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Background Aicardi-Goutières syndrome (AGS) is a severe familial, mostly autosomal recessive encephalopathy, first described in 1984. The clinical picture and genetic abnormalities are heterogeneous. US findings in AGS have thus far not been systematically described. Objective The purpose of this study was to analyse sonographic features in AGS and to compare them to CT/MRI. Materials and methods Four male infants with AGS, two brothers, underwent imaging between the ages of 4 weeks and 6 months. Results Sonographically isolated mineralization of lenticulostriate vessels, dilatation of the lateral ventricles, subependymal cysts, and diffuse and focal hyperechogenicity of the periventricular white matter and basal ganglia, respectively, were the abnormal findings, that may be present even before the development of major neurological symptoms. Conclusion Early cranial US is able to visualize the whole spectrum of cerebral anomalies in AGS: calcifying microangiopathy, white matter disease and unusual subependymal cysts. The imaging pattern is similar to that of congenital viral infection of the central nervous system, which may mislead the genetic counseling.

From 1669 onwards the Leiden surgeons held their meetings in the town's weigh-house (figs. 1–3). The Collegium Chirurgicum, presided over by a professor of medicine at the university, instructed and examined the surgeons' pupils in the midst of a collection of objects that served partly for teaching and partly for decorating the guild room (figs. 4–9). On the basis of the earliest inventory, of 1714, an attempt is made here to give an idea of the significance of the collection and the status of the guild. The instruments in the surgeons' cupboard (fig. 12; in 1714 there were around 50 of them, 22 of which could be identified) are discussed in the appendix. They were mainly used as teaching aids, but in 1714 some of them were already out-of-date and inadequate for carrying out surgical operations.

In the last decades of the sixteenth century a number of volute gables was built in the Maasland between the Belgian town of Dinant and the Dutch town of Roermond. The form and decoration of these gables were inspired by Flemish and Brabant gables in the style of Vredeman de Vries, and adapted to the local building materials and styles which characterize the so-called Mosa-Renaissance. The chief materials used were marl, a soft yellowish limestone, and brick. The way in which these materials were applied – by alternating brickwork with bands and blocks of limestone – is reminiscent of Brabant architecture. Their and the materials used set the Maasland volute gables apart from their 'Gelderland' counterparts (which are to be found roughly to the north of the line Roermond-Sittard-Aachen) which are built entirely of bricks with arched and S-shaped gable endings and upright courses, in a composition that is based on the Gothic tradition in these parts. The earliest gables with concave mouldings were probably built after 1574 (Rijkhoven in Belgium, Landcommanderij of the Teutonic Order Alde-Biesen and possibly a decade earlier there; ills. 86-91) and then only to decorate the façade of monumental buildings such as castles, religious institutions, town halls and the homes of the wealthy. As the religious orders and the nobility started building in the new fashion along the river Maas – the all-important trade route which flowed through land rich in building materials – the curved gable type gradually spread over the Maasland, initially along the river and later eastwards via Maastricht. Around 1600 the first curved gables were to be seen in South Limburg, notably in Borgharen (end sixteenth century; ill. 38), Urmond (the Schippershuis, 1612; ill. 41), Mheer (farmhouse with two gables on castle grounds, also 1612, ills. 39 and 40) and in Maastricht (southern entrance of the St. Servaes Church, tower of the bridge over the Maas, and houses on Grote Looierstraat 15 and Hexenstraat 14, all between 1600 and 1625, ills. 19-25 and 1-2). There too the volute gable, which was still relatively uncommon at that time and which did not differ essentially from its Belgian predecessor (although the composition of the top was of a some-what more heterogeneous nature) was first introduced as a decorative feature of stately buildings, chiefly for status reasons. The Eighty Years' War (1568-1648), and particularly the invasion and destruction of Maastricht by the Duke of Parma in 1579, resulted in a period of economic stagnation. Consequently few new houses could be constructed, and builders concentrated on the restoration and conservation of the existing, mostly wooden, houses. It was not until 1650 that Maastricht started building new houses on a larger scale, several of which possessed volute gables (ills. 32 and 34). Outside the towns these gables were still only to be seen on a small number of grand houses built in the relatively peaceful period of the Twelve-Year Truce (1609-1621); only at the end of the seventeenth century were such gables used as decoration for farmhouses also (Houthem, Provinciale Weg 17, was the lonely forerunner in 1608; ill. 45). Developments in Maastricht were hastened by government measures directed against the traditional method of building with wood – particularly so after 1632, the year Prince Frederick Henry took possession of Maastricht, when these measures became increasingly severe and the city started regaining its economic power. The more standardized type of curved gable that was fashionable there in the second half of the seventeenth century (Lenculenstraat 34, etc.; ills. 9-12) must have evolved from older, now lost gables (house in Pieterstraat; ill. 27) and from the earliest curved gables in the Maasland. Although curved gables had become a familiar sight in Maastricht by the mid-seventeenth century (ills. 33 and 34), the architecture of the city was still characterized by the traditional horizontal cornices. The general picture of architecture in the countryside, where gables were predominantly pointed, also remained largely unchanged, while in the south of Limburg (mainly due to its geographical position) the tradition of timber frame buildings continued. The number of seventeenth-century volute gables in rural districts is small (ills. 38-53). After a first step in the direction of a less local architecture, there was a long pause before the new development was at last resumed towards the end of the seventeenth century, with the result that curved gables started to be built on farmhouses instead of on monumental buildings. The fact that there are relatively few surviving examples of volute gables after the first quarter of the seventeenth century may be due to natural causes (vulnerability of the gables and city fires) as well as to the deliberate destruction that took place during the wars in this area. After some years (ca. 1690-1700) during which builders experimented with the shape of the gable (ills. 50 and 51) on the basis of older examples (ills. 42, 44 and 45) which in turn were derived from gable forms such as those at Alde-Biesen, a specific type of curved gable evolved that remained largely unchanged until 1785. At that time the volute gable in Maastricht had, with a few exceptions (ill. 14) long since become outdated. An answer to the question why so many volute gables were used to decorate the front of farmhouses built after 1700 may be found in the agricultural situation, which improved considerably after the turn of the century. There, in the neighbourhood of the limestone quarries, the curved gables were generally constructed entirely of this stone, as opposed to the older gables which combined limestone and brickwork – a method that was to reappear in the eighteenth century in building situated farther away from the true limestone country. There is reason to believe that the workers in the quarries and the masons who handled this stone were (to a certain extent) responsible for the eighteenth-century uniformity of the gable type: they may have worked in various places and possibly even fashioned limestone parts such as volutes, mouldings and gable ornaments in the quarry itself, in preparation for assembly on the building site.