-like onset, at the time of the first attack (7, 8, 10, 16); and (4) predictors with high specificity and positive predictive value show low sensitivity (5, 7, 17). The present study aims: (1) to analyze the clinical and neuroimaging features of a pediatric population at ADS onset; (2) to identify possible predictors of progression into MS; and (3) to compare the predictors obtained from our results with those of previous studies
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