RESUMO
We report the long-term outcome of 64 infants with infantile spasms, followed prospectively, using controlled treatment schedules and objective techniques (24-hour EEG and video monitoring) to determine response. Average age at follow-up was 50 months. Of the 64 infants, three (5%) died; of the others, 41 (67%) had developmental retardation of 50% or more or an IQ of 50 or less. Eight patients (13%) composed our cryptogenic study group and were so classified on the basis of normal CT scan, normal development prior to onset of infantile spasms, and undetermined cause. These patients had the better outcome; 38% had normal development or were only mildly retarded. Both the responders and nonresponders in our symptomatic group had a poor outcome; only 5% had normal development or mild impairment. Outcome was not significantly influenced by short versus long treatment lag or by response to therapy. Other types of seizures occurred in 34 patients (53%). In summary, the overall prognosis for long-term outcome in these 64 patients with infantile spasms was poor.
Assuntos
Hormônio Adrenocorticotrópico/uso terapêutico , Prednisona/uso terapêutico , Espasmos Infantis/tratamento farmacológico , Pré-Escolar , Deficiências do Desenvolvimento/etiologia , Eletroencefalografia , Humanos , Lactente , Deficiência Intelectual/etiologia , Monitorização Fisiológica , Prognóstico , Estudos Prospectivos , Espasmos Infantis/complicaçõesRESUMO
Twenty-four patients with infantile spasms were entered in a double-blind, placebo-controlled, crossover study to compare the therapeutic effectiveness of ACTH (20 to 30 units/day) with that of prednisone (2 mg/kg/day). Response to therapy was determined by utilizing a comprehensive monitoring system and was defined as a complete cessation of spasms and disappearance of the hypsarrhythmic EEG pattern. A major difference between the effectiveness of ACTH and that of prednisone in stopping the spasms and improving the EEG pattern was not demonstrated. Nine patients responded to ACTH (five initial drug, four crossover), and seven patients responded to prednisone (four initial drug, three crossover). Twelve responded within two weeks of initiation of therapy, and four within six weeks. Therapy was tapered and discontinued immediately after a response was obtained. Five patients had a relapse; four responded rapidly to a second course of therapy. Of the eight patients who failed to respond to hormonal therapy, seven were given clonazepam with no improvement.
Assuntos
Hormônio Adrenocorticotrópico/uso terapêutico , Prednisona/uso terapêutico , Espasmos Infantis/tratamento farmacológico , Ensaios Clínicos como Assunto , Método Duplo-Cego , Eletroencefalografia , Humanos , Hidrocortisona/sangue , Lactente , Espasmos Infantis/diagnóstico , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
The neurologic course of congenital rubella syndrome was traced in 29 nonretarded children to 9 to 12 years. During the first two years, manifestations involved abnormal tone and reflexes (69%), motor delays (66%), feeding difficulties (48%), and abnormal clinical behavior (45%). Hearing loss was documented in 76%. From three to seven years, poor balance, motor incoordination (69%), and behavioral disturbances (66%) predominated. Hearing losses increased to 86%. Currently, at 9 to 12 years, 25 have residua which include learning deficits (52%), behavioral disturbances (48%), poor balance (61%), muscle weakness (54%), and deficits in tactile perception (41%). Two additional children now have hearing loss. The encephalitic manifestations of congenital rubella syndrome are diverse. Overriding problems differ at each phase of childhood. Current deficits influence progress in educational and home environments. For these children, adequate intelligence alone does not guarantee academic success.