Síndrome de opsoclonus-mioclonus.docx

psoclonus-mioclonus syndrome
Hugo a. Arroyo, Natalia Tringler, Cecilia de los Santos
Neurology service, hospital of Pediatrics John p. Garrahan, Buenos Aires
Postal address: Dr. Hugo a. Arroyo, Neurology, Pediatrics John p. Garrahan Hospital, service battle of the wells 1881, 1245 Buenos Aires, Argentina Fax: (54-11) 49436116 email: hugoarroyo@arnet.com.ar
Summary
Syndrome opsoclonus -mioclonus is a rare, children characterized by: irritability, chaotic eye movements with vertical, horizontal components, Rotary (opsoclonus), mioclonus, and ataxia. A high percentage of cases associated with neuroblastoma although other etiologies are also acknowledged (infecciosa-parainfecciosa, toxic). An autoimmune mechanism is responsible for the dysfunction of structures in the brain stem andcerebellum, explained some of the cardinal symptoms opsoclonus-mioclonus (ataxia). However the commitment encefalopáticos signs and the high percentage of patients with neurocognitive consequences and psychiatric speak in favour of a broader dysfunction. Treatment with steroids, ACTH and inmunomoduladoras and immunosuppressive drugs is used, but is prospective studies with uniform therapeuticprotocols necessary to define whether the long-term use of these drugs favourably influence the evolution in this patient group.
Palabras claves: Opsoclonus; Mioclonus; Ataxia; Movimientos oculares sacádicos; Neuroblastoma
Abstract
Opsoclonus-myoclonus syndrome. The opsoclonus-myoclonus syndrome in children is a rare entity which is characterized by irritability, chaotic ocular movements withvertical, horizontal, rotatory components (opsoclonus) along with myoclonus and ataxia.In a high proportion of cases, it is associated with neuroblastoma although other etiologies involving infectious or toxic agents have been reported. An autoimmune mechanism would be responsible for the dysfunction of structures in brain stem and cerebellum thus explaining some of the cardinal symptoms such asopsoclonus, myoclonus and ataxia. However, encephalopathic symptoms and the high percentage of patients with neurocognitive and psychiatric sequels are in favor of a wider dysfunction. Treatment with steroids, ACTH, immunomodulatory or immunosuppressive drugs is being used although prospective studies are needed to determine whether the prolonged use of these drugs influences favorably the evolution ofthese patients.
Key words: Opsoclonus; Myoclonus; ataxia; Saccadic eye movements; Neuroblastoma
The syndrome opsoclonus-mioclonus (SOM) is a rare entity characterized by an abnormal eye movement - opsoclonus-, mioclonías, ataxia, and irritability. The SOM can be the manifestation of a paraneoplastic syndrome (childhood neuroblastoma is frequently detected tumor) but also occurs in association withvarious clinical entities (postinfecciosas, metabolic, toxic) and sometimes without a clear etiology. Also called by chaotic and rapid eye movement "dancyng eye syndrome" dancing eyes syndrome. More than half of the cases is associated, in children with neuroblastoma 1 and symptoms appear before that cancer is clinically evident. In addition, around from 1 to 3% of children with neuroblastoma haveSOM and with this specific Association have a better prognosis and a high rate of survival 2, 3 especially early 4. Most children with SOM show long-term permanent dysfunction of the nervous system with commitment 5 global neruocognitivo.Currently accepts an inmunomediado mechanism and, although still not fully elucidado treatment with steroids and immunosuppressive drugs may improve acutesymptoms 1.
Clinical characteristics - symptoms
The SOM occurs between the first year of life and the three years with an average 18-22 months 5, 6. The clinical presentation is unchanged if associated or not with neuroblastoma. The start is acute or subacute ataxia by limiting or preventing the ability to walk or sit at a generally days. Sleep disturbances are presented in the initial period while...