Atrophy: Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.Spinal Cord Injuries: Penetrating and non-penetrating injuries to the spinal cord resulting from traumatic external forces (e.g., WOUNDS, GUNSHOT; WHIPLASH INJURIES; etc.).Muscular Atrophy: Derangement in size and number of muscle fibers occurring with aging, reduction in blood supply, or following immobilization, prolonged weightlessness, malnutrition, and particularly in denervation.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)Optic Atrophy: Atrophy of the optic disk which may be congenital or acquired. This condition indicates a deficiency in the number of nerve fibers which arise in the RETINA and converge to form the OPTIC DISK; OPTIC NERVE; OPTIC CHIASM; and optic tracts. GLAUCOMA; ISCHEMIA; inflammation, a chronic elevation of intracranial pressure, toxins, optic nerve compression, and inherited conditions (see OPTIC ATROPHIES, HEREDITARY) are relatively common causes of this condition.Spinal Muscular Atrophies of Childhood: A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)Injections, Spinal: Introduction of therapeutic agents into the spinal region using a needle and syringe.Spinal Cord Diseases: Pathologic conditions which feature SPINAL CORD damage or dysfunction, including disorders involving the meninges and perimeningeal spaces surrounding the spinal cord. Traumatic injuries, vascular diseases, infections, and inflammatory/autoimmune processes may affect the spinal cord.Spinal Nerves: The 31 paired peripheral nerves formed by the union of the dorsal and ventral spinal roots from each spinal cord segment. The spinal nerve plexuses and the spinal roots are also included.Multiple System Atrophy: A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)Anesthesia, Spinal: Procedure in which an anesthetic is injected directly into the spinal cord.Spinal Nerve Roots: Paired bundles of NERVE FIBERS entering and leaving the SPINAL CORD at each segment. The dorsal and ventral nerve roots join to form the mixed segmental spinal nerves. The dorsal roots are generally afferent, formed by the central projections of the spinal (dorsal root) ganglia sensory cells, and the ventral roots are efferent, comprising the axons of spinal motor and PREGANGLIONIC AUTONOMIC FIBERS.Spinal Cord Neoplasms: Benign and malignant neoplasms which occur within the substance of the spinal cord (intramedullary neoplasms) or in the space between the dura and spinal cord (intradural extramedullary neoplasms). The majority of intramedullary spinal tumors are primary CNS neoplasms including ASTROCYTOMA; EPENDYMOMA; and LIPOMA. Intramedullary neoplasms are often associated with SYRINGOMYELIA. The most frequent histologic types of intradural-extramedullary tumors are MENINGIOMA and NEUROFIBROMA.Spinal Canal: The cavity within the SPINAL COLUMN through which the SPINAL CORD passes.Spinal DiseasesSpinal Cord Compression: Acute and chronic conditions characterized by external mechanical compression of the SPINAL CORD due to extramedullary neoplasm; EPIDURAL ABSCESS; SPINAL FRACTURES; bony deformities of the vertebral bodies; and other conditions. Clinical manifestations vary with the anatomic site of the lesion and may include localized pain, weakness, sensory loss, incontinence, and impotence.Spinal NeoplasmsSpinal Stenosis: Narrowing of the spinal canal.Spinal Fusion: Operative immobilization or ankylosis of two or more vertebrae by fusion of the vertebral bodies with a short bone graft or often with diskectomy or laminectomy. (From Blauvelt & Nelson, A Manual of Orthopaedic Terminology, 5th ed, p236; Dorland, 28th ed)Olivopontocerebellar Atrophies: A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085)Spinal Injuries: Injuries involving the vertebral column.Gyrate Atrophy: Progressive, autosomal recessive, diffuse atrophy of the choroid, pigment epithelium, and sensory retina that begins in childhood.Survival of Motor Neuron 1 Protein: A SMN complex protein that is essential for the function of the SMN protein complex. In humans the protein is encoded by a single gene found near the inversion telomere of a large inverted region of CHROMOSOME 5. Mutations in the gene coding for survival of motor neuron 1 protein may result in SPINAL MUSCULAR ATROPHIES OF CHILDHOOD.Motor Neurons: Neurons which activate MUSCLE CELLS.Magnetic Resonance Imaging: Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.Ganglia, Spinal: Sensory ganglia located on the dorsal spinal roots within the vertebral column. The spinal ganglion cells are pseudounipolar. The single primary branch bifurcates sending a peripheral process to carry sensory information from the periphery and a central branch which relays that information to the spinal cord or brain.Spinal Cord Ischemia: Reduced blood flow to the spinal cord which is supplied by the anterior spinal artery and the paired posterior spinal arteries. This condition may be associated with ARTERIOSCLEROSIS, trauma, emboli, diseases of the aorta, and other disorders. Prolonged ischemia may lead to INFARCTION of spinal cord tissue.SMN Complex Proteins: A complex of proteins that assemble the SNRNP CORE PROTEINS into a core structure that surrounds a highly conserved RNA sequence found in SMALL NUCLEAR RNA. They are found localized in the GEMINI OF COILED BODIES and in the CYTOPLASM. The SMN complex is named after the Survival of Motor Neuron Complex Protein 1, which is a critical component of the complex.Geographic Atrophy: A form of MACULAR DEGENERATION also known as dry macular degeneration marked by occurrence of a well-defined progressive lesion or atrophy in the central part of the RETINA called the MACULA LUTEA. It is distinguishable from WET MACULAR DEGENERATION in that the latter involves neovascular exudates.Paraplegia: Severe or complete loss of motor function in the lower extremities and lower portions of the trunk. This condition is most often associated with SPINAL CORD DISEASES, although BRAIN DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; and MUSCULAR DISEASES may also cause bilateral leg weakness.Muscular Disorders, Atrophic: Disorders characterized by an abnormal reduction in muscle volume due to a decrease in the size or number of muscle fibers. Atrophy may result from diseases intrinsic to muscle tissue (e.g., MUSCULAR DYSTROPHY) or secondary to PERIPHERAL NERVOUS SYSTEM DISEASES that impair innervation to muscle tissue (e.g., MUSCULAR ATROPHY, SPINAL).Survival of Motor Neuron 2 Protein: A SMN complex protein that is closely-related to SURVIVAL OF MOTOR NEURON 1 PROTEIN. In humans, the protein is encoded by an often duplicated gene found near the inversion centromere of a large inverted region of CHROMOSOME 5.Spine: The spinal or vertebral column.Thoracic Vertebrae: A group of twelve VERTEBRAE connected to the ribs that support the upper trunk region.Tuberculosis, Spinal: Osteitis or caries of the vertebrae, usually occurring as a complication of tuberculosis of the lungs.Laminectomy: A surgical procedure that entails removing all (laminectomy) or part (laminotomy) of selected vertebral lamina to relieve pressure on the SPINAL CORD and/or SPINAL NERVE ROOTS. Vertebral lamina is the thin flattened posterior wall of vertebral arch that forms the vertebral foramen through which pass the spinal cord and nerve roots.Spinal Curvatures: Deformities of the SPINE characterized by abnormal bending or flexure in the vertebral column. They may be bending forward (KYPHOSIS), backward (LORDOSIS), or sideway (SCOLIOSIS).Cervical Vertebrae: The first seven VERTEBRAE of the SPINAL COLUMN, which correspond to the VERTEBRAE of the NECK.Rats, Sprague-Dawley: A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.Hematoma, Epidural, Spinal: A rare epidural hematoma in the spinal epidural space, usually due to a vascular malformation (CENTRAL NERVOUS SYSTEM VASCULAR MALFORMATIONS) or TRAUMA. Spontaneous spinal epidural hematoma is a neurologic emergency due to a rapidly evolving compressive MYELOPATHY.Posterior Horn Cells: Neurons in the SPINAL CORD DORSAL HORN whose cell bodies and processes are confined entirely to the CENTRAL NERVOUS SYSTEM. They receive collateral or direct terminations of dorsal root fibers. They send their axons either directly to ANTERIOR HORN CELLS or to the WHITE MATTER ascending and descending longitudinal fibers.Optic Atrophy, Autosomal Dominant: Dominant optic atrophy is a hereditary optic neuropathy causing decreased visual acuity, color vision deficits, a centrocecal scotoma, and optic nerve pallor (Hum. Genet. 1998; 102: 79-86). Mutations leading to this condition have been mapped to the OPA1 gene at chromosome 3q28-q29. OPA1 codes for a dynamin-related GTPase that localizes to mitochondria.Muscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Lumbosacral Region: Region of the back including the LUMBAR VERTEBRAE, SACRUM, and nearby structures.Anterior Horn Cells: MOTOR NEURONS in the anterior (ventral) horn of the SPINAL CORD which project to SKELETAL MUSCLES.Lumbar Vertebrae: VERTEBRAE in the region of the lower BACK below the THORACIC VERTEBRAE and above the SACRAL VERTEBRAE.Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.X Chromosome: The female sex chromosome, being the differential sex chromosome carried by half the male gametes and all female gametes in human and other male-heterogametic species.Spinal Fractures: Broken bones in the vertebral column.Bulbo-Spinal Atrophy, X-Linked: An X-linked recessive form of spinal muscular atrophy. It is due to a mutation of the gene encoding the ANDROGEN RECEPTOR.Spinal Cord Regeneration: Repair of the damaged neuron function after SPINAL CORD INJURY or SPINAL CORD DISEASES.Hindlimb Suspension: Technique for limiting use, activity, or movement by immobilizing or restraining animal by suspending from hindlimbs or tails. This immobilization is used to simulate some effects of reduced gravity and study weightlessness physiology.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Quadriplegia: Severe or complete loss of motor function in all four limbs which may result from BRAIN DISEASES; SPINAL CORD DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; or rarely MUSCULAR DISEASES. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper BRAIN STEM which injures the descending cortico-spinal and cortico-bulbar tracts.Pain: An unpleasant sensation induced by noxious stimuli which are detected by NERVE ENDINGS of NOCICEPTIVE NEURONS.Trigeminal Nucleus, Spinal: Nucleus of the spinal tract of the trigeminal nerve. It is divided cytoarchitectonically into three parts: oralis, caudalis (TRIGEMINAL CAUDAL NUCLEUS), and interpolaris.Hyperalgesia: An increased sensation of pain or discomfort produced by mimimally noxious stimuli due to damage to soft tissue containing NOCICEPTORS or injury to a peripheral nerve.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Locomotion: Movement or the ability to move from one place or another. It can refer to humans, vertebrate or invertebrate animals, and microorganisms.Recovery of Function: A partial or complete return to the normal or proper physiologic activity of an organ or part following disease or trauma.Myelography: X-ray visualization of the spinal cord following injection of contrast medium into the spinal arachnoid space.Manipulation, Spinal: Adjustment and manipulation of the vertebral column.Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Neurons: The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.Gastritis, Atrophic: GASTRITIS with atrophy of the GASTRIC MUCOSA, the GASTRIC PARIETAL CELLS, and the mucosal glands leading to ACHLORHYDRIA. Atrophic gastritis usually progresses from chronic gastritis.Nerve Regeneration: Renewal or physiological repair of damaged nerve tissue.Neuralgia: Intense or aching pain that occurs along the course or distribution of a peripheral or cranial nerve.Paralysis: A general term most often used to describe severe or complete loss of muscle strength due to motor system disease from the level of the cerebral cortex to the muscle fiber. This term may also occasionally refer to a loss of sensory function. (From Adams et al., Principles of Neurology, 6th ed, p45)Nerve Tissue ProteinsEpidural Space: Space between the dura mater and the walls of the vertebral canal.Pain Measurement: Scales, questionnaires, tests, and other methods used to assess pain severity and duration in patients or experimental animals to aid in diagnosis, therapy, and physiological studies.SKP Cullin F-Box Protein Ligases: A subset of ubiquitin protein ligases that are formed by the association of a SKP DOMAIN PROTEIN, a CULLIN DOMAIN PROTEIN and a F-BOX DOMAIN PROTEIN.Cordotomy: Any operation on the spinal cord. (Stedman, 26th ed)Hindlimb: Either of two extremities of four-footed non-primate land animals. It usually consists of a FEMUR; TIBIA; and FIBULA; tarsals; METATARSALS; and TOES. (From Storer et al., General Zoology, 6th ed, p73)Spinal Puncture: Tapping fluid from the subarachnoid space in the lumbar region, usually between the third and fourth lumbar vertebrae.Myelitis: Inflammation of the spinal cord. Relatively common etiologies include infections; AUTOIMMUNE DISEASES; SPINAL CORD; and ischemia (see also SPINAL CORD VASCULAR DISEASES). Clinical features generally include weakness, sensory loss, localized pain, incontinence, and other signs of autonomic dysfunction.Electric Stimulation: Use of electric potential or currents to elicit biological responses.Scoliosis: An appreciable lateral deviation in the normally straight vertical line of the spine. (Dorland, 27th ed)Optic Atrophies, Hereditary: Hereditary conditions that feature progressive visual loss in association with optic atrophy. Relatively common forms include autosomal dominant optic atrophy (OPTIC ATROPHY, AUTOSOMAL DOMINANT) and Leber hereditary optic atrophy (OPTIC ATROPHY, HEREDITARY, LEBER).Nociceptors: Peripheral AFFERENT NEURONS which are sensitive to injuries or pain, usually caused by extreme thermal exposures, mechanical forces, or other noxious stimuli. Their cell bodies reside in the DORSAL ROOT GANGLIA. Their peripheral terminals (NERVE ENDINGS) innervate target tissues and transduce noxious stimuli via axons to the CENTRAL NERVOUS SYSTEM.Cerebellar Ataxia: Incoordination of voluntary movements that occur as a manifestation of CEREBELLAR DISEASES. Characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention TREMOR), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and GAIT ATAXIA. (From Adams et al., Principles of Neurology, 6th ed, p90)Reflex: An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord.Afferent Pathways: Nerve structures through which impulses are conducted from a peripheral part toward a nerve center.Spinal Cord Stimulation: Application of electric current to the spine for treatment of a variety of conditions involving innervation from the spinal cord.Decompression, Surgical: A surgical operation for the relief of pressure in a body compartment or on a body part. (From Dorland, 28th ed)Cats: The domestic cat, Felis catus, of the carnivore family FELIDAE, comprising over 30 different breeds. The domestic cat is descended primarily from the wild cat of Africa and extreme southwestern Asia. Though probably present in towns in Palestine as long ago as 7000 years, actual domestication occurred in Egypt about 4000 years ago. (From Walker's Mammals of the World, 6th ed, p801)Alzheimer Disease: A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)Brain Stem: The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA.Organ Size: The measurement of an organ in volume, mass, or heaviness.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Neurons, Afferent: Neurons which conduct NERVE IMPULSES to the CENTRAL NERVOUS SYSTEM.Spinal Dysraphism: Congenital defects of closure of one or more vertebral arches, which may be associated with malformations of the spinal cord, nerve roots, congenital fibrous bands, lipomas, and congenital cysts. These malformations range from mild (e.g., SPINA BIFIDA OCCULTA) to severe, including rachischisis where there is complete failure of neural tube and spinal cord fusion, resulting in exposure of the spinal cord at the surface. Spinal dysraphism includes all forms of spina bifida. The open form is called SPINA BIFIDA CYSTICA and the closed form is SPINA BIFIDA OCCULTA. (From Joynt, Clinical Neurology, 1992, Ch55, p34)Physical Stimulation: Act of eliciting a response from a person or organism through physical contact.Hematoma, Subdural, Spinal: Subdural hematoma of the SPINAL CANAL.Uveal Diseases: Diseases of the uvea.Electric Stimulation Therapy: Application of electric current in treatment without the generation of perceptible heat. It includes electric stimulation of nerves or muscles, passage of current into the body, or use of interrupted current of low intensity to raise the threshold of the skin to pain.Motor Activity: The physical activity of a human or an animal as a behavioral phenomenon.Nerve Degeneration: Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.Sciatic Nerve: A nerve which originates in the lumbar and sacral spinal cord (L4 to S3) and supplies motor and sensory innervation to the lower extremity. The sciatic nerve, which is the main continuation of the sacral plexus, is the largest nerve in the body. It has two major branches, the TIBIAL NERVE and the PERONEAL NERVE.Spinal Osteophytosis: Outgrowth of immature bony processes or bone spurs (OSTEOPHYTE) from the VERTEBRAE, reflecting the presence of degenerative disease and calcification. It commonly occurs in cervical and lumbar SPONDYLOSIS.Neuronal Apoptosis-Inhibitory Protein: An inhibitor of apoptosis protein that was initially identified during analysis of CHROMOSOME DELETIONS associated with SPINAL MUSCULAR ATROPHY. Naip contains a nucleotide binding oligomerization domain and a carboxy-terminal LEUCINE rich repeat.Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.Rats, Wistar: A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.Supranuclear Palsy, Progressive: A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)Disease Progression: The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.Immobilization: The restriction of the MOVEMENT of whole or part of the body by physical means (RESTRAINT, PHYSICAL) or chemically by ANALGESIA, or the use of TRANQUILIZING AGENTS or NEUROMUSCULAR NONDEPOLARIZING AGENTS. It includes experimental protocols used to evaluate the physiologic effects of immobility.Muscle Proteins: The protein constituents of muscle, the major ones being ACTINS and MYOSINS. More than a dozen accessory proteins exist including TROPONIN; TROPOMYOSIN; and DYSTROPHIN.Mice, Inbred C57BLMuscle Denervation: The resection or removal of the innervation of a muscle or muscle tissue.Cyclic AMP Response Element-Binding Protein: A protein that has been shown to function as a calcium-regulated transcription factor as well as a substrate for depolarization-activated CALCIUM-CALMODULIN-DEPENDENT PROTEIN KINASES. This protein functions to integrate both calcium and cAMP signals.Pyramidal Tracts: Fibers that arise from cells within the cerebral cortex, pass through the medullary pyramid, and descend in the spinal cord. Many authorities say the pyramidal tracts include both the corticospinal and corticobulbar tracts.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.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)Interneurons: Most generally any NEURONS which are not motor or sensory. Interneurons may also refer to neurons whose AXONS remain within a particular brain region in contrast to projection neurons, which have axons projecting to other brain regions.Kyphosis: Deformities of the SPINE characterized by an exaggerated convexity of the vertebral column. The forward bending of the thoracic region usually is more than 40 degrees. This deformity sometimes is called round back or hunchback.Aging: The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.Dura Mater: The outermost of the three MENINGES, a fibrous membrane of connective tissue that covers the brain and the spinal cord.Muscle Fibers, Skeletal: Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of SKELETAL MUSCLE. They consist of MYOFIBRILS enclosed within and attached to the SARCOLEMMA. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.Analgesics: Compounds capable of relieving pain without the loss of CONSCIOUSNESS.Cauda Equina: The lower part of the SPINAL CORD consisting of the lumbar, sacral, and coccygeal nerve roots.Facial Hemiatrophy: A syndrome characterized by slowly progressive unilateral atrophy of facial subcutaneous fat, muscle tissue, skin, cartilage, and bone. The condition typically progresses over a period of 2-10 years and then stabilizes.Sacrum: Five fused VERTEBRAE forming a triangle-shaped structure at the back of the PELVIS. It articulates superiorly with the LUMBAR VERTEBRAE, inferiorly with the COCCYX, and anteriorly with the ILIUM of the PELVIS. The sacrum strengthens and stabilizes the PELVIS.Animals, Newborn: Refers to animals in the period of time just after birth.Nerve Fibers, Myelinated: A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the myelinated nerve fibers are completely encased in a MYELIN SHEATH. They are fibers of relatively large and varied diameters. Their NEURAL CONDUCTION rates are faster than those of the unmyelinated nerve fibers (NERVE FIBERS, UNMYELINATED). Myelinated nerve fibers are present in somatic and autonomic nerves.Image Processing, Computer-Assisted: A technique of inputting two-dimensional images into a computer and then enhancing or analyzing the imagery into a form that is more useful to the human observer.Tomography, X-Ray Computed: Tomography using x-ray transmission and a computer algorithm to reconstruct the image.Muscle Spasticity: A form of muscle hypertonia associated with upper MOTOR NEURON DISEASE. Resistance to passive stretch of a spastic muscle results in minimal initial resistance (a "free interval") followed by an incremental increase in muscle tone. Tone increases in proportion to the velocity of stretch. Spasticity is usually accompanied by HYPERREFLEXIA and variable degrees of MUSCLE WEAKNESS. (From Adams et al., Principles of Neurology, 6th ed, p54)Neural Conduction: The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.Urinary Bladder, Neurogenic: Dysfunction of the URINARY BLADDER due to disease of the central or peripheral nervous system pathways involved in the control of URINATION. This is often associated with SPINAL CORD DISEASES, but may also be caused by BRAIN DISEASES or PERIPHERAL NERVE DISEASES.Analysis of Variance: A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable.Lampreys: Common name for the only family (Petromyzontidae) of eellike fish in the order Petromyzontiformes. They are jawless but have a sucking mouth with horny teeth.Nerve Fibers, Unmyelinated: A class of nerve fibers as defined by their nerve sheath arrangement. The AXONS of the unmyelinated nerve fibers are small in diameter and usually several are surrounded by a single MYELIN SHEATH. They conduct low-velocity impulses, and represent the majority of peripheral sensory and autonomic fibers, but are also found in the BRAIN and SPINAL CORD.Spinal Cord Vascular Diseases: Pathological processes involving any of the BLOOD VESSELS feeding the SPINAL CORD, such as the anterior and paired posterior spinal arteries or their many branches. Disease processes may include ATHEROSCLEROSIS; EMBOLISM; and ARTERIOVENOUS MALFORMATIONS leading to ISCHEMIA or HEMORRHAGE into the spinal cord (hematomyelia).Subarachnoid Space: The space between the arachnoid membrane and PIA MATER, filled with CEREBROSPINAL FLUID. It contains large blood vessels that supply the BRAIN and SPINAL CORD.Neurologic Examination: Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system.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)Ornithine-Oxo-Acid Transaminase: A pyridoxal phosphate enzyme that catalyzes the formation of glutamate gamma-semialdehyde and an L-amino acid from L-ornithine and a 2-keto-acid. EC 2.6.1.13.Shy-Drager Syndrome: A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord. This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with ORTHOSTASIS and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536)Nerve Fibers: Slender processes of NEURONS, including the AXONS and their glial envelopes (MYELIN SHEATH). Nerve fibers conduct nerve impulses to and from the CENTRAL NERVOUS SYSTEM.Paraparesis: Mild to moderate loss of bilateral lower extremity motor function, which may be a manifestation of SPINAL CORD DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; MUSCULAR DISEASES; INTRACRANIAL HYPERTENSION; parasagittal brain lesions; and other conditions.Decerebrate State: A condition characterized by abnormal posturing of the limbs that is associated with injury to the brainstem. This may occur as a clinical manifestation or induced experimentally in animals. The extensor reflexes are exaggerated leading to rigid extension of the limbs accompanied by hyperreflexia and opisthotonus. This condition is usually caused by lesions which occur in the region of the brainstem that lies between the red nuclei and the vestibular nuclei. In contrast, decorticate rigidity is characterized by flexion of the elbows and wrists with extension of the legs and feet. The causative lesion for this condition is located above the red nuclei and usually consists of diffuse cerebral damage. (From Adams et al., Principles of Neurology, 6th ed, p358)Behavior, Animal: The observable response an animal makes to any situation.Cognition Disorders: Disturbances in mental processes related to learning, thinking, reasoning, and judgment.Brain Diseases: Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.Factor X: Storage-stable glycoprotein blood coagulation factor that can be activated to factor Xa by both the intrinsic and extrinsic pathways. A deficiency of factor X, sometimes called Stuart-Prower factor deficiency, may lead to a systemic coagulation disorder.Neuroglia: The non-neuronal cells of the nervous system. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the BLOOD-BRAIN BARRIER and BLOOD-RETINAL BARRIER, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear.Dose-Response Relationship, Drug: The relationship between the dose of an administered drug and the response of the organism to the drug.Muscle Weakness: A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251)Retrospective Studies: Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.Spinocerebellar Degenerations: A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.Pain Threshold: Amount of stimulation required before the sensation of pain is experienced.Peripheral Nerves: The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.Morphine: The principal alkaloid in opium and the prototype opiate analgesic and narcotic. Morphine has widespread effects in the central nervous system and on smooth muscle.Evoked Potentials, Somatosensory: The electric response evoked in the CEREBRAL CORTEX by stimulation along AFFERENT PATHWAYS from PERIPHERAL NERVES to CEREBRUM.Neural Pathways: Neural tracts connecting one part of the nervous system with another.Cerebral Cortex: The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulchi. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.Epidural Abscess: Circumscribed collections of suppurative material occurring in the spinal or intracranial EPIDURAL SPACE. The majority of epidural abscesses occur in the spinal canal and are associated with OSTEOMYELITIS of a vertebral body; ANALGESIA, EPIDURAL; and other conditions. Clinical manifestations include local and radicular pain, weakness, sensory loss, URINARY INCONTINENCE, and FECAL INCONTINENCE. Cranial epidural abscesses are usually associated with OSTEOMYELITIS of a cranial bone, SINUSITIS, or OTITIS MEDIA. (From Adams et al., Principles of Neurology, 6th ed, p710 and pp1240-1; J Neurol Neurosurg Psychiatry 1998 Aug;65(2):209-12)Cerebellum: The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.Multiple Sclerosis: An autoimmune disorder mainly affecting young adults and characterized by destruction of myelin in the central nervous system. Pathologic findings include multiple sharply demarcated areas of demyelination throughout the white matter of the central nervous system. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia, and bladder dysfunction. The usual pattern is one of recurrent attacks followed by partial recovery (see MULTIPLE SCLEROSIS, RELAPSING-REMITTING), but acute fulminating and chronic progressive forms (see MULTIPLE SCLEROSIS, CHRONIC PROGRESSIVE) also occur. (Adams et al., Principles of Neurology, 6th ed, p903)Pedigree: The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.Autonomic Dysreflexia: A syndrome associated with damage to the spinal cord above the mid thoracic level (see SPINAL CORD INJURIES) characterized by a marked increase in the sympathetic response to minor stimuli such as bladder or rectal distention. Manifestations include HYPERTENSION; TACHYCARDIA (or reflex bradycardia); FEVER; FLUSHING; and HYPERHIDROSIS. Extreme hypertension may be associated with a STROKE. (From Adams et al., Principles of Neurology, 6th ed, pp538 and 1232; J Spinal Cord Med 1997;20(3):355-60)Reflex, Abnormal: An abnormal response to a stimulus applied to the sensory components of the nervous system. This may take the form of increased, decreased, or absent reflexes.Mice, Knockout: Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.Anesthesia, Obstetrical: A variety of anesthetic methods such as EPIDURAL ANESTHESIA used to control the pain of childbirth.Myelin Sheath: The lipid-rich sheath surrounding AXONS in both the CENTRAL NERVOUS SYSTEMS and PERIPHERAL NERVOUS SYSTEM. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.Neuromuscular Diseases: A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA.Oligodendroglia: A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.Strychnine: An alkaloid found in the seeds of STRYCHNOS NUX-VOMICA. It is a competitive antagonist at glycine receptors and thus a convulsant. It has been used as an analeptic, in the treatment of nonketotic hyperglycinemia and sleep apnea, and as a rat poison.Action Potentials: Abrupt changes in the membrane potential that sweep along the CELL MEMBRANE of excitable cells in response to excitation stimuli.RNA-Binding Proteins: Proteins that bind to RNA molecules. Included here are RIBONUCLEOPROTEINS and other proteins whose function is to bind specifically to RNA.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Peripheral Nerve Injuries: Injuries to the PERIPHERAL NERVES.Functional Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot.Myoclonic Epilepsies, Progressive: A heterogeneous group of primarily familial disorders characterized by myoclonic seizures, tonic-clonic seizures, ataxia, progressive intellectual deterioration, and neuronal degeneration. These include LAFORA DISEASE; MERRF SYNDROME; NEURONAL CEROID-LIPOFUSCINOSIS; sialidosis (see MUCOLIPIDOSES), and UNVERRICHT-LUNDBORG SYNDROME.Efferent Pathways: Nerve structures through which impulses are conducted from a nerve center toward a peripheral site. Such impulses are conducted via efferent neurons (NEURONS, EFFERENT), such as MOTOR NEURONS, autonomic neurons, and hypophyseal neurons.Anesthetics, Local: Drugs that block nerve conduction when applied locally to nerve tissue in appropriate concentrations. They act on any part of the nervous system and on every type of nerve fiber. In contact with a nerve trunk, these anesthetics can cause both sensory and motor paralysis in the innervated area. Their action is completely reversible. (From Gilman AG, et. al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 8th ed) Nearly all local anesthetics act by reducing the tendency of voltage-dependent sodium channels to activate.Temporal Lobe: Lower lateral part of the cerebral hemisphere responsible for auditory, olfactory, and semantic processing. It is located inferior to the lateral fissure and anterior to the OCCIPITAL LOBE.Synaptic Transmission: The communication from a NEURON to a target (neuron, muscle, or secretory cell) across a SYNAPSE. In chemical synaptic transmission, the presynaptic neuron releases a NEUROTRANSMITTER that diffuses across the synaptic cleft and binds to specific synaptic receptors, activating them. The activated receptors modulate specific ion channels and/or second-messenger systems in the postsynaptic cell. In electrical synaptic transmission, electrical signals are communicated as an ionic current flow across ELECTRICAL SYNAPSES.Astrocytes: A class of large neuroglial (macroglial) cells in the central nervous system - the largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the BLOOD-BRAIN BARRIER. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with MICROGLIA) respond to injury.Follow-Up Studies: Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Cerebellar Diseases: Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, GAIT ATAXIA, and MUSCLE HYPOTONIA.Phenotype: The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.Muscles: Contractile tissue that produces movement in animals.Syringomyelia: Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269)Dementia: An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness.Hippocampus: A curved elevation of GRAY MATTER extending the entire length of the floor of the TEMPORAL HORN of the LATERAL VENTRICLE (see also TEMPORAL LOBE). The hippocampus proper, subiculum, and DENTATE GYRUS constitute the hippocampal formation. Sometimes authors include the ENTORHINAL CORTEX in the hippocampal formation.Hyperesthesia: Increased sensitivity to cutaneous stimulation due to a diminished threshold or an increased response to stimuli.Pepsinogen A: This is one of 2 related pepsinogen systems in humans and is also known as pepsinogen. (The other is PEPSINOGEN C.) This includes isozymogens Pg1-Pg5 (pepsinogens 1-5, group I or products of PGA1-PGA5 genes). This is the main pepsinogen found in urine.Meninges: The three membranes that cover the BRAIN and the SPINAL CORD. They are the dura mater, the arachnoid, and the pia mater.Severity of Illness Index: Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.Reflex, Monosynaptic: A reflex in which the AFFERENT NEURONS synapse directly on the EFFERENT NEURONS, without any INTERCALATED NEURONS. (Lockard, Desk Reference for Neuroscience, 2nd ed.)Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Evoked Potentials: Electrical responses recorded from nerve, muscle, SENSORY RECEPTOR, or area of the CENTRAL NERVOUS SYSTEM following stimulation. They range from less than a microvolt to several microvolts. The evoked potential can be auditory (EVOKED POTENTIALS, AUDITORY), somatosensory (EVOKED POTENTIALS, SOMATOSENSORY), visual (EVOKED POTENTIALS, VISUAL), or motor (EVOKED POTENTIALS, MOTOR), or other modalities that have been reported.Bupivacaine: A widely used local anesthetic agent.Tibial Nerve: The medial terminal branch of the sciatic nerve. The tibial nerve fibers originate in lumbar and sacral spinal segments (L4 to S2). They supply motor and sensory innervation to parts of the calf and foot.Epidural Neoplasms: Neoplasms located in the space between the vertebral PERIOSTEUM and DURA MATER surrounding the SPINAL CORD. Tumors in this location are most often metastatic in origin and may cause neurologic deficits by mass effect on the spinal cord or nerve roots or by interfering with blood supply to the spinal cord.Macular Degeneration: Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.

Combined 3T diffusion tensor tractography and 1H-MR spectroscopy in motor neuron disease. (1/23)

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Spinal and bulbar muscular atrophy: a motoneuron or muscle disease? (2/23)

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Mitochondrial abnormalities in spinal and bulbar muscular atrophy. (3/23)

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Altered RNA splicing contributes to skeletal muscle pathology in Kennedy disease knock-in mice. (4/23)

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Autophagy and access: understanding the role of androgen receptor subcellular localization in SBMA. (5/23)

Ridding neurons of toxic misfolded proteins is a critical feature of many neurodegenerative diseases. We have recently reported that lack of access of nuclear polyglutamine-expanded androgen receptor (AR) to the autophagic degradation pathway is a critical point in pathogenesis. When mutant AR is contained within the cytoplasm, it can be degraded by autophagy, resulting in amelioration of its toxic effects, as has been observed in other polyglutamine expansion diseases involving cytoplasmic mutant proteins. However, we have also found that pharmacological induction of autophagy protects SBMA motor neurons from the toxic effects of even nuclear localized mutant AR, albeit without affecting mutant nuclear AR levels. Thus, we have further investigated the mechanism by which autophagy elicits therapeutic benefit in cell culture. We found that endogenous autophagy only slightly alters nuclear mutant AR aggregation compared to substantial effects on cytoplasmic AR aggregation. Interestingly, pharmacological activation of mTOR-dependent autophagy did not significantly alter nuclear AR aggregation, whereas we observed that it protects SBMA motor neurons. Our findings indicate that therapeutic intervention to induce autophagy represents a potential potent benefit for SBMA, and that it likely does so by protecting SBMA motor neurons independent of a direct effect on mutant AR.  (+info)

Clinical features of spinal and bulbar muscular atrophy. (6/23)

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B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy. (7/23)

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Prenatal flutamide enhances survival in a myogenic mouse model of spinal bulbar muscular atrophy. (8/23)

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Kennedys disease is a neurodegenerative disease caused by a gene mutation which encodes the androgen receptor. The signs and symptoms are clearly visible among patients. However with a prevalence of only 1 in 30.000 patients worldwide the disease is classified as rare. Research to date has shown no cure for ... read more this trinucleotide (CAG) repeat disorder which attacks the motor neurons and muscles. Small molecule drugs and biologics have failed to be beneficial and antisense technology should make a difference. Basically this approach offers new opportunities for neurodegenerative disorders especially Kennedy disease. In co-operation with Utrecht University, University of Glasgow and Prosensa we want to look at RNA modulation for Kennedys disease. The purpose of this research project is to synthesize and investigate several single stranded antisense oligonucleotides, with the antisense mRNA sequence 3 UUG-(CUGX)-CAG 5, where X stands for 10, 20, 30, 40, 50 or 60 CAG repeats (normally: ...
Objective: Spinal and bulbar muscular atrophy (SBMA), or Kennedy s disease, is a slowly progressive, X-linked motor neuron disease for which there is currently no treatment. It is caused by a mutation in the androgen receptor that results in a polyglutamine repeat expansion. The role of exercise in motor neuron disease research is debated. Although SBMA is believed to be primarily a neuronopathy, the potential effect of aberrant androgen receptor activity on muscle leads exercise to be of uncertain utility in this population. Our objective is to examine the safety and efficacy of exercise regimens in Kennedy s disease.. Study Population: We aim to enroll 80 men with genetically confirmed Kennedy s disease.. Design: This will be a randomized, evaluator blinded, trial with 25 subjects in each exercise arm. Following informed consent, the subjects will undergo an initial medical and physical evaluations followed by a series of neurological tests and blood work over a two-day outpatient visit at the ...
Kennedys Disease (KD) is a rare neuromuscular disease and a form of adult-onset Spinal Muscular Atrophy (SMA) that is a result of mutations in the Androgen Receptor gene on the X chromosome. There are small increases in one specific region of the gene. The larger the increases, the earlier the onset and the more pronounced the symptoms.. KD is also known as Spinal Bulbar Muscular Atrophy (SBMA). It is an inherited chromosomal X-linked recessive disease that affects the spinal and bulbar neurons causing muscle wasting (atrophy) predominantly in the legs, arms, face and throat. It is estimated that one in 40,000 people worldwide have KD.. ...
TY - JOUR. T1 - Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy. AU - Iida, Madoka. AU - Sahashi, Kentaro. AU - Kondo, Naohide. AU - Nakatsuji, Hideaki. AU - Tohnai, Genki. AU - Tsutsumi, Yutaka. AU - Noda, Seiya. AU - Murakami, Ayuka. AU - Onodera, Kazunari. AU - Okada, Yohei. AU - Nakatochi, Masahiro. AU - Tsukagoshi Okabe, Yuka. AU - Shimizu, Shinobu. AU - Mizuno, Masaaki. AU - Adachi, Hiroaki. AU - Okano, Hideyuki. AU - Sobue, Gen. AU - Katsuno, Masahisa. PY - 2019/12/1. Y1 - 2019/12/1. N2 - Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by an expanded CAG repeat in the androgen receptor (AR) gene. Here, we perform a comprehensive analysis of signaling pathways in a mouse model of SBMA (AR-97Q mice) utilizing a phosphoprotein assay. We measure the levels of 17 phosphorylated proteins in spinal cord and skeletal muscle of AR-97Q mice at three stages. The level of phosphorylated Src (p-Src) is ...
The views and opinions expressed on this blog are those strictly of the author. Information provided should not be considered medical advice nor the advice of a trained medical professional or physical therapist. The author has made a reasonable effort to ensure that all information provided is accurate, but as with any document, errors might occur. It is entirely the responsibility of the reader to determine the validity of any information provided. Any decisions made based upon the information provided are entirely the readers responsibility. The author and the Kennedys Disease Association do not accept any liability for any direct, indirect, special or consequential damages, or damages of any kind resulting from any cause through the use of any information obtained either directly or indirectly from this blog. ...
Kennedys disease is a rare genetic neurodegenerative disorder that affects the motor neurons (cells that are important for normal function of the brain and spinal cord). It is a progressive disorder that leads to increasing severity of motor dysfunction and subsequent deterioration of muscle strength, muscle tone, and motor coordination. It was first described by the American physician William R. Kennedy in 1966.
The views and opinions expressed on this blog are those strictly of the author. Information provided should not be considered medical advice nor the advice of a trained medical professional or physical therapist. The author has made a reasonable effort to ensure that all information provided is accurate, but as with any document, errors might occur. It is entirely the responsibility of the reader to determine the validity of any information provided. Any decisions made based upon the information provided are entirely the readers responsibility. The author and the Kennedys Disease Association do not accept any liability for any direct, indirect, special or consequential damages, or damages of any kind resulting from any cause through the use of any information obtained either directly or indirectly from this blog. ...
Reelz cable channel on Tuesday announced his casting in the role of the US Senator for the four-hour project, a follow-up to the miniseries The Kennedys.
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I posted a picture of Kennedy, kat (his mom), Elton and me under the photo section and Joyce had asked about Kennedys MVD. I would like to say thank you to all of those that have offered help and guidence for my friend here at Cavalier Talk but I want to say just how helpful it was that I wrote some of this down and how that has led to where he is now. Just a recap, Kennedys main problem was that he would wake up scared and not able to breath. His vet said he had grade 3 and the MVD was
Former First Lady of the United States, late Jacqueline Kennedy Onnasis, was so worried about her first daughter Caroline Kennedy s weight.
Carly Simon was surprised to learn that her pal, Jackie Kennedy Onassis, was willing to open up about some of the heartaches she endured with her first husband, President John F. Kennedy.
In the Washington Post, Sarah Kaufman reports on David Hallbergs interview with Kennedy Center director Michael Kaiser on Saturday, September 7, 2013 in the Eisenhower Theatre ...
Buddy Guy, Dustin Hoffman, David Letterman, ballerina Natalia Makarova and rockers Led Zeppelin have been named recipients of this years Kennedy Center Honors.
Merrit Kennedy is a reporter for The Two-Way, NPR's breaking news blog. She covers a broad range of issues, from the latest developments out of the
You know the game has forever changed when Patrick Kennedy volunteers to leave the stage. Lee Siegel on the sea change in how personal our politics have become.
A unique approach to connecting people and ideas in the life sciences, creating a space for probing conversations and deep insight into the topics and trends shaping our future.
ЛОНДОН - Мы знаем, как искоренить полиомиелит. Действующая с 1980-х годов международная программа вакцинации под руководством Всемирной организации здравоохранения привела к почти полному исчезновению вируса полиомиелита. Болезнь, которая ежегодно убивала или парализовала полмиллиона людей на земле, поражает сегодня только несколько сот человек во всем мире.. Препятствие, стоящее сегодня на пути уничтожения вируса, не связано с медицинскими или техническими ограничениями, а вызвано политическим сопротивлением вакцинации. Действительно, ...
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Comprehensive outpatient clinic providing multidisciplinary consultation, counseling, rehabilitation, treatment and case management/referral services for people affected by neuromuscular disorders, such as ALS (Lou Gehrigs disease),Spinal Muscular Atrophy, Inclusion Body Myositis, Myotonic Muscular Dystrophy, Limb-Girdle Muscular Dystrophy, Hereditary Neuropathy, Friedreichs Ataxia, Myasthenia Gravis, Spinal/Bulbar Muscular Atrophy (Kennedys Disease), Charcot-Marie Tooth Disease, Dermatomyositis and Polymyositis. Services provided by Board-Certified Neurologist and Clinical Neurophysiologist Gregory P. Hanes, MD, and Sarasota Memorials team of neuroscience professionals ...
Berberine, a plant-derived compound, was seen to clear motor neurons of the toxic protein clumps found in adults with spinal and bulbar muscular atrophy.
NEURODEGENERATIVE DISEASE. Purging X-tra protein aggregates. Adenylyl cyclase activating polypeptide modifies androgen receptor phosphorylation and spinal and bulbar muscular atrophy pathogenesis.. AUTOIMMUNITY. Toning down T cell signaling to treat autoimmunity. A novel inhibitor of interactions between signaling proteins in T cells demonstrates promising preventive and therapeutic effects in several models of autoimmune disease. ...
In January 2013, the Strikeforce organization was closed by its parent company Zuffa. A list of fighters scheduled to be brought over to the Ultimate Fighting Championship was released in mid-January and Kennedy was one of the fighters listed.[22] Kennedy faced Roger Gracie on July 6, 2013, at UFC 162, he defeated Gracie via unanimous decision after defending Gracies submission attempts and standing up with Gracie, out striking him.[23] Kennedy was expected to face Lyoto Machida on November 6, 2013, at UFC Fight For The Troops 3.[24] However, Machida was pulled from the bout in favor of a matchup with Mark Muñoz on October 26, 2013, at UFC Fight Night 30, after Muñozs original opponent, Michael Bisping was forced out of their bout with an injury.[25] Kennedy instead faced Rafael Natal in the event headliner.[26] He won the fight via knockout in the first round. The win also earned him his first Knockout of the Night bonus award.[27] For his third fight with the promotion, Kennedy faced ...
Published in National Review Online: Critical Condition, August 31, 2009. Senator Ted Kennedy was both respected and liked by colleagues on both sides of the aisle during his remarkable 47 years in the Senate.. While he always was firm in his liberal views and I seldom agreed with him, Senator Kennedy did listen to his Republican colleagues and worked to forge compromises. That bipartisan spirit has been markedly missing during his absence from the Senate this year. The health-reform legislation making its way through Congress is rigid and aggressively liberal, without any evidence of bipartisanship, and it is rightly facing a firestorm of opposition.. Senator Robert Byrd has asked that the bill be named after Senator Kennedy in hopes that will revitalize its chances of passage. But I think it is too late. The American people understand the huge impact this legislation would have on the lives of 300 million Americans, and they are not going to be swayed by sentiment.. Shortly before Senator ...
Listen to 2 Bad by Dom Kennedy Dom Kennedy releases the first official single from his upcoming album, By Dom Kennedy which drops on June 2nd. The record
Duncan Kennedy News. Find breaking news, commentary, and archival information about Duncan Kennedy From The tribunedigital-chicagotribune
Legendas The Kennedys Life Sentences - Legendas portuguese (br). The.Kennedys.Part.5.DSR.XviD-SYS, Minissérie em 8 capítulos. Tradução: Equipe Musk3teers. 1CD (pob). Enviada 2011-04-21, baixado 591x.
When we got back to her individual room, we undressed her top so monitors could be placed. The radiology doctor explained the procedure and answered all of my questions including my question about contrast. I had read that it is usually used in MRIs looking for tethered cord. She said yes, it would likely be used. She explained the IV medicine and its effects and also offered us Versid (I do not know how to spell medicines!) which was an oral relaxation medicine. I was hesitant to give her extra medication but when I considered how active Kennedy is, I decided it might be best to give her the Versid to help her relax when the IV is placed. My decision was a good one and I do recommend that to other families considering this option. I understand it may also be an option before her surgery. She calmed to the point that she was just laying in my arms but awake and content. She did not fight the IV placement. She did however, fight the IV medication/sedation. She fought hard but Mommy was kind of ...
Professor Fiona Powrie, Director of the Kennedy Institute has been awarded an honorary degree of Doctor of Science from the University of Bath.
The cheapest way to get from Kennedy to Washington costs only $41, and the quickest way takes just 5 hours! Find the travel option that best suits you!
Obviously, the political world is focused on the health of Ted Kennedy, and not the potential political implications of his diagnosis with a malignant brain tumor.
Every city has its celebrated malcontents, and among San Franciscos finest were the Dead Kennedys. Led by the articulately confrontational…
Kennedy family said in a statement, "Weve lost the irreplaceable center of our family and joyous light in our lives, but the inspiration of his faith, optimism, and perseverance will live on in our hearts forever. He loved this country and devoted his life to serving it ...
Dr. Susan M Kennedy, DO, rated 4.7/5 by patients. 59 reviews, Phone number & practice locations, Family Doctor in West Des Moines, IA.
http://www.dallasnews.com/sharedcont...m.37afff4.html My friends and I think this falls under the category of Bizarre and Off-beat News. First of all, there is a photo from 1963 of the room where Kennedy was treated and which cannot be touched or seen by the public since the government took possession of it. From the article, in case it is archived soon: QUOTE : A piece of JFK assassination history now lies buried in the most unlikely of places: a former limestone quarry in
Lesa France Kennedy, CEO (and vice-Chair of the board) ofINTERNATIONAL SPEEDWAY INC, a company that owns racetracks and runs NASCAR races. She was named the
Upon entering office in 1961, President John F. Kennedy was tasked with the challenge of maintaining the upper hand in the United States battle...
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KPDS KASIM sorularda, cümlede boş bırakılan yerlere uygun düşen kelime veya ifadeyi bulunuz. 1. A fresh look at the final months of the presidency of John F. Kennedy ---- that a second Kennedy
Background on Disease Markers The single nucleotide polymorphism (SNP), 137852571, that is being examined in this experiment is linked with Androgen Insensitivity Syndrome and Kennedy Spinal and Bulbar Muscular Atrophy. Androgen Insensitivity Syndrome occurs when a person who is genetically male (who has one X and one Y chromosome) is resistant to male hormones (called androgens). As a result, the person has some or all of the physical traits of a female, but the genetic makeup of a male. The mutation on the X chromosome makes the body unable to respond to the hormones that produce a male appearance. Kennedy Spinal and Bulbar Muscular Atrophy is a debilitating neurodegenerative disease resulting in muscle cramps and progressive weakness due to degeneration of motor neurons in the brain stem and spinal cord. The SNP is located on the X chromosome and affects the gene AR, the gene is inherited in an x-linked recessive manner therefore only males can be fully affected by the mutation and females ...
PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of Health (NIH) free digital archive of biomedical and life sciences journal literature and is a member of the broader PMC International (PMCI) network of e-repositories.
Lisa bessette photos. carolyn bessette driver s license, carolyn bessette and john f kennedyautopsy reports, carolyn bessette kennedy autopsy photos, bessett hound. Autopsyfiles.org is a website dedicated in providing autopsy reports disclosing. John F. Kennedy Jr., his wife and his sister-in-law died instantly when their plane, plummeting at nearly 60 mph, slammed into the waters off Marthas. Carolyn bessette autopsy photos Carolyn bessette kennedy death photos. lisa bessette photos, carolyn bessette kennedy morgue photos, aaliyah autopsy photoscarolyn bessette autopsylisa left eye lopaz. Carolyn bessette autopsy : Carolyn bessette autopsy picture. carolyn bessette kennedy wedding dress photos, carolyn bessette images, carolyn bessette. Bessett autopsy. carolyn bessette photo autopsy, carolyn bessette death photo, carolyn bessette kennedy death photos, carolyn bessette kennedy austopsy, carolyn ...
Perhaps the most judicious appreciation of Kennedy has come from a man who might have been expected to have been a critic, the socialist Michael Harrington, who said in 1973: "The claim I make for his historic significance is both restrained and major. Within the limits of the possible, as defined by his own pragmatic liberalism and the reactionary congressional power arrayed against it, he developed to a surprising degree. How far he would have gone, we will never know. (Robert Kennedy, who survived his brother by a little less than five years, spent them in making the most extraordinary pilgrimage a practicing politician has ever traveled. What if John Kennedy had had those years too?) John F. Kennedy … must be judged not as a shining knight nor as a failed hero but as a man of his time and place.… He was not, of course, a radical and it is silly to accuse him, as some of his disillusioned followers have, of not having carried out basic transformations of the system. That was never his ...
Senator Edward Kennedys diagnosis of brain cancer, in May 2008, touched off an extraordinary medical battle-and a veiled rivalry over who might succeed him as symbolic head of Americas fabled dynasty. Would it be R.F.K.s oldest son, Joe? J.F.K.s daughter, Caroline? Or the senators second wife, Victoria? An excerpt from the new book Ted Kennedy: The Dream That Never Died reveals the familys shifting dynamics, the confrontation that led Caroline to drop her political bid, and the triumphant, grueling winter of the last Kennedy brother.
Tucker Carlson, took his life and his career in his hands on live TV on FoxNews this week, to cover a high-money artificially taboo topic, just as he is set to advance from the Queens seat, formerly Megyn Kellys time slot, to the Kings seat, formerly Bill OReillys time slot. I hope you know what youre doing, Tucker, because the Murdochs have been (figuratively) lopping off heads, ratings be damned, at the behest of sponsors, many of whom (pharmaceuticals) are known to have no qualms about knowingly (and literally) killing peoples children via vaccines. So the vaccine industry would certainly have no qualms about literally coming after your head, too. So Carlson interviewed Robert F. Kennedy Jr. on Thursday (04/22/2017) about his vaccine safety campaign and research at the World Mercury Project, and gave him the floor. What Mr. Kennedy had to say is of worldwide extreme importance, so please listen to him, although it is extremely difficult to listen to him, owing to Mr. Kennedys voice ...
Conservative Fox News host Tucker Carlson had Robert F. Kennedy Jr. on his show last night (7/10/17). Acknowledging that Kennedy is a liberal democrat Carlson
December 15, 2008 Caroline Kennedy wants Senate seat, sources say (CNN) -- Caroline Kennedy, the 51-year-old daughter of President John F. Kennedy, has made it clear to high-level Democrats that she wants to be the next senator from New York, a source close to Kennedy has told CNN's John King. ...
Connect with Dr. Marc Kennedy, Gastroenterology, Racine, WI. Video chat, send a message, ask a text question, or make a virtual appointment on the doctors Virtual Practice on HealthTap.
Entertainment website TMZ got a surprising old photo that looks like John F. Kennedy (JKF) on a boat with a bunch of naked women probably taken in the mid-1950s. Very interesting, right!! But sadly to say the scandalous picture is a FAKE ...
Nov. 18 - Caroline Kennedy visits the Japanese Foreign Ministry for first official meetings with Japanese diplomats as newly appointed U.S. ambassador to Japan. Rough Cut (no reporter narration).
Celebrities from the Hudson Valley and beyond walked the red carpet at a Monday night gala for the Robert F. Kennedy Center for Justice and
The prognosis and treatment for Edward Kennedy are uncertain, doctors say. The 76-year-old senator was diagnosed today with brain tumor known as a malignant glioma-the same diagnosis some 9,000... Politics News Summaries. | Newser
Find historical values for graded 1898 Cameo Pepsin A. F. Kennedy #70 baseball cards by viewing prices sold on eBay and major auctions.
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Visit Healthgrades for information on Dr. Willard Kennedy, MD Find Phone & Address information, medical practice history, affiliated hospitals and more.
CAIN: Issues: Violence: Prof. Liam Kennedy (2001) They Shoot Children Dont They?: An Analysis of the Age and Gender of Victims of Paramilitary
HEFNER: Well, I think it was, you know, as with Lenny, you know, the combination of the insights and the willingness to break the boundaries. I remember, and of course his act didnt really turn radical until the early 70s, but in 1961, he was playing the Playboy Club with his partner, Jack Burns, and I took Joe Kennedy -- we had had dinner together in Chicago and I took Joe Kennedy up to the Playboy Club in Chicago to see the show, and Burns and Carlin were on stage. They did a little rift on JFK and Joe Kennedy was not amused. KING: No, I dont imagine he would be. You, I look forward to seeing you soon. I wish we had more time but I thank you very much for your memories of George Carlin. Bill Maher, how will he be remembered ...
Date: Tue, 21 May 1996 21:06:33 -0500 From: "Colin P. A. Kennedy" ,[email protected], Message-Id: ,[email protected], To: [email protected], [email protected] Subject: Re: Web neurons There is a reason for CGI, you know ...
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Dawn wrote: Interesting thread too. But the Kennedys DID NOT kill MM. She was murdered to be sure. And it was to balme it on the Kennedys. Was not a suici...
FRAGMENTO. Fluckieger en 1884 fue el primero en describir la asociaci n entre da o hep tico y alteraciones en la funci n pulmonar al describir un caso caracterizado por cianosis, dedos en palillo de tambor y cirrosis hep tica. El t rmino de s ndrome hepatopulmonar (SHP), fue acu ado en 1977 por Kennedy y Knudson.1 ...
Health,Scientists have found that turmeric a widely used ingredient in the I...ASC-J9 a synthetic chemical compound derived from curcuma longa ...Curcumin a pigment of the spice turmeric has been shown to aid in t...Thus there are several citations to turmerics therapeutic propertie... But the new found turmerics efficacy against Kennedys disease is ...,Turmeric,,in,the,Fight,Against,,Prostate,Cancer,medicine,medical news today,latest medical news,medical newsletters,current medical news,latest medicine news
The goal of U.S. policy when Kennedy was elected was combating the spread of Communism in Asia and Africa. And the first foreign policy crisis faced by President-elect John F. Kennedy was not in any of the better-known hot spots of the Cold War, but in landlocked, poverty stricken Laos. This was the major issue Kennedy and his foreign policy team-Secretary of State Dean Rusk, Secretary of Defense Robert S. McNamara, and National Security Advisor McGeorge Bundy-focused on during the days leading up to Kennedys inauguration on January 20, 1961. Kennedy met with President Eisenhower the day before his inauguration and his substantive focus was on Laos. "I was anxious," he recounted, "to get some commitment from the outgoing administration as to how they would deal with Laos which they were handing to us.". The Eisenhower administration was leaving Kennedy a confused, complex, and intractable situation. Laos was a poverty-stricken and weak state, but was the "cork in the bottle," as Eisenhower ...
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Bob Kennedy grew up on the South Side of Chicago and worked as a vendor at Comiskey Park as a youth. He made his big league debut for Chicago White Sox as a 19-year old in 1939. Kennedy entered Naval Aviation in October 1942, was transferred to the Marines and was discharged in January 1946. In Korea, he gave flying lessons to fellow fighter jockey Ted Williams. After a playing career that lasted nearly two decades, Kennedy was the Cleveland Indians assistant farm director in 1960-1961. He was part of the Chicago Cubs College of Coaches as a coach in 1962, then went on to manage the team from 1963to 1965, and the Oakland Athletics in 1968. From 1970 to 1972, Kennedy was the Director of Player Development for the St. Louis Cardinals and was the assistant GM of the Cards from 1973 to 1975. He became General Manager of the Cubs (from 1977 to May 1981). Kennedy moved to the Houston Astros as assistant GM from 1982 to 1985, and held the same post with the San Francisco Giants from 1986 to 1992. ...
The Assassination of John F. Kennedy and the subsequent conspiracy theories surrounding his death have been discussed, referenced, or recreated in popular culture numerous times. John F. Kennedy (May 29, 1917 - November 22, 1963) was an American politician who served as the 35th President of the United States from January 20, 1961 until his assassination on November 22, 1963 by Lee Harvey Oswald, who was killed by Jack Ruby two days later. The fictional novel Gideons March by J. J. Marric, was published in 1962 by Hodder and Stoughton in London, the year before the Kennedy assassination. Inspector George Gideon learns of a plot to assassinate President Kennedy during a state visit to London. The assassination is to take place during a parade, by means of a bomb; the assassin, called OHara, is a Southern bigot who hates the President for his Roman Catholic faith and his civil-rights initiatives. J. G. Ballard wrote a 1967 short-short story titled "The assassination of John Fitzgerald Kennedy ...
Officeholder ,name=John F. Kennedy ,image=John_kennedy.jpg ,party=[[Democratic]] ,spouse=[[Jacqueline Kennedy,Jacqueline Lee Bouvier Kennedy]] ,religion=[[Roman Catholic]] ,offices= {{Officeholder/president ,country=the United States ,number=35th ,terms=January 20, 1961 - November 22, 1963 ,vp=[[Lyndon Johnson]] ,preceded=[[Dwight Eisenhower]] ,former=y ,succeeded=[[Lyndon Johnson]] }} {{Officeholder/senator ,state=Massachusetts ,terms=January 3, 1953 - December 22, 1960 ,preceded=Henry Cabot Lodge, Jr. ,former=y ,succeeded=Benjamin A. Smith }} {{Officeholder/representative ,state=Massachusetts ,district=11th ,terms=January 3, 1947 - January 3, 1953 ,preceded=James Michael Curley ,former=y ,succeeded=Thomas P. ONeill, Jr. }} }} John Fitzgerald Kennedy, often called JFK, (May 29, 1917 - November 22, 1963) was the 35,sup>th,/sup> [[President of the United States of America]], serving for 1033 days in 1961-63. He was the youngest person elected president and one of the most glamorous ...
The Kennedys and staff at the White House watching the first US manned space flight by astronaut Alan Shepard, on 5th May, 1961. From left to right are: Vice President Lyndon Johnson, Arthur M. Schlesinger, Jr., Admiral Arleigh Burke, President Kennedy, Mrs. Kennedy. - Stock Image C030/8834
QUESTION. I would like to ask the Senator what his reaction to this entire midwestern trip is, and how much of the Midwest does he think he can carry. Senator KENNEDY. First, I want to express my appreciation to Governor Loveless and to all of you. My reaction to this trip is that it has been most useful and worth while. As I said earlier this afternoon, the decline in agricultural income is the No. 1 domestic problem of the United States. This meeting held today, and the only one that we shall hold in the month of August, I think indicates the importance that we put in demonstrating that the Democratic Party is determined to reverse this downward curve. We are going to move it upward, and I think that in November we will be endorsed in this view by the farmers of the Midwest. QUESTION. Senator Kennedy, you heard the milk comments of Governor Freeman. Are you for that type of a program that will enable the midwestern milk to get into the big eastern markets? Governor LOVELESS. The question was, ...
We are now in a regular floor room at Duke and have had wonderful nurses and NAs since her arrival to the floor. Kennedy continues to fight the effects of the anasthesia and is having a hard time sleeping because as soon as she falls asleep, she twitches - which hurts and then she cries and is awake again. Since 12:15am, shes gotten more sleep and seems to be doing a little better; however, now her temp is rising. It was over 100 F at the last check. The major risk with fever is meningitis since that cord and fluid were exposed. However, Kennedy is also pulling on her right ear like crazy. Since weve had 6 ear infections in the past 10-12 weeks, I would not be surprised if we have another. An order was put in at 4am by Dr. Fuchs resident to do an ENT referral while we are here and flat for 5 days. Poor baby girl ...
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Guest Book for Terence Kennedy of Whittier, CA. Share your condolences, send flowers, find funeral home information. Guestbook provided by Legacy.com.
The construction of the Nuffield Department of Medicine and the Kennedy Institute of Rheumatology is enabling state of the art biomedical research into auto-immune diseases and inflammatory conditions.
Playwright Larry Kramer caused more Heart trouble over the weekend.Kramer, outspoken author of the AIDS drama The Normal Heart, Friday charged that politics caused the Kennedy Center to back off
Describing the American healthcare system as a mess, U.S. Congressman Patrick J. Kennedy (D-RI) yesterday unveiled new legislation that he says would reduce costs in the American healthcare industry and improve clinical outcomes for patients by 2015.
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Detroit, MI (PRWEB) December 26, 2012 -- Kennedy University, a renowned education provider, has established a Learning Development Center to foster faculty and
When PPP conducted a survey last month about the New York Senate vacancy, we only interviewed Democrats. This time we interviewed all voters, so to make a complete apples to apples comparison of how folks are looking at Kennedy now compared to a month ago we need to look at her standing on a few key indicators just within her own party ...
From KIIS FMs Jingle Ball to the 38th Annual Kennedy Center Honors Gala -- the stars stepped out this weekend looking as gorgeous and chic as can be.
Supreme Court Justice Anthony Kennedy wants to preserve the gains of his 2015 decision establishing a right to same-sex marriage.
The announcement comes after the remaining members of the Presidents Committee on the Arts and the Humanities resigned on Friday.
Illinois businessman Chris Kennedy, son of Robert F. Kennedy and nephew of US President John F. Kennedy, will join the states Democratic race for governor.
But then a news broadcast came on and JFK leaped up, ran to the radio, and turned up the volume to listen to it. Offended, Beckwith threw him out. Another curious observation that the book establishes is that Kennedy did not smoke and was only a social drinker. So if, as I detailed in the Mary Meyer tale, Kennedy ended up a White House coke-sniffer and acid head, it was a definite break with the past. The Blairs book established some paradigms that would be followed in the anti-Kennedy genre. First, and probably foremost, is the influence of Kennedys father in his career. In fact, Joe Kennedys hovering presence over all his children is a prime motif of the book. The second theme that will be followed is the aforementioned female associations. The third repeating pattern the Blairs established is the use of Kennedys health problems as some kind of character barometer. That because Kennedy and his circle were not forthright about this, it indicates a covert tendency and a penchant for ...
The San Jose Sharks acquired forward Tyler Kennedy from the Pittsburgh Penguins for a second-round pick at Sundays NHL Draft.The Sharks sent the 50th overall selection in this years draft to Pittsburgh for Kennedy, who had spent his entire career with the Penguins
If Justice Kennedy retires at the end of this coming SCOTUS term, as he is rumored to, certain types of pro-equality decisions will be at risk.
Kerry Kennedy, ex-wife of New York Gov. Andrew Cuomo, was swaying and slurring her speech when police found her after her car hit a tractor-trailer on a highway, police said in a court document.
Fifty years ago, Jacqueline Bouvier Kennedy found herself thrust into a role she had never planned for-she became the nations first mourner-in-chief. ...
Had the sturdy New England stock that coursed through the Irish bloodlines of John Fitzgerald Kennedy not been drained by an assassins bullet, imagine what he would be thinking about world affairs.
FEW children relish the thought of going to school, but for teenager Joy Kennedy it is a delight compared with the prospect of weeks spent in hospital.
Although he was an expert skier on an intermediate slope, Michael Kennedy was taking uncommon risks, fitting the profile of an all-too-average skiing fatality.Like the son of Robert F. Kennedy, most
Baby Sensory - Baby Development Classes. Classes run weekly in Surrey at Baby Sensory Barn, at Applegarth Farm, in Grayshott - near Haslemere, Hindhead and Liphook. Baby Sensory classes include Baby Signing, baby music, light shows, bubbles, puppets and sensory experiences to help your baby develop.
My guess is that most readers of this blog hadnt been born in November 1963 when Kennedy was assassinated, or April 1968 for the assassination of Martin Luther King, or November of that same year when Bobby Kennedy was killed. The media had a political field day with these three tragedies, and it is terrible…
Join Ashley Kennedy for an in-depth discussion in this video Displaying event data, part of Final Cut Pro X 10.0.9 Essential Training
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Objectives: According to recent publications, some patients with spinal and bulbar muscular atrophy (BSMA) develop cardiac disease, manifesting as ST-segment abnormalities, Brugada-syndrome, dilative cardiomyopathy, or sudden cardiac death. Here we present neurological and cardiac data of a BSMA patient who was followed up for 10 y. Case report: In a male patient aged 47 y, BSMA was diagnosed at age 37 y upon the typical clinical presentation (postural tremor since age 12 y, dysarthria since age 15 y, muscle cramps since age 29 y, general myalgias since age 32 y, general fasciculations since age 34 y, myoclonic jerks, easy fatigability, dyspnea upon exercise since age 36 y) and a CAG-repeat expansion of 47 ± 1 repeats in the androgen-receptor gene detected at age 37 y ...
Objectives: According to recent publications, some patients with spinal and bulbar muscular atrophy (BSMA) develop cardiac disease, manifesting as ST-segment abnormalities, Brugada-syndrome, dilative cardiomyopathy, or sudden cardiac death. Here we present neurological and cardiac data of a BSMA patient who was followed up for 10 y. Case report: In a male patient aged 47 y, BSMA was diagnosed at age 37 y upon the typical clinical presentation (postural tremor since age 12 y, dysarthria since age 15 y, muscle cramps since age 29 y, general myalgias since age 32 y, general fasciculations since age 34 y, myoclonic jerks, easy fatigability, dyspnea upon exercise since age 36 y) and a CAG-repeat expansion of 47 ± 1 repeats in the androgen-receptor gene detected at age 37 y ...
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"The lateral corticospinal tract and spinal ventral horn in X-linked recessive spinal and bulbar muscular atrophy: a ... multiple system atrophy and X-linked recessive bulbospinal neuronopathy, with special reference to the loss of small neurons in ... Multiple system atrophy (MSA), has also been linked to the lateral grey column. MSA has been shown to reduce the cell count in ... Muscular atrophy has also been shown to have an effect on neurons of the anterior column. A large loss of large alpha motor ...
This leads to the development of paralytic poliomyelitis, the various forms of which (spinal, bulbar, and bulbospinal) vary ... CS1 maint: Unfit url (link) Accessed 16 December 2009. Spice B (4 April 2005). "Tireless polio research effort bears fruit and ... the muscles atrophy, becoming weak, floppy and poorly controlled, and finally completely paralyzed. Maximum paralysis ... Depending on the site of paralysis, paralytic poliomyelitis is classified as spinal, bulbar, or bulbospinal. Encephalitis, an ...
... is the normal or spontaneous atrophy or shrinkage of the breasts.[1] Breast atrophy commonly occurs in women during menopause when estrogen levels decrease.[2][3][4] It can also be caused by hypoestrogenism and/or hyperandrogenism in women in general,[1] such as in antiestrogen treatment for breast cancer, in polycystic ovary syndrome (PCOS),[5][6] and in malnutrition such as that associated with eating disorders like anorexia nervosa or with chronic disease.[1][7][8] It can also be an effect of weight loss.[8][9] In the treatment of gynecomastia in males and macromastia in women, and in hormone replacement therapy (HRT) for trans men,[10] breast atrophy may be a desired effect. Examples of treatment options for breast atrophy, depending on the situation/when appropriate, can include estrogens, antiandrogens, and proper nutrition or weight gain.[citation needed] ...
... is defined as a decrease in the mass of the muscle; it can be a partial or complete wasting away of muscle, and is most commonly experienced when persons suffer temporary disabling circumstances such as being restricted in movement and/or confined to bed as when hospitalized. When a muscle atrophies, this leads to muscle weakness, since the ability to exert force is related to mass. Modern medicine's understanding of the quick onset of muscle atrophy is a major factor behind the practice of getting hospitalized patients out of bed and moving about as active as possible as soon as is feasible, despite sutures, wounds, broken bones and pain. Muscle atrophy results from a co-morbidity of several common diseases, including cancer, AIDS, congestive heart failure, COPD (chronic obstructive pulmonary disease), renal failure, and severe burns; patients who have "cachexia" in these disease settings have a poor prognosis. Moreover, starvation eventually leads to muscle ...
... , or corticosteroid-induced dermal atrophy, is a side effect of prolonged application of topical corticosteroids. The potential for the condition exists whenever topical corticosteroids are used, even with low potency preparations. Skin atrophy, along with other undesirable side effects such as telangectasia and striae, can appear within 2 to 3 weeks of starting daily application of Class I and II topical corticosteroids, the greatest potential occurring when the application is occluded or when the preparation is applied to fragile skin. Risk depends on the strength of the steroid, the length of application, the site treated, and the nature of the skin problem. Within two weeks of starting Topical Steroid treatment, and probably within a few days, microscopic degenerative changes may be seen in the epidermis with a reduction of cell size and the number of cell layers. These effects may be rapidly reversible but with chronic administration, dermal changes become apparent. ...
... 1 (LCCS1), also called Multiple contracture syndrome, Finnish type, is an autosomal recessive genetic disorder characterized by total immobility of a fetus, detectable at around the 13th week of pregnancy. LCCS1 invariably leads to prenatal death before the 32nd gestational week. LCCS1 is one of 40 Finnish heritage diseases. It was first described in 1985 and since then, approximately 70 cases have been diagnosed. LCCS1 is characterized by total lack of the movements of the fetus, and is detectable at 13th week of pregnancy. It is accompanied by oedema, small chin, small lungs, crooked joints and occasional skin webs of the neck and elbows. The fetus has characteristic pattern of malpositions recognizable even in severely macerated fetuses with club feet and hyperextension of the knees but the elbows and wrists showing flexion contractures. Neuropathological analysis shows lack of anterior horn motoneurons and severe atrophy of the ventral spinal cord. The ...
... , also known as subcortical leukoencephalopathy, is a form of small vessel vascular dementia caused by damage to the white brain matter. White matter atrophy can be caused by many circumstances including chronic hypertension as well as old age. This disease is characterized by loss of memory and intellectual function and by changes in mood. These changes encompass what are known as executive functions of the brain. It usually presents between 54 and 66 years of age, and the first symptoms are usually mental deterioration or stroke. It was described by Otto Binswanger in 1894, and Alois Alzheimer first used the phrase "Binswanger's disease" in 1902. However, Olszewski is credited with much of the modern-day investigation of this disease which began in 1962. Symptoms include mental deterioration, language disorder, transient ischemic attack, muscle ataxia, and impaired movements including change of walk, slowness of movements, and change in posture. These symptoms usually ...
... , or estrogen deficiency, refers to a lower than normal level of estrogen, the primary sex hormone in women. In general, lower levels of estrogen may cause differences in the breasts, genitals, urinary tract, and skin. Hypoestrogenism is most commonly found in women who are postmenopausal, have premature ovarian failure, or are suffering from amenorrhea; however, it is also associated with hyperprolactinemia and the use of gonadotropin-releasing hormone (GnRH) analogues in treatment of endometriosis. It has also been linked to scoliosis and young women with type 1 diabetes mellitus. Presentations of low estrogen levels include hot flashes, headaches, lowered libido, and breast atrophy. Reduced bone density leading to secondary osteoporosis and atrophic changes such as pH change in the vagina is also linked to hypoestrogenism. Low levels of estrogen can lead to dyspareunia and limited genital arousal because of changes in the four layers of the vaginal ...
... (27 March 1845 - 10 May 1917) was a French neurologist from Reims, and whose father and grandfather were also physicians.. He studied medicine in Reims and Paris, earning his doctorate in 1876. He spent much his career at the University of Paris, becoming a professor of therapy in 1893 and a dean of medicine in 1901.. His name is associated with the "Landouzy-Dejerine syndrome", a type of muscular dystrophy with atrophic changes to the facial muscles and scapulo-humeral region. It is named along with neurologist Joseph Jules Dejerine, who was a colleague and close friend. Landouzy was a witness at the wedding of Dejerine to Augusta Marie Klumpke (1859-1927) in 1888.. Landouzy's primary area of interest dealt with tuberculosis, and he was a major figure involving education of the public for its eradication. He was a member of several international committees in regards to tuberculosis.. He is credited with coining the word "camptodactyly" to describe a flexion ...
Over the past two decades, hundreds of studies have shed light on the neuroanatomical structural correlates of neurological and psychiatric disorders. Many of these studies were performed using voxel-based morphometry (VBM), a whole-brain technique for characterizing between groups'regional volume and tissue concentration differences from structural magnetic resonance imaging (MRI) scans.[5]. One of the first VBM studies and one that came to attention in mainstream media was a study on the hippocampus brain structure of London taxicab drivers.[6] The VBM analysis showed the back part of the posterior hippocampus was on average larger in the taxi drivers compared to control subjects while the anterior hippocampus was smaller. London taxi drivers need good spatial navigational skills and scientists have usually associated hippocampus with this particular skill. Another famous VBM paper was a study on the effect of age on gray and white matter and CSF of 465 normal adults.[7] The VBM analysis ...
At the time the High-Risk-for-Schizophrenia study began, in 1962, the offspring of the women with schizophrenia were average age 15 and had not come into the risk period for schizophrenia. (See a review by[3] Cannon and Mednick, 1993). By the early eighties, many of the study's subjects had fallen ill with schizophrenia. Colleagues and students of Mednick began to examine the association between schizophrenia outcomes and earlier risk factors. Perhaps the first study to support Kraepelin's notion of dementia praecox (that persons with schizophrenia had early dementia), was a study that showed that offspring of those with schizophrenia who had the most serious symptomatology had enlarged ventricles on CT scans suggestive of brain atrophy. In a study by Silverton et al. those with the most severe schizophrenia symptoms on outcome had low birthweights decades before.[4] Their hypothesis that low birthweight might be associated with insults in utero was corroborated. In a follow-up study, the ...
A spinal cord injury (SCI) is damage to the spinal cord that causes changes in its function, either temporary or permanent. These changes translate into loss of muscle function, sensation, or autonomic function in parts of the body served by the spinal cord below the level of the lesion. Injuries can occur at any level of the spinal cord and can be classified as complete injury, a total loss of sensation and muscle function, or incomplete, meaning some nervous signals are able to travel past the injured area of the cord. Depending on the location and severity of damage along the spinal cord, the symptoms can vary widely, from pain or numbness to paralysis to incontinence. The prognosis also ranges widely, from full recovery in rare cases to permanent tetraplegia (also called quadriplegia) in injuries at the level of the neck, and paraplegia in lower injuries. Complications that can occur in the short and long term after ...
A spinal tumor is when unusual tissue begins growing and spreading in the spinal columns or spinal cords. The unusual tissue builds up from abnormal cells that multiply quickly in a specific region. Tumors generally are broken down into categories known as benign, meaning non-cancerous, or malignant, meaning cancerous, and also primary or secondary. Primary spinal tumors begin in either the spinal cord or spinal column, whereas secondary spinal tumors begin elsewhere and spread to the spinal region.[9] Symptoms for spinal tumors may vary due to factors such as the type of tumor, the region of the spine, and the health of the patient. Back pain is the most common symptom and it can be a problem if the pain is severe, has a time frame that lasts longer than it would for a normal injury, and becomes worse while laying down or at rest. Other symptoms, excluding ...
The chemokine (C-X-C motif) ligand 1 (CXCL1) is a small cytokine belonging to the CXC chemokine family that was previously called GRO1 oncogene, GROα, KC, neutrophil-activating protein 3 (NAP-3) and melanoma growth stimulating activity, alpha (MSGA-α). In humans, this protein is encoded by the CXCL1 gene. CXCL1 is secreted by human melanoma cells, has mitogenic properties and is implicated in melanoma pathogenesis. CXCL1 is expressed by macrophages, neutrophils and epithelial cells, and has neutrophil chemoattractant activity. CXCL1 plays a role in spinal cord development by inhibiting the migration of oligodendrocyte precursors and is involved in the processes of angiogenesis, arteriogenesis, inflammation, wound healing, and tumorigenesis. This chemokine elicits its effects by signaling through the chemokine receptor CXCR2. The gene for CXCL1 is located on human chromosome 4 amongst genes for other CXC chemokines. An initial study in mice showed evidence that CXCL1 decreased the severity of ...
புற நரம்பு மண்டலம் அல்லது புற நரம்புத் தொகுதி (Peripheral Nervous System - PNS) எனப்படுவது, நரம்புத் தொகுதியின் இரு பெரும் பிரிவுகளில் ஒன்றாகவும், அத் தொகுதியின் மைய நரம்பு மண்டலம் தவிர்ந்த ஏனைய உணர் நரம்புக் கலங்களையும், அவற்றை நரம்பு நாண்கள் (nerve cord), முண்ணாண் (spinal cord), மூளை என்பவற்றுடன் இணைக்கும் நரம்புகளையும் கொண்டது. மையநரம்புத் தொகுதியுடன், கண், காது போன்ற உணர் உறுப்புக்களையும், தசைகள், ...
Kennedys Disease (Bulbospinal Muscular Atrophy, X-Linked Spinal and Bulbar Muscular Atrophy). Kennedys disease is a rare ... Spinal bulbar muscular atrophy or SBMA. Symptoms tend to develop most commonly in middle age, but it is possible for them to ... Kennedys disease is classed as an X-linked condition, which is caused by a genetic mutation in a gene responsible for ... producing the protein known as AR (androgen receptor). X-linked disorders are related to the X chromosome. Normally, a person ...
z linked bulbo spinal atrophy, c linked bulbo spinal atrophy, d linked bulbo spinal atrophy, s linked bulbo spinal atrophy, x ... zx linked bulbo spinal atrophy, xz linked bulbo spinal atrophy, cx linked bulbo spinal atrophy, xc linked bulbo spinal atrophy ... dx linked bulbo spinal atrophy, xd linked bulbo spinal atrophy, sx linked bulbo spinal atrophy, xs linked bulbo spinal atrophy ... x l inked bulbo spinal atrophy, x li nked bulbo spinal atrophy, x lin ked bulbo spinal atrophy, x link ed bulbo spinal atrophy ...
"Bulbo-Spinal Atrophy, X-Linked" by people in this website by year, and whether "Bulbo-Spinal Atrophy, X-Linked" was a major or ... Bulbo-Spinal Atrophy, X-Linked*Bulbo-Spinal Atrophy, X-Linked. *Atrophies, X-Linked Bulbo-Spinal ... "Bulbo-Spinal Atrophy, X-Linked" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH ( ... Below are the most recent publications written about "Bulbo-Spinal Atrophy, X-Linked" by people in Profiles. ...
Bulbo-Spinal Atrophy, X-Linked. *Kennedy Disease. *Other: No intervention, observational. Observational. *Rigshospitalet, ... High Intensity Training in Patients With Spinal and Bulbar Muscular Atrophy. *Spinal and Bulbar Muscular Atrophy ... and Efficacy of BVS857 in Patients With Spinal and Bulbar Muscular Atrophy. *Spinal and Bulbar Muscular Atrophy ... Effect of Goserelin (Zoladex®) in Spinal and Bulbar Muscular Atrophy. *Spinobulbar Muscular Atrophy ...
Atrophy. Muscular Atrophy. Motor Neuron Disease. Muscular Disorders, Atrophic. Bulbo-Spinal Atrophy, X-Linked. Pathological ... Spinal Bulbar Muscular Atrophy. Spinal and Bulbar Muscular Atrophy. SBMA. Motor Neuron Disease. ... Objective: Spinal and bulbar muscular atrophy (SBMA), or Kennedy s disease, is a slowly progressive, X-linked motor neuron ... Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology. 1968 Jul;18(7): ...
Muscular Atrophy. Motor Neuron Disease. Amyotrophic Lateral Sclerosis. Bulbo-Spinal Atrophy, X-Linked. Atrophy. Pathological ... Muscular Atrophy, Spinal. Heredodegenerative Disorders, Nervous System. Genetic Diseases, X-Linked. Genetic Diseases, Inborn. ... Kennedy WR, Alter M. Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. J ... Study of Hepatic Function in Patients With Spinal and Bulbar Muscular Atrophy. The safety and scientific validity of this study ...
Bulbo-Spinal Atrophy, X-Linked. Atrophy. Pathological Conditions, Anatomical. Muscular Atrophy, Spinal. Spinal Cord Diseases. ... Resource links provided by the National Library of Medicine Genetics Home Reference related topics: Spinal and bulbar muscular ... It typically starts with muscle spasms and tremors in the arms, followed by muscle weakness and atrophy of muscles in the arms ...
The XBSMA meaning is X-linked bulbospinal muscular atrophy. The definition of XBSMA by AcronymAndSlang.com ... XBSN - X-Linked Recessive Bulbospinal Neuronopathy. *DMD - X-linked muscular dystrophy. *X-BSMA - X-linked bulbar and spinal ... "X-linked bulbospinal muscular atrophy". Q: A: How to abbreviate "X-linked bulbospinal muscular atrophy"?. "X-linked bulbospinal ... What is the abbreviation for X-linked bulbospinal muscular atrophy?. X-linked bulbospinal muscular atrophy can be abbreviated ...
The most common neurodegenerative disease in childhood is spinal muscular atrophy (SMA). The severe infantile type 1 (Werdnig- ... Bulbo-spinal Atrophy, X-linked. An X-linked recessive form of spinal muscular atrophy. It is due to a mutation of the gene ... In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are ... Lumbosacral ventral spinal nerve root atrophy identified on MRI in a case of spinal muscular atrophy type II. ...
Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology. 1968;18(7):671- ... Decremental responses to repetitive nerve stimulation in x-linked bulbospinal muscular atrophy. J Clin Neurol. 2013;9(1):32-35. ... Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature. 1991;352(6330):77-79.. View this ... Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy. Nat ...
1968) Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology 18:671-680 ... 2002) Expression of X-linked bulbospinal muscular atrophy (Kennedy disease) in two homozygous women. Neurology 59:770-772. ... 1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352:77-79. ... 2005) Distal spinal and bulbar muscular atrophy caused by dynactin mutation. Ann Neurol 57:687-694. ...
X-linked, late onset neuromuscular disorder. The disease is caused by a CAG trinucleotide repeat expansion in the first exon of ... X-linked, late onset neuromuscular disorder. The disease is caused by a CAG trinucleotide repeat expansion in the first exon of ... Spinal and bulbar muscular atrophy (SBMA), also known as Kennedys disease, is a rare, ... Spinal and bulbar muscular atrophy (SBMA), also known as Kennedys disease, is a rare, ...
X-linked bulbospinal muscular atrophy (kennedy disease) 60 genetic heterogeneity mutations of different genes cause similar ...
Bulbo-Spinal Muscular Atrophy (Kennedys Syndrome; X-linked). BSMA. Bank Security Management Association. ... Want to thank TFD for its existence? Tell a friend about us, add a link to this page, or visit the webmasters page for free ...
Bulbospinal Muscular Atrophy, X-linked. *Kennedy Disease. *Spinal and Bulbar Muscular Atrophy (SBMA) ... 1968) Progressive proximal spinal and bulbar muscular atrophy of later onset: A sex -linked recessive trait. Neurology 18:671- ... 2008) Spinal and bulbar muscular atrophy: skeletal muscle pathology in male patients and heterozygous females. J Neurol Sci 264 ... 1999; Updated July 2014) Spinal and Bulbar Muscular Atrophy. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews ...
208000006269 X-Linked Bulbo-Spinal Atrophy Diseases 0 claims description 3 * 125000005418 aryl aryl group Chemical group 0 ... 206010068597 Bulbospinal muscular atrophy congenital Diseases 0 claims description 3 * 201000001971 Huntingtons diseases ...
Adult Onset X-Linked SMA, also known as Kennedys Syndrome or Bulbo-Spinal Muscular Atrophy, occurs only in men. Facial and ... Spinal Muscular Atrophy. by Lenny Parracino , Date Released : 17 Dec 2005 0 comments Print ... Spinal muscular atrophy (SMA), the number one genetic killer of children under the age of two, is a group of inherited and ... Would you please be able to give me some information on the disease spinal muscular atrophy? ...
Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology. 1968;18(7):671- ... Sobue G, Hashizume Y, Mukai E, Hirayama M, Mitsuma T, Takahashi A. X-linked recessive bulbospinal neuronopathy. A ... Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature. 1991;352(6330):77-79.. View this ... Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy. Nat ...
muscular atrophy synonyms, muscular atrophy pronunciation, muscular atrophy translation, English dictionary definition of ... muscular atrophy. Translations. English: muscular atrophy n Muskelatrophie f, Muskelschwund m. German / Deutsch: Muskelatrophie ... 4,5) Specific genetic tests are available for X-linked bulbospinal neuronopathy (Kennedys disease), which causes a slowly ... Spinal muscular atrophy (SMA) has plagued humanity for generations.. Facts about spinal muscular atrophy ...
Kennedy syndrome is a late-onset, bulbar-spinal type of muscular atrophy, with X-linked recessive inheritance. The ... Kennedys syndrome - bulbo-spinal muscular atrophy]. SZABÓ Antal, MECHLER Ferenc. [ ... such as gynaecomastia or testicular atrophy. The electrophysiological examinations are the keypoint to the diagnosis. ...
bulbospinal atrophy; X-linked disease from CAG repeat expansion encoding androgen receptor apparently; leads to distal limb ... juvenile spinal muscular atrophy, hereditary or sporadic, affects proximal limbs, gradually progressive. KULCHITSKYS CELLS. ... Related Links · Eponym Books on Amazon.com. ARTICLE TOOLS. · Eponyms section. · Articles by Andrew J. Yee, M.D.. · Add to my ... criteria for linking a specific microorganism to a disease 1. organism must be found in lesion of disease; 2. organism can be ...
Hanemann CO, Sperfeld AD, Karitzky J, Schreiber H, Brummer D, Haussler J & Ludolph AC 2002 X-linked bulbospinal neuronopathy ... RudnikSchoneborn S 1997 Congenital axonal neuropathy caused by deletions in the spinal muscular atrophy region ANNALS OF ... Hanemann CO, Sperfeld AD, Karitzky J, Schreiber H, Brummer D, Haussler J & Ludolph AC 2002 X-linked bulbospinal neuronopathy ... Sperfeld AD, Karitzky J, Brummer D, Schreiber H, Haussler J, Ludolph AC & Hanemann CO 2002 X-linked bulbospinal neuronopathy ...
"The lateral corticospinal tract and spinal ventral horn in X-linked recessive spinal and bulbar muscular atrophy: a ... multiple system atrophy and X-linked recessive bulbospinal neuronopathy, with special reference to the loss of small neurons in ... Multiple system atrophy (MSA), has also been linked to the lateral grey column. MSA has been shown to reduce the cell count in ... Muscular atrophy has also been shown to have an effect on neurons of the anterior column. A large loss of large alpha motor ...
DNA testing for X-linked bulbospinal muscular atrophy was negative, as were antibody titers to HIV-1 and 2, acetylcholine ... Spinal fluid was acellular with normal glucose (68 mg/dl) and protein (21 mg/dl). Gadolinium-enhanced MRI of the brain and ... Unilateral hypoglossal nerve atrophy as a late complication of radiation therapy of head and neck carcinoma: a report of four ... There was diffuse atrophy of the anterior cervical musculature, more prominent on the left, with continuous undulating ...
Kennedys disease, also known as bulbospinal muscular atrophy, is a rare, X -linked recessive neurodegenerative disorder ... Non-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients. ... To carry out a deep characterisation of the main androgen-responsive tissues involved in spinal and bulbar muscular atrophy ( ... The patient was suspicious of AIS family history and the pedigree was made to analyze her family which was possibly X-linked ...
  • PBP is a progressive degenerative disorder of the motor nuclei in the medulla (specifically involving the glossopharyngeal, vagus, and hypoglossal nerves) that produces atrophy and fasciculations of the lingual muscles, dysarthria, and dysphagia. (medscape.com)
  • This type of mutation has also been found to cause a variety of neurodegenerative disorders, termed polyglutamine diseases, such as Huntington's disease (HD), several forms of spinocerebellar ataxia, and dentatorubral pallidoluysian atrophy ( Gatchel and Zoghbi, 2005 ). (jneurosci.org)
  • Polyglutamine-induced transcriptional dysregulation of the dynactin p150 subunit (dynactin 1), an axonal motor-associated protein, resulted in perturbation of retrograde axonal transport in spinal motor neurons in the early stage of the disease. (jneurosci.org)
  • Designed to address the needs of patients suffering from numerous respiratory complications, including chronic obstructive pulmonary disease, pneumonia, severe asthma, pleurisy, muscular atrophy , respiratory failure, neuromuscular disease and other diseases or injuries that impair respiration. (thefreedictionary.com)
  • X-linked disease). (invictagenetics.pl)
  • Kennedy disease is caused by a mutation in the androgen receptor ( AR ) gene and is inherited in an X-linked recessive manner. (cdc.gov)
  • Kennedy disease is inherited in an X-linked recessive manner. (cdc.gov)
  • An x-linked recessive hepatic glycogen storage disease resulting from lack of expression of phosphorylase-b-kinase activity. (umassmed.edu)
  • An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders. (umassmed.edu)
  • An X-linked recessive muscle disease caused by an inability to synthesize DYSTROPHIN, which is involved with maintaining the integrity of the sarcolemma. (umassmed.edu)
  • Machado Jospeh disease= spinocerebellar atrophy tpe 3. (blogspot.com)
  • There are several different types of spinal muscular atrophy: type I (severe, also known as Werdnig-Hoffmann syndrome), type II (intermediate) and type III (mild, also known as Kugelberg-Welander disease) affect children, while there are a variety of types of adult-onset SMA that develop later in life. (taoiststudy.com)
  • Subtle decreases in grey matter volume, mainly localised in frontal areas, were found, but extensive white matter atrophy was observed, particularly in frontal areas, but also involving multiple additional subcortical areas, the cerebellar white matter and the dorsal brainstem from the midbrain to the medulla oblongata. (pubmedcentralcanada.ca)
  • Login to edit your profile (add a photo, awards, links to other websites, etc. (harvard.edu)