Kindling of motor seizures from the bed nucleus of the stria terminalis.

Intermittent application of localized electrical stimulation to certain regions of the brain results in the development of generalized seizures (kindling effect); of all those regions the limbic system, and particularly the amygdala, is highly susceptible to this phenomenon. The kindling rate for different regions of the limbic system seems to be related to the extent of their anatomic connections with the amygdala. The present report deals with the kindling susceptibility of the bed nucleus of the stria terminalis (BST), a structure which has substantial direct connections with the amygdala and which has never been tested for this purpose. The BST was found to be among the structures having the highest susceptibility to kindling, just after the amygdala. Kindling of the BST was very similar to that in the amygdala but it did not result from an indirect activation of this last structure because we demonstrated that bilateral amygdalectomy did not prevent its development nor modify its expression. Several fiber tracts such as the stria terminalis and the anterior commissure surround or traverse the BST. We confirmed that motor seizures developed more rapidly when these fibers, rather than either the BST or the amygdala itself, were stimulated.     

Tissue catecholamines and daily rhythm of liver microsomal enzyme activity

A daily rhythm of liver microsomal enzyme activity and plasma corticosterone levels with a minimum during the light period and a maximum in darkness was found in rats. Treatment with α-methyl-tyrosine consistently increases the plasma corticosterone concentrations and abolishes the fluctuations of microsomal enzyme activity. Intraventricular or intravenous injection of 6-OH-dopamine does not modify the fluctuation of the corticosterone levels nor the enzymatic activity of the microsomes at the different times of the day. The relationship between the brain catecholamine availability and the daily rhythm of drug metabolism is discussed.     

Pharmacokinetics of injectable phenobarbital in the premature infant. Study of a new lyophilized form

A lyophilized preparation of phenobarbital was studied in newborns without cerebral palsy. Plasma levels were determined using gas chromatograph fitted with thermo ionic probe after either an intra-muscular (IM) injection in premature infants or an intravenous (IV) injection over single dose of phenobarbital 10 mg/kg within 6 hours after birth. Five term babies were included in the study as controls and received an IM injection. The results showed rapid increase in plasma concentration after IM injection in 10 of 13 subjects with a peak concentration reached 60 minutes after injection. The mean ratio (maximal concentration/dose) was 1.25 and 1.10 for term infants and preterm infants respectively. In all cases, the drug was well tolerated. In 15 preterm infants (n: 7 IM and n: 8 IV) the plasma concentrations were followed over a period of 15 days. The disappearance curve was biphasic; it varied the first 7 days, then remained constant for the following week (apparent half life 106 hours).     

High grade glioblastoma is associated with aberrant expression of ZFP57, a protein involved in gene imprinting,and of CPT1A and CPT1C that regulate fatty acid metabolism

The diagnosis of glioblastoma is still based on tumor histology, but emerging molecular diagnosis is becoming an important part of glioblastoma classification. Besides the well-known cell cycle-related circuitries that are associated with glioblastoma onset and development, new insights may be derived by looking at pathways involved in regulation of epigenetic phenomena and cellular metabolism, which may both be highly deregulated in cancer cells. We evaluated if in glioblastoma patients the high grade of malignancy could be associated with aberrant expression of some genes involved in regulation of epigenetic phenomena and lipid metabolism. We measured the mRNA levels of ZFP57, TRIM28, CPT1A, CPT1B, and CPT1C in a cohort of 80 patients divided in two groups: grade II and grade IV. We evidenced that high grade glioblastoma is associated with increased level of ZFP57, a protein involved in gene imprinting, and aberrant expression of CPT1A and CPT1C, regulators of fatty acid oxidation. Our study may pave the way to identify new markers that could be potentially useful for diagnosis and/or prognosis of glioblastoma.     

Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.     

Functional hypersomatotropism in small for gestational age (SGA) newborn infants

Basal plasma GH levels and the GH responses to an injection of 1 microgram/kg 1-44(NH2) GHRH were determined on day 3 postnatally in 5 small gestational age (SGA) twin newborns and their appropriate gestational age (AGA) co-twins, and in 10 SGA singleton newborns and 6 AGA singleton newborns. The mean basal plasma GH level was higher in the SGA than in the AGA infants but the difference was significant only for singleton newborns (p less than 0.01). The mean peak plasma GH level was markedly increased in SGA compared to AGA infants (p less than 0.05 for twins, p less than 0.01 for singletons). Twelve SGA infants re-tested at 1 month had lower basal and peak plasma GH levels (p less than 0.001 and p less than 0.01). In 21 SGA and 17 AGA infants, serum IGF-I, measured by RIA between 12 and 96 hours after birth, was significantly higher in SGA than in AGA (p less than 0.001). These results suggest that, whatever the mechanism, functional hypersomatotropism is present at day 3 in SGA infants. This hypersomatotropism may participate in the early catch-up growth process.