Open AccessSíndrome de opsoclonus mioclonus secundario a neuroblastoma abdominal. Presentación de un caso clínico pediátrico
Author(s) -
Gabriela Urman,
Maia Leivi,
Noelia Chamorro,
Brenda García,
Adrián Callea,
Arnoldo Grosman
Publication year - 2019
Publication title -
archivos argentinos de pediatría
Language(s) - Spanish
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2019.e651
Subject(s) - medicine , opsoclonus , neuroblastoma , irritability , ataxia , pediatrics , surgery , gynecology , psychiatry , cell culture , genetics , menopause , biology
Opsoclonus-myoclonus syndrome is a rare disorder among pediatric patients. The diagnosis is clinical and is characterized by the presence of at least three of the following: opsoclonus, myoclonus, ataxia, irritability and sleep disorders. In over 50 % of cases it is associated with the presence of Neuroblastoma. It is a disorder of immune origin and its treatment is based on immunosuppressants, immunomodulators and tumor resection in cases secondary to Neuroblastoma. Up to 70 % to 80 % of cases may present neurological sequelae, depending on the cause, the initial severity of symptoms and the delay of proper treatment. We present the case of a 2-year-old male with diagnosis of opsoclonus-myoclonus syndrome secondary to a left adrenal Neuroblastoma. Tumor resection and treatment with corticosteroids, immunoglobulin and rituximab were performed.