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Clinical course of paediatric neuromeylitis optica with recurrent clinical course

Introduction: Recurrent NMO is an idiopathic inflammatory demyelinating disease of the CNS that preferentially affects the optical nerve and spinal cord. Only recently some studies reported RNMO in pediatric population. Objective: describe the clinical course of pediatric patients diagnosed with RNMO. Patients and Methods: The medical records of fifteen patients from Hospital da Lagoa (Rio de Janeiro) diagnosed by Wingerchuk et al criteria (2006) whose first event was set before age 18, were reviewed and compared with 80 NMOR cases initiated after 18 years. Results: all fifteen patients had their first event between 7 and 17 years (median: 13,67); the majority was female (86,7%) and Afro-brazilians (60%). The most common initial event was transverse myelitis (40% of cases), followed by bilateral optic neuritis (26,7%). Involvement of brain stem was found in 20% of the cases. From all events, the most common was the spinal cord event, with 42,35%; second place were the optical events (28,83%); third, the sensitive events (16,22%). The spinal cord magnetic resonance (MRI) showed extensive lesions in 13 patients (86,7%) and initial brain MRI was normal in 14 patients (93,3%) Four patients were tested for anti-AQP4, two were positive. The average time of disease was 17, 5 years and the mortality rate was 26,7%.No difference was found between the groups. Conclusions: Recurrent NMO in a pediatric population in Rio de Janeiro is characterized by involvement of optic nerve and spinal cord, predominantly, and trunk in 20% of cases, leaving severe visual and motor sequeale in a period of 10 years of disease. Morbidity and mortality were similar to non/pediatric NMOR patients

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