http://www.ajronline.org/cgi/reprint/158/6/1193

MS Pont and AD Elster

The neurocutaneous syndromes, or phakomatoses, are a diverse group of disorders affecting the skin and CNS. This review highlights some important but lesser known aspects of the more common phakomatoses while concentrating on rarer and more recently discovered syndromes. The roles of newer imaging techniques such as contrast-enhanced MR imaging and three dimensional CT in the evaluation of these disorders also are discussed