RESUMO
Intermittent rhythmic delta activity is reported in various disorders and is classified as a nonspecific abnormal electroencephalographic pattern. We have investigated its clinical and electroencephalographic features in childhood. Intermittent rhythmic delta activity was identified in 54 children over a period of 48 months. Epilepsy was present in 81%, 4% had only a single generalized tonic-clonic seizure, and 15% had no seizures. Generalized seizures were more common than partial seizures (83% versus 13%; 4% were mixed). The largest group of patients had idiopathic epilepsy. Epileptiform features were present in 70%. No patient identified prospectively has had a space-occupying lesion. Intermittent rhythmic delta activity should be considered an epileptiform pattern in children, most commonly occurring as an interictal pattern in primary generalized epilepsy.
Assuntos
Ritmo Delta , Eletroencefalografia , Epilepsia/fisiopatologia , Adolescente , Córtex Cerebral/fisiopatologia , Criança , Pré-Escolar , Epilepsias Mioclônicas/diagnóstico , Epilepsias Mioclônicas/etiologia , Epilepsias Mioclônicas/fisiopatologia , Epilepsias Parciais/classificação , Epilepsias Parciais/etiologia , Epilepsias Parciais/fisiopatologia , Epilepsia/classificação , Epilepsia/etiologia , Epilepsia Tipo Ausência/classificação , Epilepsia Tipo Ausência/etiologia , Epilepsia Tipo Ausência/fisiopatologia , Epilepsia Generalizada/classificação , Epilepsia Generalizada/etiologia , Epilepsia Generalizada/fisiopatologia , Epilepsia Tônico-Clônica/classificação , Epilepsia Tônico-Clônica/etiologia , Epilepsia Tônico-Clônica/fisiopatologia , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
Four children with the classic form of maple syrup urine disease (MSUD) died of cerebral edema during an intercurrent infection that caused severe dehydration and acidosis. The diagnosis of MSUD had been established during the neonatal period in all four patients, on day 1 of life in three of them. All were in satisfactory control before the intercurrent illness. Two patients underwent peritoneal dialysis. Signs of brain-stem compression occurred after treatment, when biochemical abnormalities were improving. Computed tomography of the head, which was done in two patients, revealed cerebral edema; one of these patients also had subarachnoid hemorrhage. Autopsy in one case revealed cerebral edema with herniation. Our experience documents that cerebral edema may occur in the older child with MSUD as well as in the neonate. The pathogenesis of cerebral edema in MSUD remains unclear. Early treatment of dehydration and acidosis may prevent the catastrophic consequences that we have observed.