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Striving for the best treatment for Pediatric Acute demyelinating syndromes: results from cohort analysis
Last modified: 2014-04-03
Abstract
%INTRODUCTION: The 2013 International Pediatric MS Study Group report brings awareness to the difficulties in managing the diagnostic work-up and treatment of Pediatric Acute Demyelinating Syndromes (ADS), and highlights their differences from adult onset forms and the need for pediatric guidelines.
Taking into account that pediatric ADS course with increased inflammatory response, frequent relapses, long term disabilities and quality of life losses, a specialized outpatient clinic to follow-up children with ADS was created in our hospital. Cohort analysis of these patients and work-up/therapeutic strategies are described in this study.
METHODOLOGY: Prospective cohort analysis from a Pediatric ADS clinic population over the past year.
RESULTS: Fourteen patients (7 boys), mean age of 7.3yo (range: 1.8-13yo), at their first episode of ADS were seen in clinic, and protocols were created to be used through their differential diagnosis, relapses and remission phases. Four presented with multiple sclerosis (MS), 5 with Acute Disseminated Encephalomyelitis (ADEM), 2 with Neuromyelitis Optica (NMO), and 3 with Clinically Isolated Syndromes. Six patients presented ADS symptoms prior to temporal-spatial dissemination diagnosis. Serum from all patients were tested for inflammatory biomarkers: IgG index calculation, oligoclonal Bands, lymphocyte counts, cytokines (IFN-gamma, TNF-alpha, IL-17, IL-4 and IL-10). Currently, three patients with MS are treated with subcutaneous Interferon beta-1A, and a fourth with Glatiramer acetate due to prior treatment failure. NMO is treated with Azathioprine or Glatiramer acetate. Immunotherapy/cyclophosphamide treatment is used for ADEM with good response.
CONCLUSION: This preliminary cohort analysis describes our current work-up and therapeutic protocols for ADS at our hospital.
Taking into account that pediatric ADS course with increased inflammatory response, frequent relapses, long term disabilities and quality of life losses, a specialized outpatient clinic to follow-up children with ADS was created in our hospital. Cohort analysis of these patients and work-up/therapeutic strategies are described in this study.
METHODOLOGY: Prospective cohort analysis from a Pediatric ADS clinic population over the past year.
RESULTS: Fourteen patients (7 boys), mean age of 7.3yo (range: 1.8-13yo), at their first episode of ADS were seen in clinic, and protocols were created to be used through their differential diagnosis, relapses and remission phases. Four presented with multiple sclerosis (MS), 5 with Acute Disseminated Encephalomyelitis (ADEM), 2 with Neuromyelitis Optica (NMO), and 3 with Clinically Isolated Syndromes. Six patients presented ADS symptoms prior to temporal-spatial dissemination diagnosis. Serum from all patients were tested for inflammatory biomarkers: IgG index calculation, oligoclonal Bands, lymphocyte counts, cytokines (IFN-gamma, TNF-alpha, IL-17, IL-4 and IL-10). Currently, three patients with MS are treated with subcutaneous Interferon beta-1A, and a fourth with Glatiramer acetate due to prior treatment failure. NMO is treated with Azathioprine or Glatiramer acetate. Immunotherapy/cyclophosphamide treatment is used for ADEM with good response.
CONCLUSION: This preliminary cohort analysis describes our current work-up and therapeutic protocols for ADS at our hospital.
Keywords
Pediatric Multiple Sclerosis; pediatrics; acute demyelinating syndromes; Acute Disseminated Encephalomyelitis ; Neuromyelitis Optica ; Clinically Isolated Syndromes; Immunotherapy; neuroimmunology
References
Krupp et al., International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler. 2013 Sep;19(10):1261-7
Dale et al., Pediatric nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica and multiple sclerosis. Curr. Opin. Pediatr, 22: 233-240, 2009.
Chitnis et al.International Pediatric MS Study Group clinical Trials Summit: meeting report. Neurology. 2013 Mar 19;80(12):1161-8
Dale et al., Pediatric nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica and multiple sclerosis. Curr. Opin. Pediatr, 22: 233-240, 2009.
Chitnis et al.International Pediatric MS Study Group clinical Trials Summit: meeting report. Neurology. 2013 Mar 19;80(12):1161-8
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