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Summary: Purpose: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. Methods: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. Results: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic–clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of ≥1 year after 5 years. Conclusions: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this... [ABSTRACT FROM AUTHOR]

Pompe disease is a metabolic myopathy caused by deficiency of lysosomal acid α-glucosidase. In this report we review the first 36 weeks of a clinical study on the safety and efficacy of enzyme therapy aimed at correcting the deficiency. Four patients with infantile Pompe disease were enrolled. They received recombinant human α-glucosidase from transgenic rabbit milk. The product is generally well tolerated and reaches the primary target tissues. Normalization of α-glucosidase activity in skeletal muscle was obtained and degradation of PAS-positive material was seen in tissue sections. The clinical condition of all patients improved. The effect on heart was most significant, with an impressive reduction of the left ventricular mass index (LVMI). Motor function improved. The positive preliminary results stimulate continuation and extension of efforts towards the realization of enzyme therapy for Pompe disease. [ABSTRACT FROM AUTHOR]

Which factors explain intra- and inter-country variations in levels of public support for national health care systems within the European Union, and why? We propose that public opinion towards public health care is dependent on (1) the type of welfare state regime to which the various European welfare states belong, (2) typical features of the national care system and (3) individual social and demographic characteristics, which are related to self-interest or morality oriented motives. To assess the explanatory power of these factors, data from the Eurobarometer survey series are analysed. Support for public health care appears to be particularly positively related to social-democratic attributes of welfare states, whereas support drops with increasing degrees of liberalism and conservatism. Further, support for public health care proves to be associated with wider coverage and public funding of national care services. We also find higher levels of support in countries with scarce social services for children and the elderly, and larger proportions of female (part-time) employment. Lastly, with respect to individual characteristics, we find remarkably little evidence for self-interest oriented motives affecting the preference for solidary health care arrangements. [ABSTRACT FROM AUTHOR]

In a prospectively studied group of 43 children with centro-temporal spikes on the EEG, only 26 had the classical epileptic syndrome related to this EEG abnormality. Ten patients never had epileptic manifestations. In the other seven cases two types of epileptiform activity were encountered: in addition to the centro-temporal spikes generalized spike-and-wave complexes or--in other patients--occipital epileptiform phenomena occurred. In these cases the clinical signs and symptoms correlated best with the non-Rolandic EEG abnormalities. Some patients had only a few centro-temporal spikes during the recording of the EEG; in others this epileptiform activity was almost continuously present. This quantitative difference had no clinical correlation. Centro-temporal spikes should probably be looked upon as an (epi)phenomenon of cerebral disfunction with a wide range of clinical and electroencephalographic expressions.