JAMA Neurology: Adrenal Diseases Topic Collection http://archneur.jamanetwork.com/ en-us Mon, 22 Apr 2013 00:00:00 GMT Mon, 22 Apr 2013 15:45:08 GMT Silverchair editor@archneur.jamanetwork.com webmaster@archneur.jamanetwork.com Improvement of White Matter Changes on Neuroimaging Modalities After Stem Cell Transplant in Metachromatic Leukodystrophy White Matter Changes After HSCT for MLD http://archneur.jamanetwork.com/article.aspx?articleID=1680458 Mon, 22 Apr 2013 00:00:00 GMT van Egmond ME, Pouwels PW, Boelens J, et al. <span class="paragraphSection"><div class="boxTitle">Importance</div>We sought to illustrate improvement of cerebral white matter changes in metachromatic leukodystrophy after treatment with hematopoietic stem cell transplant (HSCT).<div class="boxTitle">Observations</div>We conducted serial magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (<sup>1</sup>H-MRS) as standard follow-up after HSCT with cord blood in 1 patient with juvenile metachromatic leukodystrophy diagnosed before frank degenerative symptoms developed. We measured MRI and <sup>1</sup>H-MRS changes. The white matter changes first increased after HSCT, then decreased in relation to the pre-HSCT MRI and <sup>1</sup>H-MRS.<div class="boxTitle">Conclusions and Relevance</div>Hematopoietic stem cell transplant, if performed early in metachromatic leukodystrophy, can not only stabilize but even improve cerebral white matter abnormalities. Our findings suggest a biological effect of HSCT.</span> 1 4 10.1001/jamaneurol.2013.629 http://archneur.jamanetwork.com/article.aspx?articleID=1680458