Dados do Trabalho
Título
OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME: A PEDIATRIC ONCOLOGY HOSPITAL EXPERIENCE
Introdução
Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare, immune-mediated neurological disorder that usually starts in the second year of life. The triad of signs is composed of opsoclonus, myoclonus and ataxia. In addition, there is often irritability and sleep disturbance. In about 50% of children there is an underlying neuroblastoma.
Objetivo
The aim of study was to investigate and describe the epidemiology, clinical features, tumor association, treatment profile and outcome of patients with OMAS.
Métodos
We conducted a retrospective study over 17 years (2005–2022) including all patients aged under 18 years who were managed for OMAS in an oncologic hospital (GRAACC Hospital in São Paulo – SP). Epidemiological and clinical data were analyzed.
Resultados
Eleven patients were included. The male–female ratio was 1:4,5. Median age of onset was 2.15 years (25.8 months). Time to diagnosis ranged between 10 days and 3 years. All patients had ataxia, tremor, dysmetria and irritability at some point. Acute ataxia was the predominant initial symptom, corresponding to 81% of the cases. Opsoclonus was the initial symptom in only 9% of cases. Eighty two percent of the patients had brain magnetic resonance imaging. Eighty one percent realized cerebrospinal fluid analysis. Most patients had association with tumor (72%), with neuroblastoma and ganglioneuroblastoma corresponding to half of the cases each. Time to diagnosis among OMAS and tumor ranged from 0 days to 1 year and 7 months, but the majority (63%) were diagnosed at the same time. Only one patient did not resect the tumor. All patients received immunomodulatory treatment, and 62% received combination therapy (immunoglobulin plus dexamethasone, or immunoglobulin plus methylprednisolone, or immunoglobulin plus prednisolone, or immunoglobulin plus dexamethasone and rituximab). Comparing the “tumor group” and the “no tumor group”, there were no differences in sex ratio and the main presenting symptom. Children in the tumor group had an earlier age of onset (mean 19.1 vs. 25.8 months). Of the total, there was relapse in 36% and 63% have sequelae, with language and cognition as the most affected areas. The percentage of sequelae was higher in the “Group of tumors” (75% vs 33%).
Conclusões
OMAS is a rare neurologic condition that can be associated with poor cognitive outcomes. An early diagnosis with aggressive immunomodulation might lead to a better outcome. The disorder requires careful monitoring and longer-term follow-up.
Palavras chave
Opsoclonus-myoclonus-ataxia syndrome, neuroblastoma
Referências (se houver)
1. Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children. An International Perspective. Thomas Rossor, E. Ann Yeh, Yasmin Khakoo, Paola Angelini, Cheryl Hemingway, Sarosh R. Irani, Gudrun Schleiermacher, Paramala Santosh, Tim Lotze, Russell C. Dale, Kumaran Deiva, Barbara Hero, Andrea Klein, Pedro de Alarcon, Mark P. Gorman, Wendy G. Mitchell, Ming Lim. Neurol Neuroimmunol Neuroinflamm May 2022, 9 (3) e1153; DOI: 10.1212/NXI.0000000000001153
2. Pranzatelli MR, Tate ED, McGee NR. Demographic, clinical, and immunologic features of 389 children with opsoclonus-myoclonus syndrome: a cross-sectional study. Front Neurol 2017;8(September):153-158.
3. Ki Pang K, de Sousa C, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol 2010;14(2):156-161.
4. Hasegawa S, Matsushige T, Kajimoto M, et al. A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children. Brain Dev 2015;37(7):656-660.
5. Pranzatelli MR, Tate ED, McGee NR. Multifactorial analysis of opsoclonus-myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children. Pediatr Blood Cancer 2018;65:e27097.
6 Catañon-González A, Barragán-Pérez E, Hernández-Pliego G, López-Valdés JC. Immunosuppressive therapy in opsoclonus-myoclonus-ataxia syndrome associated with paravertebral neuroblastoma. Neurología. 2019.
7. Ben Achour N et al. Childhood opsoclonus–myoclonus syndrome: A case series from Tunisia. Brain Dev (2017), http://dx.doi.org/10.1016/j.braindev.2017.05.001
8. Muthusamy K, Thomas M, Yoganathan S, Sudhakar SV. Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India. Ann Indian Acad Neurol. 2019 Jul-Sep;22(3):295-301. doi: 10.4103/aian.AIAN_101_18. PMID: 31359941; PMCID: PMC6613420.
9. Jiménez Legido M, Extremera VC, de la Concepción Fournier Del Castillo M, Llorente JM, Gutiérrez-Solana LG. Síndrome opsoclonus mioclonus. experiencia en los últimos 12 anos ˜ en un hospital terciario. An Pediatr (Barc). 2020;93:339---342.
10. Huddar A, Bindu PS, Nagappa M, Bharath RD, Sinha S, Mathuranath PS, Taly AB. Pediatric opsoclonus-myoclonus-ataxia syndrome: Experience from a tertiary care university hospital. Neurol India 2018;66:1332-7
Declaração de conflito de interesses de TODOS os autores
Não
Área
Neoplasias
Instituições
UNIFESP - São Paulo - Brasil
Autores
Lorena Raulik Cyrino, Ricardo Silva Pinho, Marcelo Melo Aragão, Caroline Corrêa Maranhão, Jose Marcos Vieira Albuquerque Filho, Katrine Freitas Valeriano, Mateus Oliveira Torres, Alulin Tacio Quadros Monteiro Fonseca