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Huntington Disease: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive Chorea and Dementia in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; Depression; Hallucinations; and Delusions. Eventually intellectual impairment; loss of fine motor control; Athetosis; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including Seizures; Ataxia; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease

1The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 2Center for Imaging Science, Johns Hopkins University, 3Institute for Computational Medicine, Johns Hopkins University, 4Department of Applied Mathematics and Statistics, Johns Hopkins University, 5Division of Neurobiology, Departments of Psychiatry, Neurology, Neuroscience and Pharmacology, and Program in Cellular and Molecular Medicine, Johns Hopkins University School of Medicine, 6F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, 7Department of Biomedical Engineering, Johns Hopkins University

JoVE 57256


 Medicine

Combined Invasive Subcortical and Non-invasive Surface Neurophysiological Recordings for the Assessment of Cognitive and Emotional Functions in Humans

1Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University, 2Department of Neurology, Center for Movement Disorders and Neuromodulation, University Clinic Düsseldorf, 3Department of Neurosurgery, Functional Neurosurgery and Stereotaxy, Center for Movement Disorders and Neuromodulation, University Clinic Düsseldorf

JoVE 53466


 Behavior

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