Inherited defects in the degradation of glycosphingolipids (GSLs) cause a group of severe diseases known as GSL storage disorders. There are currently no effective treatments for the majority of these disorders. We have explored a new treatment paradigm, substrate deprivation therapy, by constructing a genetic model in mice. Sandhoff's disease mice, which abnormally accumulate GSLs, were bred w...

Zika virus (ZIKV) is a member of the Flaviviridae family causing asymptomatic or mildly symptomatic infections with fever, rash, arthralgia and headache. It is transmitted by the Aedes species mosquitoes and also sexually and transplacentally, and has been recently associated with congenital neurologic birth defects in South and Central America. We report the case of a newly married couple from...

We describe 71 cases of oromandibular reconstruction using microvascular composite free flaps. There was an overall flap success rate of 94%, while 97% of the patients in this series had their mandibles reconstructed with free vascularized bone flaps. Fifteen patients were rehabilitated with implant-borne dental prostheses. Primary repair of discontinuity defects of the inferior-alveolar nerve ...

BACKGROUND In children, frequent congenital malformations with concomitant agenesis of the corpus callosum are diagnosed by neuroimaging in association with other cerebral malformations, including interhemispheric cysts and ventriculomegaly. Similar studies providing full characterization of brain defects by in vivo magnetic resonance imaging (MRI), and correlations with the pertinent anatomic ...

INADEQUATE/INSUFFICIENT EVIDENCE TO DETERMINE WHETHER AN ASSOCIATION EXISTS • Any cancer • Cardiovascular conditions or conditions of the blood and blood-forming organs • Endocrine, nutritional, and metabolic conditions • Neurodegenerative diseases other than ALS and multiple sclerosis • Neurocognitive and neurobehavioral performance • Migraines and other headache disorders • Other neurologic o...

OBJECTIVES To further characterize mtDNA defects associated with autistic features, especially the A3243G mtDNA mutation and mtDNA depletion.Study design Five patients with autistic spectrum disorders and family histories of mitochondrial DNA diseases were studied. We performed mtDNA analysis in all patients and magnetic resonance spectroscopy in three. RESULTS Three patients manifested isola...

Does campaign duration affect election outcomes? To date, this question has largely evaded political scientists, but it is reasonable to expect systematic links between campaign length and candidate performance in elections. We hypothesize that longer campaigns would help challengers' electoral fortunes, thereby curbing incumbency advantage and potentially boosting competitiveness in elections....

Research on black representation in Congress emphasizes the material gains associated with black office holding over the intangible goods associated with citizens’ ability to identify racially with their legislators. This article considers the effect of descriptive representation on the relationships among citizens, legislators, and the Congress. With data from the 1980–1998 ANES, I show that w...