RESUMO
We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.
Assuntos
Hormônio Adrenocorticotrópico/uso terapêutico , Ataxia/tratamento farmacológico , Mioclonia/tratamento farmacológico , Neuroblastoma/cirurgia , Transtornos da Motilidade Ocular/tratamento farmacológico , Neoplasias Abdominais/complicações , Neoplasias Abdominais/patologia , Neoplasias Abdominais/cirurgia , Neoplasias Abdominais/urina , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/urina , Ataxia/complicações , Ataxia/metabolismo , Biomarcadores/urina , Pré-Escolar , Deficiências do Desenvolvimento/etiologia , Feminino , Seguimentos , Neoplasias de Cabeça e Pescoço/complicações , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias de Cabeça e Pescoço/urina , Ácido Homovanílico/urina , Humanos , Lactente , Masculino , Mioclonia/complicações , Mioclonia/metabolismo , Neuroblastoma/complicações , Neuroblastoma/patologia , Neuroblastoma/urina , Transtornos da Motilidade Ocular/complicações , Transtornos da Motilidade Ocular/metabolismo , Proteínas Proto-Oncogênicas c-myc/metabolismo , Recidiva , Neoplasias Torácicas/complicações , Neoplasias Torácicas/patologia , Neoplasias Torácicas/cirurgia , Neoplasias Torácicas/urina , Fatores de Tempo , Resultado do Tratamento , Ácido Vanilmandélico/urinaRESUMO
Inherited myoclonus in Poll Hereford calves and spasticity in the spastic mouse (spa/spa) are characterized by myoclonic jerks of the skeletal musculature which occur spontaneously and in response to sensory stimuli, symptoms resembling those in subconvulsive strychnine poisoning. The primary, biochemical defect in these myoclonic animals is a deficit of inhibitory glycine receptors in the central nervous system. We now report the occurrence of similar stimulus-induced myoclonus in individual, pure-bred Peruvian Paso horses and an associated, specific deficiency in the density of [3H]strychnine binding to inhibitory glycine receptors sites in spinal cord of these animals. Specificity of the deficit was confirmed by a demonstrated lack of change in the density of several other receptor types in affected spinal cord, including muscarinic receptors and GABAA/benzodiazepine receptors. In light of the existence of genetically-inherited myoclonus in other species, these results suggest the occurrence of an equine form of the disorder.