The Aicardi-Goutières syndrome: Variable clinical expression in two siblings

We report 2 siblings with the Aicardi-Goutières syndrome (encephalopathy, basal ganglia calcifications, and persistent cerebrospinal fluid pleiocytosis). The eldest sibling is severely retarded; his younger brother has only mild, slowly progressive neurological deficits. To our knowledge, such a striking difference in clinical expression has not been reported previously.     

Dental caries in 5-, 7-, 9- and 11-year-old schoolchildren during a 9-year dental health campaign in The Hague

Abstract In the autumn of 1969, 1972, 1975 and 1978 clinical and radiographic dental examinations were carried out on about 800 children in The Hague of kindergartens, and 2nd, 4th and 6th grades of elementary schools. The aim of this investigation was to assess what changes, if any, in dental health may have taken place as a result of the campaign ‘Haagse Aktie’, based on dietary and dental health education. In the 5-, 7- and 9-year-old children no differences in dental health were found between 1969 and 1972. The first considerable improvement was found in 1975, especially in children from high social levels. In 1978 the children showed an even better dental health, compared with 1975. In the 5-year-old children the average dsmfs was 6.8. The average D3MFS in children of 7, 9 and 11 years of age in 1978 was 1.8, 3.9 and 8.2. At all socioeconomic levels the improvement of dental health was due to a considerable increase in the percentage of caries free children between 1975 and 1978; 27.6%, 51.8%, 21.2% and 7.4%, respectively, of the 5-, 7-, 9- and 11-year-old children were caries free in 1978, compared with 10.0%, 14.2%, 3.1 % and 0.9% in 1975. However, the reason for the reduction in caries is not known: it can only be the subject of speculation.