Contributions
Abstract: EP1323
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Paediatric MS
Introduction: The diagnosis of chronic inflammatory central nervous system (CNS) diseases in pediatric patients is challenging given the variety of differential diagnosis and high frequency of monophasic events. Also, initial clinical presentations of chronic inflammatory CNS diseases such as multiple sclerosis (MS) may have lower predictive value in pediatric patients regarding the final diagnosis.
Objectives: We aim to describe the clinical and serologic characteristics of Brazilian pediatric patients after the first CNS inflammatory demyelinating event.
Methods: We analyzed the data of patients included in an ongoing Brazilian pediatric multicentric cohort (EMOCEMP Study) recruited from 4 centers.
Results: Thirty-three patients were included with a median age of 10 years (0-17 years) with a male to female ratio of 1.2. Of these, 69.7% (23) were Caucasian, 24.2% (8) were Mullatoes, one African and one Asian. Regarding clinical presentation, 33,3% (11/33) presented with optic neuritis (ON), 27.3% (9/33) presented with myelitis, 21% (7/33) with acute disseminated encephalomyelitis and 12% (4/33) had multifocal neurologic symptoms without encephalopathy. Thirty patients were tested for antibodies against aquaporin-4 (AQP4-Ab) and myelin oligodendrocyte glycoprotein (MOG-Ab). Of those 5 were MOG-Ab positive and one was AQP4-Ab positive. Most patients (90%) were treated in the acute phase with intravenous methylprednisolone. We did not observe significant differences in initial clinical presentations between patients younger and older than 10 years. However, isolated ON and/or myelitis was observed as initial presentation of 68% of the patients older than 10 years.
Conclusions: These preliminary results confirmed the difficulties of predicting the evolution of chronic inflammatory CNS disease after the first clinical episode in pediatric patients. As we expect a higher incidence of conversion to MS in patients older than 10 years, ON / myelitis presentations might add additional difficulties in the differential diagnosis with neuromyelitis optica spectrum disorders and MOG-Ab associated ON, encephalitis and myelitis (MONEM). The follow-up of these patients might allow us to identify the predictors to the conversion to MS and other diseases in this age group.
Disclosure: EMOCEMP study has received support from TEVA, FAPERGS, CNPq/Brazil. Bruna Klein da Costa receives scolarship from CNPq/Brazil and received travel grant from Merck. Rafael Canoni Sommer has nothing to disclose. Ricardo Zalewsky has nothing to disclose. Jefferson Becker has received reimbursement for developing educational presentations, educational and research grants, consultations fees and travel stipends from Bayer, Biogen, Ipsen, Merck, Novartis, Roche, Sanofi, Teva. Renata Barbosa Paolilo has received travel grant from Biogen and Roche. José Albino da Paz has nothing to disclose. Dagoberto Callegaro: Research support from CAPES/Brasil (CSF-PAJT88887.091277/2014-00). Fernanda Silveira de Quadros has notjing to disclose. Marlise de Castro Ribeiro has nothing to disclose. Hanaie Cavalli has nothing to disclose. Henry Koiti Sato received grants and consulting fees from Bayer, Biogen, Merck-Serono, TEVA, Sanofi, Genzyme, Roche, Novartis. Douglas K. Sato has received a Grants-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (KAKENHI 15K19472); research support from CNPq/Brasil (425331/2016-4), TEVA (research grant for EMOCEMP Investigator Initiated Study - NCT no. 61080516.4.1001.5336), and Euroimmun AG (Neuroimmunological Complications associated with Arboviruses); and speaker honoraria from Biogen, Novartis, Genzyme, TEVA, Merck-Serono, Roche, Bayer and is an advisory board member of Shire, Roche, TEVA, Merck-Serono and Quest/Athena Diagnostics.
Abstract: EP1323
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Paediatric MS
Introduction: The diagnosis of chronic inflammatory central nervous system (CNS) diseases in pediatric patients is challenging given the variety of differential diagnosis and high frequency of monophasic events. Also, initial clinical presentations of chronic inflammatory CNS diseases such as multiple sclerosis (MS) may have lower predictive value in pediatric patients regarding the final diagnosis.
Objectives: We aim to describe the clinical and serologic characteristics of Brazilian pediatric patients after the first CNS inflammatory demyelinating event.
Methods: We analyzed the data of patients included in an ongoing Brazilian pediatric multicentric cohort (EMOCEMP Study) recruited from 4 centers.
Results: Thirty-three patients were included with a median age of 10 years (0-17 years) with a male to female ratio of 1.2. Of these, 69.7% (23) were Caucasian, 24.2% (8) were Mullatoes, one African and one Asian. Regarding clinical presentation, 33,3% (11/33) presented with optic neuritis (ON), 27.3% (9/33) presented with myelitis, 21% (7/33) with acute disseminated encephalomyelitis and 12% (4/33) had multifocal neurologic symptoms without encephalopathy. Thirty patients were tested for antibodies against aquaporin-4 (AQP4-Ab) and myelin oligodendrocyte glycoprotein (MOG-Ab). Of those 5 were MOG-Ab positive and one was AQP4-Ab positive. Most patients (90%) were treated in the acute phase with intravenous methylprednisolone. We did not observe significant differences in initial clinical presentations between patients younger and older than 10 years. However, isolated ON and/or myelitis was observed as initial presentation of 68% of the patients older than 10 years.
Conclusions: These preliminary results confirmed the difficulties of predicting the evolution of chronic inflammatory CNS disease after the first clinical episode in pediatric patients. As we expect a higher incidence of conversion to MS in patients older than 10 years, ON / myelitis presentations might add additional difficulties in the differential diagnosis with neuromyelitis optica spectrum disorders and MOG-Ab associated ON, encephalitis and myelitis (MONEM). The follow-up of these patients might allow us to identify the predictors to the conversion to MS and other diseases in this age group.
Disclosure: EMOCEMP study has received support from TEVA, FAPERGS, CNPq/Brazil. Bruna Klein da Costa receives scolarship from CNPq/Brazil and received travel grant from Merck. Rafael Canoni Sommer has nothing to disclose. Ricardo Zalewsky has nothing to disclose. Jefferson Becker has received reimbursement for developing educational presentations, educational and research grants, consultations fees and travel stipends from Bayer, Biogen, Ipsen, Merck, Novartis, Roche, Sanofi, Teva. Renata Barbosa Paolilo has received travel grant from Biogen and Roche. José Albino da Paz has nothing to disclose. Dagoberto Callegaro: Research support from CAPES/Brasil (CSF-PAJT88887.091277/2014-00). Fernanda Silveira de Quadros has notjing to disclose. Marlise de Castro Ribeiro has nothing to disclose. Hanaie Cavalli has nothing to disclose. Henry Koiti Sato received grants and consulting fees from Bayer, Biogen, Merck-Serono, TEVA, Sanofi, Genzyme, Roche, Novartis. Douglas K. Sato has received a Grants-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (KAKENHI 15K19472); research support from CNPq/Brasil (425331/2016-4), TEVA (research grant for EMOCEMP Investigator Initiated Study - NCT no. 61080516.4.1001.5336), and Euroimmun AG (Neuroimmunological Complications associated with Arboviruses); and speaker honoraria from Biogen, Novartis, Genzyme, TEVA, Merck-Serono, Roche, Bayer and is an advisory board member of Shire, Roche, TEVA, Merck-Serono and Quest/Athena Diagnostics.