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Motor Neuron Disease: Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In Amyotrophic lateral sclerosis there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see Muscular atrophy, Spinal) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (Bulbar palsy, Progressive), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)

Motor Exam I

JoVE 10052

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.…

 Physical Examinations III

Isolation and Culture of Oculomotor, Trochlear, and Spinal Motor Neurons from Prenatal Islmn:GFP Transgenic Mice

1Department of Neurology, Boston Children's Hospital, 2FM Kirby Neurobiology Center, Boston Children's Hospital, 3Department of Neurology, Harvard Medical School, 4Medical Genetics Training Program, Harvard Medical School, 5Department of Ophthalmology, Boston Children's Hospital, 6Department of Ophthalmology, Harvard Medical School, 7Broad Institute of M.I.T. and Harvard, 8Howard Hughes Medical Institute, 9Department of Neurology, Kokura Memorial Hospital, 10Department of Genetics, Albert Einstein College of Medicine

Video Coming Soon

JoVE 60440

 JoVE In-Press

The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

1Physiology and Bosch Institute, University of Sydney, 2Motor Neuron Disease Research Group, Australian School of Advanced Medicine, Macquarie University, 3Advanced Microscopy Facility, Bosch Institute, University of Sydney

JoVE 52220

 Neuroscience

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

1Department of Neurology, Duke University School of Medicine, 2Duke Center for Neurodegeneration & Neurotherapeutics, Duke University School of Medicine, 3Biomedical Sciences Graduate Program, University of California San Diego, 4Department of Neurobiology, Duke University School of Medicine, 5Department of Cell Biology, Duke University School of Medicine

JoVE 59878

 Behavior

Conversion of Human Induced Pluripotent Stem Cells (iPSCs) into Functional Spinal and Cranial Motor Neurons Using PiggyBac Vectors

1Department of Biology and Biotechnology Charles Darwin, Sapienza University of Rome, Italy, 2Center for Life Nano Science, Istituto Italiano di Tecnologia, Italy, 3Laboratory of Stem Cell biology and Molecular Embryology, The Rockefeller University, USA, 4Department of Physiology and Pharmacology, Sapienza University of Rome, Italy

JoVE 59321

 Neuroscience

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

 Behavior

Assessment of Calcium Sparks in Intact Skeletal Muscle Fibers

1Department of Surgery, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, 2Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, 3Department of Molecular Biophysics and Physiology, Rush University Medical Center, 4Department of Internal Medicine, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center

JoVE 50898

 Biology

Abbiategrasso Brain Bank Protocol for Collecting, Processing and Characterizing Aging Brains

1Department of Neurology and Neuropathology, Golgi-Cenci Foundation, 2Laboratory of Neurobiology and Neurogenetic, Golgi-Cenci Foundation, 3Department of Neuropsychology and Social Sciences, Golgi-Cenci Foundation, 4Department of Rehabilitation, ASP Golgi-Redaelli Geriatric Hospital, 5Genomic and Post-Genomic Center, IRCCS Mondino Foundation, 6Department of Brain and Behavioral Sciences, University of Pavia, 7Department of Pathology, ASP Golgi-Redaelli Geriatric Hospital, 8Department of Neurological Science, IRCCS Mondino Foundation, University of Pavia

Video Coming Soon

JoVE 60296

 JoVE In-Press
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