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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)

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

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

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. When the patient enters the examination area, the clinician observes the patient's ability to walk unassisted and the 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 help 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.


 Physical Examinations III

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

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