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Objective: To study the frequency of additional cerebral demyelination in Dutch patients with adrenomyeloneuropathy (AMN)., Methods: Consecutive patients with AMN from the Dutch X-linked adrenoleukodystrophy cohort without cerebral demyelination on MRI at inclusion, seen between January 1, 1992, and January 1, 1999, were included. Primary endpoints were brain involvement, death, or the end of follow-up on January 1, 2011. Three levels of certainty were used for cerebral demyelination: (1) signs and symptoms reported by relatives and treating physicians, confirmed by brain MRI; if follow-up MRIs were not available, (2) based upon information from treating physicians and relatives, and (3) based upon information obtained from relatives only. Results were compared with a study published in 2001, in which 13/68 (19.1%) patients with AMN developed cerebral demyelination in 9.5 ± 5.5 years. Differences of the proportions of patients with cerebral demyelination and their 95% confidence intervals (CIs) were calculated., Results: Of 27 patients with AMN, 17 (63%) developed cerebral demyelination 10.2 ± 6.9 years after onset of myelo(neuro)pathy. Mean survival was 3.4 ± 2.9 years. Brain involvement was higher in Dutch patients with AMN (difference 44%, 95% CI 0.23-0.64)., Conclusions: Cerebral demyelination in AMN may be more frequent than previously reported. Survival is as poor as in childhood cerebral adrenoleukodystrophy. Therapies that can halt cerebral demyelination in these patients are needed., (© 2014 American Academy of Neurology.)

In The Netherlands a national PACS development programme has been started, supported by the Dutch Society of Radiology and funded by the Dutch Department of Health because of the national character of the project. Three main partners are cooperating in this development: the Utrecht University Hospital (AZU), BAZIS and Philips International (Product Division Medical Systems), with the Delft University of Technology as the main BAZIS subcontractor. The non-profit foundation BAZIS, developing and supporting the ZIS Hospital Information System (in use in some 30 Dutch hospitals, over 15,000 acute beds), initiated its current IMAGe Information System (IMAGIS) projects in 1984. The activities were later integrated into the Dutch PACS project started in 1986. The final goal of the project is to achieve a PACS which is fully integrated with already existing hospital information systems (HIS). The development and operation of a HIS-PACS include many aspects of technical and clinical. The current efforts of BAZIS are concentrated on three main issues: diagnostic image quality evaluation (e.g. effects of data compression); modelling, software simulation and technology assessment of a prototype PACS (both general and detailed aspects); and coupling and integration of PACS and HIS (e.g. the BAZIS ZIS). Philips, Hamburg, is supplying equipment, particularly prototype components. A systematic clinical evaluation will take place at the Utrecht University Hospital.2+ We outline the background of the intermediate results as demonstrated during the 6th EuroPACS Conference:the psychophysical software package for Feature Evaluation And System Inspection By Logged Experiments (FEASIBLE); the modelling and simulation software package for Medical Image Representation, Archiving and Communication, Learned by Extensive Simulation (MIRACLES); and first results of the coupling experiments.(ABSTRACT TRUNCATED AT 250 WORDS)