Neuromyelitis Optica With Onset in Childhood and Adolescence

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• 作者：Yara Dadalti Fragoso ; Maria L.B. Ferreira ; Enedina M.L. Oliveira ; Renan B. Domingues ; Taysa A.G.J. Ribeiro ; Joseph B.B. Brooks ; Rinaldo Claudino ; Jussara M.K. Netto ; Sidney Gomes ; Tarso Adoni ; Denise S.D. Carneiro ; Daiana R.P. Fonseca ; Manuela O. Fragomeni ; Francisco T.M. Oliveira ; Celso L.S. Oliveira ; Patricia C.O. Saldanha ; Jorge M.B. Souza
• 关键词：neuromyelitis optica ; Devic ; children ; adolescents ; demyelinating diseases ; multiple sclerosis
• 刊名：Pediatric Neurology
• 出版年：January, 2014
• 年：2014
• 卷：50
• 期：1
• 页码：66-68
• 全文大小：148 K

文摘

Background

Neuromyelitis optica with onset before the age of 18 years is a relatively rare, yet potentially devastating condition. The objective of the present study was to contribute to the study of early-onset neuromyelitis optica with a case series.

Patients

Data were collected from medical records of Brazilian neurologists caring for patients with neuromyelitis optica occurring in childhood and adolescence.

Results

Twenty-nine patients with neuromyelitis optica occurring before the age of 18 years and fulfilling the diagnostic criteria were identified. The average age at disease onset was 13 years and the patients had had an average disease duration of 6 years. The expanded disability scale score at the latest consultation was, on average, 4.7, and one patient had died from the disease. The 29 patients had had an average 4.5 relapses during the disease, accounting for 0.75 relapses per year, irrespective of the medication used. All patients were using one or more of the following medications: azathioprine, prednisone, immunoglobulin, and glatiramer acetate.

Conclusions

Neuromyelitis optica with onset in childhood and adolescence is a poorly understood condition that is often disabling and difficult to manage.