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Amyotrophic Lateral Sclerosis: A degenerative disorder affecting upper Motor neurons in the brain and lower motor neurons in the brain stem and Spinal cord. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, Fasciculation, hyperreflexia, Dysarthria, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous Astrocytes and atrophy of anterior Spinal nerve roots and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)

Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis

1School for Engineering of Matter, Transport and Energy, Arizona State University, 2Department of Neurology, Georgetown University Medical Center, 3Department of Pathology, Georgetown University Medical Center

JoVE 52584


 Bioengineering

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

1Department of Speech-Language Pathology, University of Toronto, 2ALS/ MN Clinic, Sunnybrook Health Science Centre, 3Department of Special Education and Communication Disorders, University of Nebraska-Lincoln, 4Department of Neurology, Munroe-Meyer Institute, University of Nebraska Medical Center, 5Department of Neurology, University of Toronto

JoVE 2422


 Medicine

Adapting Human Videofluoroscopic Swallow Study Methods to Detect and Characterize Dysphagia in Murine Disease Models

1Department of Otolaryngology - Head and Neck Surgery, University of Missouri, 2Department of Communication Science and Disorders, University of Missouri, 3Department of Medicine, University of Missouri

JoVE 52319


 Medicine

Antibody Binding Specificity for Kappa (Vκ) Light Chain-containing Human (IgM) Antibodies: Polysialic Acid (PSA) Attached to NCAM as a Case Study

1Department of Neurology, Mayo Clinic, 2Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 3Center for Regenerative Medicine, Neuroregeneration, Mayo Clinic, 4Division of Neonatal Medicine, Mayo Clinic, 5Department of Pediatric and Adolescent Medicine, Mayo Clinic

JoVE 54139


 Immunology and Infection

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

High-throughput Analysis of Locomotor Behavior in the Drosophila Island Assay

1Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, 2Centre for Molecular and Biomolecular Informatics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, 3Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center

Video Coming Soon

JoVE 55892


 JoVE In-Press

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

Cranial Nerves Exam II (VII-XII)

JoVE 10005

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

The cranial nerve examination follows the mental status evaluation in a neurological exam. However, the examination begins with observations made upon greeting the patient. For example, weakness of the facial muscles (which are innervated by cranial nerve VII) can be readily apparent during the first encounter with the patient. Cranial nerve VII (the facial nerve) also has sensory branches, which innervate the taste buds on the anterior two-thirds of the tongue and the medial aspect of the external auditory canal. Therefore, finding ipsilateral taste dysfunction in a patient with facial weakness confirms the involvement of cranial nerve VII. In addition, knowledge of the neuroanatomy helps the clinician to localize the level of the lesion: unilateral weakness of the lower facial muscles suggests a supranuclear lesion on the opposite side, while lesions involving the nuclear or infranuclear portion of the facial nerve manifest with an ipsilateral paralysis of all the facial muscles on the involved side. Cranial nerve VIII (the acoustic nerve) has two divisions: the hearing (cochlear) division and the vestibular division, which innervates the semi


 Physical Examinations III

Ultrasound-guided Botulinum Toxin-A Injections: A Method of Treating Sialorrhea

1Clinical and Biological Sciences Department, Neurology Unit, University of Torino, San Luigi Gonzaga Hospital, 2Oncology Department, Radiology Unit, University of Torino, San Luigi Gonzaga Hospital, 3Clinical and Biological Sciences Department, Dietologic and Nutrition Unit, University of Torino, San Luigi Gonzaga Hospital

JoVE 54606


 Medicine

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