[West's syndrome. A clinical, therapeutic and prognostic study. Apropos of 66 cases].
The charts of 66 children suffering from West syndrome followed by the paediatric department of the University Central Hospital, Lille, between 1957 and 1984 were reviewed: 24 cases were followed for more than 10 years; 20 cases from 5 to 10 years; 22 cases from 1 to 5 years. Age at onset of the disease ranged from 3 to 5 months (37.8%) and 6 to 8 months (30.3%) with a slight male predominance. Prenatal causes were the most important (42.4%) with anomalies and cerebral malformations in 15.2% and neurocutaneous syndromes in 9.1% of cases. Etiology remained unknown in 27.3% of cases. Some clinical aspects were characteristic (flexion or extension spasms, psychomotor retardation) in 84.8% of cases. Hyparrhythmia occurred in 62.1% of cases. There were no significant differences in the results of the varying therapies used (ACTH, Synacten, hydrocortisone) or its associations. The best results were obtained with a treatment started as early as possible in the cryptogenic forms and in older infants (6 to 8 months).
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D002675:Child, Preschool; D003366:Cosyntropin / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007223:Infant; D008297:Male; D011379:Prognosis; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology
D000927:Anticonvulsants; D003366:Cosyntropin; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone
Le syndrome de West. Etude clinique, thérapeutique et pronostique. A propos de 66 observations.