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Several known or putative glycosyltransferases are required for the synthesis of laminin-binding glycans on alpha-dystroglycan (αDG), including POMT1, POMT2, POMGnT1, LARGE, Fukutin, FKRP, ISPD and GTDC2. Mutations in these glycosyltransferase genes result in defective αDG glycosylation and reduced ligand binding by αDG causing a clinically heterogeneous(More)
  • W Halliday
  • 1995
The development of magnetic resonance imaging has increased the number of clinical and pathological reports of Hallervorden-Spatz disease and Hallervorden-Spatz syndrome. The case-to-case variability is considerable. However, if gene loci and basic pathogenetic mechanisms are to be appreciated, it is imperative that like cases be compared and studied. The(More)
West Nile virus (WNV) infection causes neurological disease at all levels of the neural axis, accompanied by neuroinflammation and neuronal loss, although the underlying mechanisms remain uncertain. Given the substantial activation of neuroinflammatory pathways observed in WNV infection, we hypothesized that WNV-mediated neuroinflammation and cell death(More)
BACKGROUND The cortical changes resulting from chronic hydrocephalus in adults are not well defined. METHODS Retrospective analysis of twenty-one patients (age 64-88 years) with a clinical diagnosis of "normal pressure hydrocephalus" who underwent cortical biopsy at the time of intracranial pressure monitoring or shunt insertion, and eight patients who(More)
PURPOSE Cortical dysplasia (CD) has been classified as Taylor's focal cortical dysplasia (FCD type II) or other CD (FCD type I and mild malformation of cortical development) based on histological findings. The aims of this study were to determine whether MRI and magnetoencephalography (MEG) could distinguish between these two groups and to evaluate surgical(More)
OBJECT The subthalamic nucleus (STN) is a target in surgery for Parkinson disease, but its location according to brain atlases compared with its position on an individual patient's magnetic resonance (MR) images is incompletely understood. In this study both the size and location of the STN based on MR images were compared with those on the Talairach and(More)
The authors review the cases of 60 children (aged 5 months to 18 years) with thalamic tumors who were seen at The Hospital for Sick Children between 1951 and 1983. The diagnosis of thalamic tumor was based on neuroradiological studies; 33 children were first seen in the pre-computerized tomography (CT) era and 27 since the advent of CT scanning.(More)
Capillary hemangiomas are rare benign vascular tumors that tend to occur in children. Whereas the majority of hemangiomas may regress spontaneously, those associated with functional sequelae or severe symptoms may require treatment. Two patients with capillary hemangiomas of the cavernous sinus that caused neurological symptoms were treated with(More)
Artificial neural network classification methods were applied to infrared spectra of histopathologically confirmed Alzheimer's diseased and control brain tissue. Principal component analysis was used as a preprocessing technique for some of these artificial neural networks while others were trained using the original spectra. The leave-one-out method was(More)
Fetal magnetic resonance provides a new tool in the imaging of the posterior fossa and is proving useful in cases that are difficult to assess sonographically by allowing further assessment of the fourth ventricle, cisterna magna, and vermian growth and development. We describe various criteria with which to evaluate vermian growth, including vermian(More)