Dados do Trabalho

Título

OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME ASSOCIATED WITH COXSACKIE INFECTION: A CASE REPORT

Apresentação do caso único

Introduction: Opsoclonus-myoclonus-ataxia syndrome is a rare disease caused by immunomediated reactions, most often due to paraneoplastic syndrome, though it can more rarely be caused by infections.

Case Report: We present a case of a previously healthy 2-year-old infant who developed a febrile illness with vomiting and papulovesicular skin lesions on the hands, feet, mouth, and buttocks, suggesting hand-foot-mouth disease. The next day, the patient exhibited ataxia, sleep disturbances, and irritability, leading to inpatient treatment with fluids and analgesics. A cranial tomography scan was normal, as was a lumbar puncture (glucose 58, protein 79, cells 55: lymphocytes 34%, monocytes 11%, neutrophils 54%). The patient developed myoclonia on the third day and opsoclonus on the fourth, leading to a diagnosis of OMAS. Thoracic, abdominal, and pelvic tomographies were normal. Treatment with methylprednisolone 30 mg/kg and immunoglobulin 2 g/kg was initiated, and prednisolone 1 mg/kg was maintained, resulting in complete symptom improvement. A full-body magnetic resonance imaging scan was performed with normal results, and MIBG scintigraphy was unavailable in the state.

Discussão

Discussion: Opsoclonus-myoclonus-ataxia syndrome is caused in almost 50% of cases by paraneoplastic syndrome, most commonly neuroblastoma. In this case, a typical Coxsackie infection was associated with the disease. Different forms of disease presentation must be considered to the accurate diagnosis of Opsoclonus-myoclonus-ataxia syndrome and possible causes associated with the disease.

Comentários finais

Questioning about previous infections when the Opsoclonus-myoclonus-ataxia syndrome is suspected is relevant to establish the possible cause of the events, in addition to tumoral screening.

Referências

Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children, An International Perspective. Thomas Rossor et al. Neurol Neuroimmunol Neuroinflamm 2022.
Opsoclonus-Myoclonus Syndrome in Children and Adolescents: A Therapeutic Challenge. Children Auconi, M et al. children,2021
Opsoclonus-myoclonus syndrome: Clinical characteristics, therapeutic considerations, and prognostic factors in a Spanish paediatric cohort. V. Cantarín-Extremera et al, Neurología,2020.

Palavras Chave

opsoclonus; neuroimmunology; coxsackie