Opsoclonus-myoclonus-ataxia syndrome in children.
J Neurol
; 269(2): 750-757, 2022 Feb.
Article
en En
| MEDLINE
| ID: mdl-33779841
ABSTRACT
Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Trastornos de la Motilidad Ocular
/
Síndrome de Opsoclonía-Mioclonía
/
Neuroblastoma
Tipo de estudio:
Prognostic_studies
Límite:
Humans
/
Infant
Idioma:
En
Revista:
J Neurol
Año:
2022
Tipo del documento:
Article
País de afiliación:
Estados Unidos