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Muscular Atrophy, Spinal: A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset Spinal muscular atrophies of childhood, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
 JoVE In-Press

Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease

1Institute of Imaging Science, Vanderbilt University, 2Department of Radiology and Radiological Sciences, Vanderbilt University, 3Department of Biomedical Engineering, Vanderbilt University, 4Department of Molecular Physiology and Biophysics, Vanderbilt University, 5Department of Physical Medicine and Rehabilitation, Vanderbilt University, 6Department of Physics and Astronomy, Vanderbilt University

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JoVE 52352

 JoVE Behavior

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles

1Department of Neurology, The Ohio State University Wexner Medical Center, 2Department of Physical Medicine and Rehabilitation, The Ohio State University, 3Department of Neuroscience, The Ohio State University Wexner Medical Center, 4Department of Biochemistry and Pharmacology, The Ohio State University Wexner Medical Center


JoVE 52899

 JoVE Medicine

Delivery of Therapeutic Agents Through Intracerebroventricular (ICV) and Intravenous (IV) Injection in Mice

1Department of Molecular Microbiology and Immunology, Bond Life Sciences Center, University of Missouri, 2Department of Biological Sciences, Columbia University, 3Department of Veterinary Pathobiology, Bond Life Sciences Center, University of Missouri


JoVE 2968

 JoVE Neuroscience

Stereotaxic Injection of a Viral Vector for Conditional Gene Manipulation in the Mouse Spinal Cord

1Département Nociception et Douleur, Institut des Neurosciences Cellulaires et Intégratives, Centre National de la Recherche Scientifique (CNRS), 2Departments of Anesthesiology and Pharmacology, Columbia University, 3Department of Anesthesiology, Niigata University Graduate School of Medical and Dental Sciences


JoVE 50313

 JoVE Biology

A Guide to Modern Quantitative Fluorescent Western Blotting with Troubleshooting Strategies

1Division of Neurobiology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 2Division of Developmental Biology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, 3Centre for Integrative Physiology, University of Edinburgh, 4Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh


JoVE 52099

 JoVE Neuroscience

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia

1Department of Biochemistry, University of Washington, 2Department of Neurology, University of Washington, 3Division of Genetics, Departments of Pediatrics and Cellular and Molecular Medicine, and the Institute for Genomic Medicine, University of California, San Diego - Rady Children’s Hospital


JoVE 1787

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 JoVE Immunology and Infection

Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination

1Physical Medicine and Rehabilitation, University of Alabama at Birmingham, 2Department of Pathology, University of Alabama at Birmingham, 3Department of Neurobiology, University of Alabama at Birmingham, 4Center for Glial Biology and Medicine, University of Alabama at Birmingham


JoVE 54348

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 JoVE Bioengineering

Training Persons with Spinal Cord Injury to Ambulate Using a Powered Exoskeleton

1Department of Veterans Affairs (VA) Rehabilitation Research and Development National Center of Excellence for the Medical Consequences of Spinal Cord Injury, James J. Peters VA Medical Center, 2Department of Veterans Affairs (VA) Spinal Cord Injury Service, James J. Peters VA Medical Center


JoVE 54071

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 JoVE Medicine

Contrast Enhanced Ultrasound Imaging for Assessment of Spinal Cord Blood Flow in Experimental Spinal Cord Injury

1Laboratoire d'étude de la microcirculation, Faculté de Médecine Paris Diderot Paris VII, U942, 2Department of orthopaedic surgery, Bicetre Universitary Hospital, Public Assistance of Paris Hospital, 3Institute of Medical Science, Faculty of Medicine, University of Toronto, 4Department of Intensive care and Anesthesiology, Bicetre Universitary Hospital, Public Assistance of Paris Hospital


JoVE 52536

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 JoVE Neuroscience

Transplantation of Olfactory Ensheathing Cells to Evaluate Functional Recovery after Peripheral Nerve Injury

1UPRES EA3830, Institute for Research and Innovation in Biomedicine, University of Rouen, 2Neuroscience, Karolinska Institutet, 3Otorhinolaryngology, Head and Neck Surgery Department, Rouen University Hospital, 4Otorhinolaryngology, Head and Neck Surgery Department, Amiens University Hospital


JoVE 50590

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 JoVE Medicine

Lesion Explorer: A Video-guided, Standardized Protocol for Accurate and Reliable MRI-derived Volumetrics in Alzheimer's Disease and Normal Elderly

1LC Campbell Cognitive Neurology Research Unit, Heart & Stroke Foundation Canadian Partnership for Stroke Recovery, Brain Sciences Research Program, Sunnybrook Health Sciences Centre, 2Department of Medicine (Neurology), Institute of Medical Science, University of Toronto


JoVE 50887

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 Science Education: Essentials of Lab Animal Research

Diagnostic Necropsy and Tissue Harvest

JoVE Science Education

Source: Kay Stewart, RVT, RLATG, CMAR; Valerie A. Schroeder, RVT, RLATG. University of Notre Dame, IN

Many animal experiments rely on final data collection time points that are gathered from the harvesting and testing of organs and tissues. The use of appropriate methods for the collection of organs and tissues can impact the quality of the samples and the analysis of the data that is gleaned for the testing of the tissues. The method of euthanasia of the animal can also impact the quality of the samples. This manuscript will outline proper necropsy techniques for rats.

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 Science Education: Essentials of Physical Examinations III

Motor Exam I

JoVE Science Education

Source: Tracey A. Milligan, MD; Tamara B. Kaplan, MD; Neurology, Brigham and Women's/Massachusetts General Hospital, Boston, Massachusetts, USA

Abnormalities in the motor function are associated with a wide range of diseases, from movement disorders and myopathies to strokes. The motor assessment starts with observation of the patient. When the patient enters the examination area, the clinician observes their ability to walk unassisted and their speed and coordination while moving. Taking the patient's history provides an additional opportunity to observe for evidence of tremors or other abnormal movements, such as chorea or tardive dyskinesia. Such simple but important observations can yield valuable clues to the diagnosis and helps to focus the rest of the examination. The motor assessment continues in a systematic fashion, including inspection for muscle atrophy and abnormal movements, assessment of muscle tone, muscle strength testing, and finally, the examination of the muscle reflexes and coordination. The careful systematic testing of the motor system and the integration of all the findings provide insight to the level at which the motor pathway is affected, and also help the clinician to formulate the differential diagnosis and determine the course of the subsequent evaluation and treatment.

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 JoVE Medicine

Isolation and Immortalization of Patient-derived Cell Lines from Muscle Biopsy for Disease Modeling

1Department of Cell Biology, UT Southwestern Medical Center, 2National Institute of Neurological Disorders and Stroke, National Institute of Health, 3Division of Pediatric Pathology, Department of Pathology and Laboratory Medicine, Medical College of Wisconsin, 4Division of Genetics and Genomics, Boston Children's Hospital


JoVE 52307

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