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Carbamate and organophosphate pesticides are widely used all over the world. Poisoning with these substances may produce both immediate and delayed neurotoxic effects. We report the case of a 4-year-old boy who was admitted to the Pediatric Department of the Second University of Naples for evaluation of stupor, lethargy, severe hypotonia, generalized weakness of his arms and legs, ataxia, dysmetria, miosis, excessive salivation and tearing. The pesticide carbaryl (1-naphthyl-N-methylcarbamate) was identified in blood and urine samples. On admission, magnetic resonance imaging (MRI) was unremarkable; on day 11, MRI showed central pontine myelinolysis. The demyelination improved after 4 months and disappeared after 2 years. Various underlying and concomitant diseases have been described in children with central pontine myelinolysis but, to our knowledge, the finding of pontine myelinolysis after carbamate poisoning has not yet been described.

Background: Ventricular fibrillation can be the presenting arrhythmia in children with asymptomatic Wolff-Parkinson-White syndrome. Deaths due to this arrhythmia are potentially preventable., Methods: We performed a randomized study in which prophylactic radiofrequency catheter ablation of accessory pathways was compared with no ablation in asymptomatic children (age range, 5 to 12 years) with the Wolff-Parkinson-White syndrome who were at high risk for arrhythmias. The primary end point was the occurrence of arrhythmic events during follow-up., Results: Of the 165 eligible children, 60 were determined to be at high risk for arrhythmias. After randomization, but before any ablation had been performed, the parents withdrew 13 children from the study. Of the remaining children, 20 underwent prophylactic ablation and 27 had no treatment. The characteristics of the two groups were similar. There were three ablation-related complications, one of which led to hospitalization. During follow-up, 1 child in the ablation group (5 percent) and 12 in the control group (44 percent) had arrhythmic events. Two children in the control group had ventricular fibrillation, and one died suddenly. The cumulative rate of arrhythmic events was lower among children at high risk who underwent ablation than among those at high risk who did not. The reduction in risk associated with ablation remained significant after adjustment in a Cox regression analysis. In both the ablation and the control groups, the independent predictors of arrhythmic events were the absence of prophylactic ablation and the presence of multiple accessory pathways., Conclusions: In asymptomatic, high-risk children with the Wolff-Parkinson-White syndrome, prophylactic catheter ablation performed by an experienced operator reduces the risk of life-threatening arrhythmias., (Copyright 2004 Massachusetts Medical Society)

FG syndrome (FGS, MIM 305450) is a rare X-linked recessive disorder comprising mental retardation and multiple malformations. Various families have been described to date, increasing our knowledge of the phenotype variability and making the clinical diagnosis complex, especially in sporadic patients. The first locus for FG syndrome (FGS1) was linked to chromosome region Xq12-q21.31, but other families have been excluded from this locus. The genetic heterogeneity of FG syndrome has been confirmed by analysis of an X chromosome inversion [inv(X)(q11q28)] in an affected boy and in his mentally retarded maternal uncle, suggesting that an additional locus for FG syndrome (FGS2, MIM 300321) is located at either Xq11 or Xq28. Recently, a third locus (FGS3) has been mapped to Xp22.3. We have identified and clinically characterized an Italian FG family, including 31 members with three affected males in two generations and two obligate carriers. We have excluded linkage to known FGS loci, whereas an extensive study of the whole X chromosome has yielded a maximum LOD score (Z(max)) of 2.66 (recombination fraction=0) for markers between DXS8113 and sWXD805. This new locus for FG syndrome corresponds to a region of approximately 4.6 Mb on the X chromosome.