RESUMO
We report 22 cases of alternating hemiplegia of childhood. In addition to repeated episodes of hemiplegia lasting from a few minutes to several days, the disease was characterized by an onset before 18 months of age, the occurrence of tonic or dystonic attacks, nystagmus, dyspnea and other autonomic phenomena, and the development of cognitive impairment and of a choreoathetotic movement disorder. All the patients also had episodes of quadriplegia that occurred either when a hemiplegia was shifting from one side to the other or as an isolated manifestation. Such episodes were often severe and followed by developmental deterioration. In all children, sleep consistently relieved both weakness and associated paroxysmal phenomena, but these would reappear 10 to 20 minutes after the children awakened, during long-lasting episodes. Although six patients also had epileptic seizures, the condition seems to be distinct from epilepsy, and the clinical features and poor outcome differentiate it from migraine. Treatment with the calcium-entry blocker flunarizine was partially effective.
Assuntos
Hemiplegia/fisiopatologia , Diagnóstico Diferencial , Feminino , Hemiplegia/diagnóstico , Humanos , Lactente , Recém-Nascido , MasculinoRESUMO
We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.
Assuntos
Transtornos Cognitivos/diagnóstico , Coma/diagnóstico , Transtornos dos Movimentos/diagnóstico , Doença Aguda , Encéfalo/diagnóstico por imagem , Edema Encefálico/diagnóstico , Edema Encefálico/etiologia , Edema Encefálico/metabolismo , Criança , Pré-Escolar , Transtornos Cognitivos/etiologia , Transtornos Cognitivos/metabolismo , Coma/etiologia , Coma/metabolismo , Eletroencefalografia , Humanos , Masculino , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/metabolismo , Testes Neuropsicológicos , Prognóstico , Convulsões/diagnóstico , Convulsões/etiologia , Convulsões/metabolismo , Síndrome , Tomografia Computadorizada por Raios XAssuntos
Diazepam/administração & dosagem , Diazepam/sangue , Criança , Pré-Escolar , Humanos , Lactente , RetoRESUMO
Blood concentrations of phenobarbital, following an intramuscular administration of a single large dose of approximately 10 mg/kg, were studied in 39 infants. A rapid rise was obtained with a mean concentration of 9.30 mug/ml at 30 minutes, 12.76 mug/ml at 90 minutes, and a mean peak concentration of 13.28 mug/ml, which was reached in most cases within 2 hours of injection or less. Cerebrospinal fluid values in 13 patients averaged half the blood phenobarbital concentrations.