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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

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JoVE 57256


 JoVE In-Press

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

Simple Generation of a High Yield Culture of Induced Neurons from Human Adult Skin Fibroblasts

1Department of Experimental Medical Science, Wallenberg Neuroscience Center, Division of Neurobiology and Lund Stem Cell Center, Lund University, 2John van Geest Centre for Brain Repair & Department of Neurology, Department of Clinical Neurosciences and Cambridge Stem Cell Institute, University of Cambridge

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JoVE 56904


 JoVE In-Press

Battery of Behavioral Tests Assessing General Locomotion, Muscular Strength, and Coordination in Mice

1Centre for Neuroscience Studies, Queen's University, 2Department of Biomedical and Molecular Sciences, Queen's University, 3Medical Genetics/ Department of Pediatrics, Kingston General Hospital, Centre for Neuroscience Studies, and Department of Biomedical and Molecular Sciences, Queen's University

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JoVE 55491


 JoVE In-Press

Purification of Transcripts and Metabolites from Drosophila Heads

1Department of Neurology, McKnight Brain Institute, University of Florida, 2Department of Entomology and Nematology, University of Florida, 3Genetics Institute, Department of Molecular Genetics and Microbiology, University of Florida, 4McKnight Brain Institute, Department of Neuroscience, Genetics Institute, Center for Translational Research on Neurodegenerative Diseases, and Center for Movement Disorders and Neurorestoration, University of Florida

JoVE 50245


 Biology

Direct Induction of Human Neural Stem Cells from Peripheral Blood Hematopoietic Progenitor Cells

1Translational Neuroscience Center, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 2Laboratory of Molecular Medicine and Neuroscience, National Institute of Neurological Disorders and Stroke, National Institutes of Health

JoVE 52298


 Developmental Biology

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