This is a prospective study designed to evaluate the efficacy and safety of vigabatrin as first-choice monotherapy in infants with West syndrome. One hundred sixteen patients with newly diagnosed West syndrome were studied in Argentina, from June 1994 to April 1998. The follow-up ranged from 17 to 40 months (mean, 23 months). Vigabatrin was administered upon diagnosis, starting with a 50-mg/kg/day dose and increasing 50 mg/kg every 48 hours to reach a maximum dose of 200 mg/kg/day. Twenty-nine percent of cases were considered to be cryptogenic or idiopathic West syndrome, while 70.7% were symptomatic. Response to vigabatrin treatment was measured according to five categories: (1) seizures free: 61.8% of cases for cryptogenic and 29.3% for symptomatic West syndrome, (2) more than 75% reduction in the number of infantile spasms: 14.7% for cryptogenic and 26.8% for symptomatic West syndrome, (3) from 50% to 74% reduction in the number of infantile spasms: 11.8% for cryptogenic and 24.4% for symptomatic West syndrome, (4) poor or null response: 11.8% for cryptogenic and 18.3% for symptomatic West syndrome, and (5) increase in the number of infantile spasms: one symptomatic case (1.2%). All seizure-free cryptogenic cases showed normal neuropsychic development. The most effective dose of vigabatrin was 150 mg/kg of body weight per day. The most frequent adverse events were somnolence in 19 cases and irritability in 15 cases, but none required treatment interruption.

We studied eight children who had measles at 6 to 10 months of age during the 1998 Argentine measles outbreak and in whom subacute sclerosing panencephalitis developed 4 years later. We report the genetic characterization of brain tissue–associated measles virus samples from three patients. Phylogenetic relationships clustered these viruses with the wild-type D6 genotype isolated during the 1998 outbreak. The children received measles vaccine; however, vaccinal strains were not found.

ExtractFatty acid biosynthesis in the developing rat brain was studied. During development, total fatty acid synthesis was maximal a t 15-16 days of age-a time of rapid myelination for this species. Results were expressed as mpmoles of 14C-labeled acetyl-CoA incorporated into fatty acids per milligram of brain mitochondrial or microsomal protein, and as 14C-labeled malonyl-CoA incorporated per milligram of microsomal protein.Elongation of saturated fatty acids by microsomes followed a similar developmental pattern. The peak incorporation of malonyl-CoA into saturated fatty acids occurred a t 15 days of age, while synthesis of polyunsaturated fatty acid did not change significantly with maturation. I n the mitochondrial-acetyl-CoA system, elongation of polyunsaturated fatty acids was also greatest at 15 days of age, while synthesis of saturated and monounsaturated fatty acids was unchanged significantly with maturation. These results support the concept that chain elongation of fatty acids is directly related to myelination. The marked increase in the rate of elongation of saturated fatty acid by the microsomal fraction, occurring a t this time, suggests that this system is involved in synthesis of myelin fatty acids.

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