Contributions
Abstract: EP1311
Type: ePoster
Abstract Category: Clinical aspects of MS - 3 Paediatric MS
Introduction: NMOSD is an inflammatory central nervous system condition mediated by serum aquaporin-4 immunoglobulin G antibody (AQP4-IgG). After knowledge of importance AQP4 IgG new diagnostic criteria to adult NMOSD has been described. Pediatric onset NMOSD is rarely described and long-term follow up are missing. The present report aim to describe a long-term follow-up pediatric onset NMOSD using the International Painel of NMO Diagnosis (IPND) updated in 2015.
Method: We assessed all patients enrolled in our neuroimmunology service from January 2005 to April 2017 whose NMOSD symptoms began before 18 years-old and met the updated 2015-IPND diagnostic criteria.
Results: from 375 patients with NMOSD, 16 were pediatric cases (4,2%). There were 2/16 males, 4/16 caucasian and 12/16 of mixed ethnic background. Three patients had previous infectious disease. Two patients had other autoimmune conditions. Median age at onset was 11(6-18) years. Presenting symptoms were optic neuritis (9), myelitis (4) and acute brainstem syndrome (3). Median time to the second relapse was 6(1-36) months. Median annualized relapse rate was 1relapse/year. The main relapse syndromes were: optic neuritis (45), myelitis (36), brainstem (5), area postrema (2) and diencephalic (1). Eleven (68,8%) patients were AQP4-IgG positive. Thirteen patients had abnormalities in the cerebrospinal fluid but none had positive oligoclonal bands. MRI findings were compatible with NMOSD in all patients. No patient full field Mcdonald-2010 Multiple Sclerosis criteria. All patients were acutely treated with methylprednisolone, followed by plasmapheresis (8), cyclophosphamide (6) and immunoglobulin (3). The median relapse number prior to maintenance treatment introduction was 2(1-6). Patients were first treated with azathioprine (14) or mycophenolate (2). Five patients switched azathioprine to mycophenolate (4) or rituximab (1), due to relapses. Median EDSS of 3.5(1-8) after 5(1-17) years of follow-up. Eleven patients had visual acuity less than 20/200 in the worst eye.
Conclusion: Our results confirm literature emphasizing optic neuritis as mainly inaugural presentation in pediatric NMOSD. This is the first long term follow up of pediatric NMOSD using 2015-IPND criteria showing a higher disability than previous report.
Disclosure:
Renata Barbosa Paolilo: nothing to disclose.
José Albino da Paz: nothing to disclose.
Samira Luisa Apostolos Pereira: received grants related to congress meetings from Genzyme and Roche.
Carolina de Medeiros Rimkus: nothing to disclose.
Ana Luiza Camara Araujo: nothing to disclose.
Lais Maria Gomes de Brito Ventura: nothing to disclose.
Milena Sales Pitombeira: nothing to disclose.
Ana Beatriz Gomes: nothing to disclose.
Aline de Moura Brasil Matos: nothing to disclose.
Pedro Henrique Bruel Torretta: nothing to disclose.
Umbertina Conti Reed: nothing to disclose.
Dagoberto Callegaro: received grants related to congress meetings from Genzyme and Roche.
Douglas Kazutoshi Sato: CAPES/brasil, Japan Society of Science. Speaker for Merck, Shire, TEVA.
Abstract: EP1311
Type: ePoster
Abstract Category: Clinical aspects of MS - 3 Paediatric MS
Introduction: NMOSD is an inflammatory central nervous system condition mediated by serum aquaporin-4 immunoglobulin G antibody (AQP4-IgG). After knowledge of importance AQP4 IgG new diagnostic criteria to adult NMOSD has been described. Pediatric onset NMOSD is rarely described and long-term follow up are missing. The present report aim to describe a long-term follow-up pediatric onset NMOSD using the International Painel of NMO Diagnosis (IPND) updated in 2015.
Method: We assessed all patients enrolled in our neuroimmunology service from January 2005 to April 2017 whose NMOSD symptoms began before 18 years-old and met the updated 2015-IPND diagnostic criteria.
Results: from 375 patients with NMOSD, 16 were pediatric cases (4,2%). There were 2/16 males, 4/16 caucasian and 12/16 of mixed ethnic background. Three patients had previous infectious disease. Two patients had other autoimmune conditions. Median age at onset was 11(6-18) years. Presenting symptoms were optic neuritis (9), myelitis (4) and acute brainstem syndrome (3). Median time to the second relapse was 6(1-36) months. Median annualized relapse rate was 1relapse/year. The main relapse syndromes were: optic neuritis (45), myelitis (36), brainstem (5), area postrema (2) and diencephalic (1). Eleven (68,8%) patients were AQP4-IgG positive. Thirteen patients had abnormalities in the cerebrospinal fluid but none had positive oligoclonal bands. MRI findings were compatible with NMOSD in all patients. No patient full field Mcdonald-2010 Multiple Sclerosis criteria. All patients were acutely treated with methylprednisolone, followed by plasmapheresis (8), cyclophosphamide (6) and immunoglobulin (3). The median relapse number prior to maintenance treatment introduction was 2(1-6). Patients were first treated with azathioprine (14) or mycophenolate (2). Five patients switched azathioprine to mycophenolate (4) or rituximab (1), due to relapses. Median EDSS of 3.5(1-8) after 5(1-17) years of follow-up. Eleven patients had visual acuity less than 20/200 in the worst eye.
Conclusion: Our results confirm literature emphasizing optic neuritis as mainly inaugural presentation in pediatric NMOSD. This is the first long term follow up of pediatric NMOSD using 2015-IPND criteria showing a higher disability than previous report.
Disclosure:
Renata Barbosa Paolilo: nothing to disclose.
José Albino da Paz: nothing to disclose.
Samira Luisa Apostolos Pereira: received grants related to congress meetings from Genzyme and Roche.
Carolina de Medeiros Rimkus: nothing to disclose.
Ana Luiza Camara Araujo: nothing to disclose.
Lais Maria Gomes de Brito Ventura: nothing to disclose.
Milena Sales Pitombeira: nothing to disclose.
Ana Beatriz Gomes: nothing to disclose.
Aline de Moura Brasil Matos: nothing to disclose.
Pedro Henrique Bruel Torretta: nothing to disclose.
Umbertina Conti Reed: nothing to disclose.
Dagoberto Callegaro: received grants related to congress meetings from Genzyme and Roche.
Douglas Kazutoshi Sato: CAPES/brasil, Japan Society of Science. Speaker for Merck, Shire, TEVA.