ICNC Abstracts, ICNC 2018

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Pediatric Acquired Demyelination Syndromes (ADS) profile in a Brazilian center
José Albino da Paz, Renata Paolilo, Samira Apostolos-Pereira, Dagoberto Callegaro, Ana Luiza Araújo, Douglas Sato, Carolina Medeiros Rimkus

Last modified: 2018-09-09

Abstract


Introduction: ADS represent a group of inflammatory disorders of the central nervous system such as Multiple Sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), acute disseminating encephalomyelitis (ADEM), clinically isolated syndromes (CIS) and the recently described Myelin Oligodendrocyte Glycoprotein antibody (MOG-IgG) associated disease. ADS are rare in childhood and their prevalence is unknown in Brazil.

Objective: Describe the demographic and clinical aspects of pediatric ADS patients in a Brazilian center.

Methods: Review of records from patients at Pediatric Neuroimmunology Clinic São Paulo University. All patients under 18 years of age and fulfilled current International Pediatric Multiple Sclerosis Study Group diagnostic criteria for MS, NMOSD, ADEM, CIS were included in addition to patients with MOG-IgG seropositivity.

Results: 150 patients were identified, 145 were analyzed: 80 were females (55,1%); median age of onset was 14 years. Autoantibodies (MOG-IgG and aquaporin4-IgG) were tested for 61 (42%) patients. MS patients: 75 (52%), all with recurrent course. NMOSD patients: 26 (18%), 18 were aquaporin4-IgG positives (69%), all recurrent; 5 were MOG-IgG positives (19%), 4 recurrent; 3 were double seronegatives. ADEM patients: 11 (7,5%), all monophasic. Optic neuritis (ON) patients: 15 (10%), 8 monophasic. Transverse myelitis (TM) patients: 9 (6%), 7 monophasic. CIS patients: 8 (5,5%), all monophasic. One patient with MOG-IgG had leukodystrophy-like phenotype with recurrent course.

Conclusions: most patients had recurrent ADS (75%) and MS was the most common. Autoantibodies tested in some patients revealed 19% of seropositivity and MOG-IgG patients showed heterogeneous clinic manifestation.


Keywords


multiple sclerosis; neuromyelitis optica spectrum disorder; acute disseminating encephalomyelitis

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