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)Superoxide Dismutase: An oxidoreductase that catalyzes the reaction between superoxide anions and hydrogen to yield molecular oxygen and hydrogen peroxide. The enzyme protects the cell against dangerous levels of superoxide. EC 1.15.1.1.Motor Neuron Disease: Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)Motor Neurons: Neurons which activate MUSCLE CELLS.RNA-Binding Protein FUS: A multifunctional heterogeneous-nuclear ribonucleoprotein that may play a role in homologous DNA pairing and recombination. The N-terminal portion of protein is a potent transcriptional activator, while the C terminus is required for RNA binding. The name FUS refers to the fact that genetic recombination events result in fusion oncogene proteins (ONCOGENE PROTEINS, FUSION) that contain the N-terminal region of this protein. These fusion proteins have been found in myxoid liposarcoma (LIPOSARCOMA, MYXOID) and acute myeloid leukemia.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)Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.DNA Repeat Expansion: An increase number of repeats of a genomic, tandemly repeated DNA sequence from one generation to the next.Frontotemporal Dementia: The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.Guam: An island in Micronesia, east of the Philippines, the largest and southernmost of the Marianas. Its capital is Agana. It was discovered by Magellan in 1521 and occupied by Spain in 1565. They ceded it to the United States in 1898. It is an unincorporated territory of the United States, administered by the Department of the Interior since 1950. The derivation of the name Guam is in dispute. (From Webster's New Geographical Dictionary, 1988, p471)Riluzole: A glutamate antagonist (RECEPTORS, GLUTAMATE) used as an anticonvulsant (ANTICONVULSANTS) and to prolong the survival of patients with AMYOTROPHIC LATERAL SCLEROSIS.Frontotemporal Lobar Degeneration: Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA.Inclusion Bodies: A generic term for any circumscribed mass of foreign (e.g., lead or viruses) or metabolically inactive materials (e.g., ceroid or MALLORY BODIES), within the cytoplasm or nucleus of a cell. Inclusion bodies are in cells infected with certain filtrable viruses, observed especially in nerve, epithelial, or endothelial cells. (Stedman, 25th ed)Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.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.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.Neurodegenerative Diseases: Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.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.Fasciculation: Involuntary contraction of the muscle fibers innervated by a motor unit. Fasciculations can often by visualized and take the form of a muscle twitch or dimpling under the skin, but usually do not generate sufficient force to move a limb. They may represent a benign condition or occur as a manifestation of MOTOR NEURON DISEASE or PERIPHERAL NERVOUS SYSTEM DISEASES. (Adams et al., Principles of Neurology, 6th ed, p1294)TDP-43 Proteinopathies: Diseases characterized by the presence of abnormally phosphorylated, ubiquitinated, and cleaved DNA-binding protein TDP-43 in affected brain and spinal cord. Inclusions of the pathologic protein in neurons and glia, without the presence of AMYLOID, is the major feature of these conditions, thus making these proteinopathies distinct from most other neurogenerative disorders in which protein misfolding leads to brain amyloidosis. Both frontotemporal lobar degeneration and AMYOTROPHIC LATERAL SCLEROSIS exhibit this common method of pathogenesis and thus they may represent two extremes of a continuous clinicopathological spectrum of one disease.Anterior Horn Cells: MOTOR NEURONS in the anterior (ventral) horn of the SPINAL CORD which project to SKELETAL MUSCLES.Sclerosis: A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve.Bulbar Palsy, Progressive: A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)Neurofilament Proteins: Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. They consist of three distinct polypeptides, the neurofilament triplet. Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. It appears that the metabolism of neurofilaments is disturbed in Alzheimer's disease, as indicated by the presence of neurofilament epitopes in the neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit of the neurofilament triplet in brains of Alzheimer's patients. (Can J Neurol Sci 1990 Aug;17(3):302)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.Rats, Transgenic: Laboratory rats that have been produced from a genetically manipulated rat EGG or rat EMBRYO, MAMMALIAN. They contain genes from another species.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.Age of Onset: The age, developmental stage, or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual.Tuberous Sclerosis: Autosomal dominant neurocutaneous syndrome classically characterized by MENTAL RETARDATION; EPILEPSY; and skin lesions (e.g., adenoma sebaceum and hypomelanotic macules). There is, however, considerable heterogeneity in the neurologic manifestations. It is also associated with cortical tuber and HAMARTOMAS formation throughout the body, especially the heart, kidneys, and eyes. Mutations in two loci TSC1 and TSC2 that encode hamartin and tuberin, respectively, are associated with the disease.Lithium Carbonate: A lithium salt, classified as a mood-stabilizing agent. Lithium ion alters the metabolism of BIOGENIC MONOAMINES in the CENTRAL NERVOUS SYSTEM, and affects multiple neurotransmission systems.Mutation, Missense: A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)Cycas: A plant genus of the family Cycadaceae, order Cycadales, class Cycadopsida, division CYCADOPHYTA of palm-like trees. It is a source of CYCASIN, the beta-D-glucoside of methylazoxymethanol.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.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.Neuroprotective Agents: Drugs intended to prevent damage to the brain or spinal cord from ischemia, stroke, convulsions, or trauma. Some must be administered before the event, but others may be effective for some time after. They act by a variety of mechanisms, but often directly or indirectly minimize the damage produced by endogenous excitatory amino acids.Excitatory Amino Acid Transporter 2: A glutamate plasma membrane transporter protein found in ASTROCYTES and in the LIVER.DNA-Binding Proteins: Proteins which bind to DNA. The family includes proteins which bind to both double- and single-stranded DNA and also includes specific DNA binding proteins in serum which can be used as markers for malignant diseases.Microglia: The third type of glial cell, along with astrocytes and oligodendrocytes (which together form the macroglia). Microglia vary in appearance depending on developmental stage, functional state, and anatomical location; subtype terms include ramified, perivascular, ameboid, resting, and activated. Microglia clearly are capable of phagocytosis and play an important role in a wide spectrum of neuropathologies. They have also been suggested to act in several other roles including in secretion (e.g., of cytokines and neural growth factors), in immunological processing (e.g., antigen presentation), and in central nervous system development and remodeling.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.Pseudobulbar Palsy: A syndrome characterized by DYSARTHRIA, dysphagia, dysphonia, impairment of voluntary movements of tongue and facial muscles, and emotional lability. This condition is caused by diseases that affect the motor fibers that travel from the cerebral cortex to the lower BRAIN STEM (i.e., corticobulbar tracts); including MULTIPLE SCLEROSIS; MOTOR NEURON DISEASE; and CEREBROVASCULAR DISORDERS. (From Adams et al., Principles of Neurology, 6th ed, p489)Mutant Proteins: Proteins produced from GENES that have acquired MUTATIONS.Sialorrhea: Increased salivary flow.Rotarod Performance Test: A performance test based on forced MOTOR ACTIVITY on a rotating rod, usually by a rodent. Parameters include the riding time (seconds) or endurance. Test is used to evaluate balance and coordination of the subjects, particular in experimental animal models for neurological disorders and drug effects.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Noninvasive Ventilation: Techniques for administering artificial respiration without the need for INTRATRACHEAL INTUBATION.Amino Acid Substitution: The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.Ribonuclease, Pancreatic: An enzyme that catalyzes the endonucleolytic cleavage of pancreatic ribonucleic acids to 3'-phosphomono- and oligonucleotides ending in cytidylic or uridylic acids with 2',3'-cyclic phosphate intermediates. EC 3.1.27.5.Axonal Transport: The directed transport of ORGANELLES and molecules along nerve cell AXONS. Transport can be anterograde (from the cell body) or retrograde (toward the cell body). (Alberts et al., Molecular Biology of the Cell, 3d ed, pG3)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.Case-Control Studies: Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.Animals, Genetically Modified: ANIMALS whose GENOME has been altered by GENETIC ENGINEERING, or their offspring.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)Mitochondria: Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)Amino Acids, DiaminoCell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability.Peripherins: Type III intermediate filament proteins expressed mainly in neurons of the peripheral and CENTRAL NERVOUS SYSTEMS. Peripherins are implicated in neurite elongation during development and axonal regeneration after injury.Protein Folding: Processes involved in the formation of TERTIARY PROTEIN STRUCTURE.Scleroderma, Systemic: A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.Phenotype: The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Laughter: An involuntary expression of merriment and pleasure; it includes the patterned motor responses as well as the inarticulate vocalization.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.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.Neuromuscular Junction: The synapse between a neuron and a muscle.Nerve Tissue ProteinsNeuroglia: 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.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.Parkinson Disease: A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)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.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)Communication Aids for Disabled: Equipment that provides mentally or physically disabled persons with a means of communication. The aids include display boards, typewriters, cathode ray tubes, computers, and speech synthesizers. The output of such aids includes written words, artificial speech, language signs, Morse code, and pictures.Copper: A heavy metal trace element with the atomic symbol Cu, atomic number 29, and atomic weight 63.55.Mice, Inbred C57BLTracheostomy: Surgical formation of an opening into the trachea through the neck, or the opening so created.ItalyGlial Fibrillary Acidic Protein: An intermediate filament protein found only in glial cells or cells of glial origin. MW 51,000.Gliosis: The production of a dense fibrous network of neuroglia; includes astrocytosis, which is a proliferation of astrocytes in the area of a degenerative lesion.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.Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.Chromosomes, Human, Pair 9: A specific pair of GROUP C CHROMSOMES of the human chromosome classification.Ubiquitin: A highly conserved 76-amino acid peptide universally found in eukaryotic cells that functions as a marker for intracellular PROTEIN TRANSPORT and degradation. Ubiquitin becomes activated through a series of complicated steps and forms an isopeptide bond to lysine residues of specific proteins within the cell. These "ubiquitinated" proteins can be recognized and degraded by proteosomes or be transported to specific compartments within the cell.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.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.Motor Cortex: Area of the FRONTAL LOBE concerned with primary motor control located in the dorsal PRECENTRAL GYRUS immediately anterior to the central sulcus. It is comprised of three areas: the primary motor cortex located on the anterior paracentral lobule on the medial surface of the brain; the premotor cortex located anterior to the primary motor cortex; and the supplementary motor area located on the midline surface of the hemisphere anterior to the primary motor cortex.Genetic Predisposition to Disease: A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.Pyrazolones: Compounds with a five-membered heterocyclic ring with two nitrogens and a keto OXYGEN. Some are inhibitors of TNF-ALPHA production.Respiratory Insufficiency: Failure to adequately provide oxygen to cells of the body and to remove excess carbon dioxide from them. (Stedman, 25th ed)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)Oxidative Stress: A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).Nervous System Diseases: Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle.Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter.Multiple Sclerosis, Chronic Progressive: A form of multiple sclerosis characterized by a progressive deterioration in neurologic function which is in contrast to the more typical relapsing remitting form. If the clinical course is free of distinct remissions, it is referred to as primary progressive multiple sclerosis. When the progressive decline is punctuated by acute exacerbations, it is referred to as progressive relapsing multiple sclerosis. The term secondary progressive multiple sclerosis is used when relapsing remitting multiple sclerosis evolves into the chronic progressive form. (From Ann Neurol 1994;36 Suppl:S73-S79; Adams et al., Principles of Neurology, 6th ed, pp903-914)Cell Survival: The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability.Proteins: Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Neuroimaging: Non-invasive methods of visualizing the CENTRAL NERVOUS SYSTEM, especially the brain, by various imaging modalities.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.

A clinical study of motor evoked potentials using a triple stimulation technique. (1/1872)

Amplitudes of motor evoked potentials (MEPs) are usually much smaller than those of motor responses to maximal peripheral nerve stimulation, and show marked variation between normal subjects and from one stimulus to another. Consequently, amplitude measurements have low sensitivity to detect central motor conduction failures due to the broad range of normal values. Since these characteristics are mostly due to varying desynchronization of the descending action potentials, causing different degrees of phase cancellation, we applied the recently developed triple stimulation technique (TST) to study corticospinal conduction to 489 abductor digiti minimi muscles of 271 unselected patients referred for possible corticospinal dysfunction. The TST allows resynchronization of the MEP, and thereby a quantification of the proportion of motor units activated by the transcranial stimulus. TST results were compared with those of conventional MEPs. In 212 of 489 sides, abnormal TST responses suggested conduction failure of various degrees. By contrast, conventional MEPs detected conduction failures in only 77 of 489 sides. The TST was therefore 2.75 times more sensitive than conventional MEPs in disclosing corticospinal conduction failures. When the results of the TST and conventional MEPs were combined, 225 sides were abnormal: 145 sides showed central conduction failure, 13 sides central conduction slowing and 67 sides both conduction failure and slowing. It is concluded that the TST is a valuable addition to the study of MEPs, since it improves detection and gives quantitative information on central conduction failure, an abnormality which appears to be much more frequent than conduction slowing. This new technique will be useful in following the natural course and the benefit of treatments in disorders affecting central motor conduction.  (+info)

Nitric oxide, mitochondria and neurological disease. (2/1872)

Damage to the mitochondrial electron transport chain has been suggested to be an important factor in the pathogenesis of a range of neurological disorders, such as Parkinson's disease, Alzheimer's disease, multiple sclerosis, stroke and amyotrophic lateral sclerosis. There is also a growing body of evidence to implicate excessive or inappropriate generation of nitric oxide (NO) in these disorders. It is now well documented that NO and its toxic metabolite, peroxynitrite (ONOO-), can inhibit components of the mitochondrial respiratory chain leading, if damage is severe enough, to a cellular energy deficiency state. Within the brain, the susceptibility of different brain cell types to NO and ONOO- exposure may be dependent on factors such as the intracellular reduced glutathione (GSH) concentration and an ability to increase glycolytic flux in the face of mitochondrial damage. Thus neurones, in contrast to astrocytes, appear particularly vulnerable to the action of these molecules. Following cytokine exposure, astrocytes can increase NO generation, due to de novo synthesis of the inducible form of nitric oxide synthase (NOS). Whilst the NO/ONOO- so formed may not affect astrocyte survival, these molecules may diffuse out to cause mitochondrial damage, and possibly cell death, to other cells, such as neurones, in close proximity. Evidence is now available to support this scenario for neurological disorders, such as multiple sclerosis. In other conditions, such as ischaemia, increased availability of glutamate may lead to an activation of a calcium-dependent nitric oxide synthase associated with neurones. Such increased/inappropriate NO formation may contribute to energy depletion and neuronal cell death. The evidence available for NO/ONOO--mediated mitochondrial damage in various neurological disorders is considered and potential therapeutic strategies are proposed.  (+info)

The role of immunophilins in mutant superoxide dismutase-1linked familial amyotrophic lateral sclerosis. (3/1872)

It has been reported that expression of familial amyotrophic lateral sclerosis (FALS)-associated mutant Cu/Zn superoxide dismutase-1 (SOD) induces apoptosis of neuronal cells in culture associated with an increase in reactive oxygen species. SOD recently has been shown to prevent calcineurin inactivation, initiating the present investigations examining the role of calcineurin in mutant SOD-induced cell death. Wild-type or mutant SOD was expressed in neuronal cells by infection with replication-deficient adenoviruses. PC12 cells overexpressing human wild-type SOD exhibited higher calcineurin activity than cells expressing FALS-related mutant SOD (SODV148G); however, cells expressing SODV148G had calcineurin activity equal to mock-infected cells, suggesting that cell death induced by mutant SOD was not related to a decrease in calcineurin activity. Calcineurin antagonists such as cyclosporin A and FK506, as well as nonimmunosuppressant analogs of cyclosporin A, significantly enhanced SODV148G- and SODA4V-induced cell death. Because both groups of drugs inhibit the rotamase activity of cyclophilins (CyP), but only the immunosuppressant analogs inhibit calcineurin activity, these data suggest that rotamase inhibition underlies the enhanced cell death after SODV148G expression. The importance of rotamase activity in mutant SOD-mediated apoptosis was supported by experiments showing that overexpressed wild-type cyclophilin A (CyPA), but not CyPA with a rotamase active site point mutation, protected cells from death after SODV148G expression. These data suggest that mutant SOD produces a greater need for rotamase and, also, highlights possible new therapeutic strategies in FALS.  (+info)

Release of copper ions from the familial amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutants. (4/1872)

Point mutations of Cu,Zn-superoxide dismutase (SOD) have been linked to familial amyotrophic lateral sclerosis (FALS). We reported that the Swedish FALS Cu,Zn-SOD mutant, D90A, exhibited an enhanced hydroxyl radical-generating activity, while its dismutation activity was identical to that of the wild-type enzyme (Kim et al. 1998a; 1998b). Transgenic mice that express a mutant Cu,Zn-SOD, Gly93 --> Ala (G93A), have been shown to develop amyotrophic lateral sclerosis (ALS) symptoms. We cloned the cDNA for the FALS G93A mutant, overexpressed the protein in E. coli cells, purified the protein, and studied its enzymic activities. Our results showed that the G93A, the D90A, and the wild-type enzymes have identical dismutation activity. However, the hydroxyl radical-generating activity of the G93A mutant was enhanced relative to those of the D90A and the wild-type enzyme (wild-type < D90A < G93A). These higher free radical-generating activities of mutants facilitated the release of copper ions from their own molecules (wild-type < D90A < G93A). The released copper ions can enhance the Fenton-like reaction to produce hydroxyl radicals and play a major role in the oxidative damage of macromolecules. Thus, the FALS symptoms may be associated with the enhancements in both the free radical-generating activity and the releasing of copper ions from the mutant enzyme.  (+info)

Amyotrophic lateral sclerosis: Lou Gehrig's disease. (5/1872)

Amyotrophic lateral sclerosis (ALS), commonly called Lou Gehrig's disease, is a progressive neuromuscular condition characterized by weakness, muscle wasting, fasciculations and increased reflexes. Approximately 30,000 Americans currently have the disease. The annual incidence rate is one to two cases per 100,000. The disease is most commonly diagnosed in middle age and affects more men than women. It usually presents with problems in dexterity or gait resulting from muscle weakness. Difficulty in speaking or swallowing is the initial symptom in the bulbar form of the disease. Over a period of months or years, patients with ALS develop severe, progressive muscular weakness and other symptoms caused by loss of function in both upper and lower motor neurons. Sphincter control, sensory function, intellectual abilities and skin integrity are preserved. Patients become completely disabled, often requiring ventilatory support and gastrostomy. Death usually occurs within five years of diagnosis and is attributed to respiratory failure or cachexia. The etiology of the disease is unknown. Current research is focused on abnormalities of neuronal cell metabolism involving glutamate and the role of potential neurotoxins and neurotrophic factors. New drugs are being developed based on these theories. Current management involves aggressive, individualized alleviation of symptoms and complications.  (+info)

Atypical form of amyotrophic lateral sclerosis. (6/1872)

OBJECTIVE: To investigate patients with an unusual type of muscular atrophy confined to the upper limbs (proximally dominant) and the shoulder girdle, while sparing the face and the legs until the terminal stage. METHODS: Eight patients (six men and two women) were clinically examined. The age at onset ranged from 42 to 73 years, and the clinical course varied from 28 to 81 months. There was no family history of motor neuron disease in any of these patients. Necropsy was performed in two of them. RESULTS: All eight patients basically showed a similar distribution of muscular weakness and atrophy. Subluxation of the shoulder joints was found in all patients. Reflexes were absent in the upper limbs in all patients, but were almost normal in the face and legs in most patients. Pathological reflexes could be elicited in only one patient. Electromyography showed typical neurogenic changes in the limbs of all patients. Cervical MRI disclosed moderate spondylotic changes in seven patients. Antiganglioside antibodies were negative in six patients tested. Abnormal trinucleotide (CAG) repeat expansion of androgen receptor gene was not recognised in five patients examined. Bulbar involvement developed in three patients during the course of the disease. At necropsy, one patient showed degeneration of the pyramidal tracts and motor cortex including Betz cells as well as loss of spinal anterior horn cells and brainstem motor neurons, which is consistent with ALS; in another patient there was neuronal loss of anterior horn cells at the spinal cord accompanied by astrogliosis, whereas the motor cortex and brainstem motor nuclei were relatively well preserved. Intracytoplasmic inclusions such as Bunina bodies, skein-like inclusions, and Lewy body-like inclusions were found in both patients. CONCLUSION: These patients with their peculiar pattern of muscular atrophy seem to have ALS or a subtype of ALS.  (+info)

Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds. (7/1872)

Mutations in superoxide dismutase 1 (SOD1) polypeptides cause a form of familial amyotrophic lateral sclerosis (FALS). In different kindreds, harboring different mutations, the duration of illness tends to be similar for a given mutation. For example, patients inheriting a substitution of valine for alanine at position four (A4V) average a 1.5 year life expectancy after the onset of symptoms, whereas patients harboring a substitution of arginine for histidine at position 46 (H46R) average an 18 year life expectancy after disease onset. Here, we examine a number of biochemical and biophysical properties of nine different FALS variants of SOD1 polypeptides, including enzymatic activity (which relates indirectly to the affinity of the enzyme for copper), polypeptide half-life, resistance to proteolytic degradation and solubility, in an effort to determine whether a specific property of these enzymes correlates with clinical progression. We find that although all the mutants tested appear to be soluble, the different mutants show a remarkable degree of variation with respect to activity, polypeptide half-life and resistance to proteolysis. However, these variables do not stratify in a manner that correlates with clinical progression. We conclude that the basis for the different life expectancies of patients in different kindreds of sod1-linked FALS may result from an as yet unidentified property of these mutant enzymes.  (+info)

Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls and retropulsion. (8/1872)

Three patients with sporadic amyotrophic lateral sclerosis (ALS) presented with a history of backward falls. Impaired postural reflexes and retropulsion accompanied clinical features of ALS. Hypokinesia, decreased arm swing, and a positive glabellar tap were noted in two of these three patients. Cognitive impairment, tremor, axial rigidity, sphincter dysfunction, nuchal dystonia, dysautonomia, and oculomotor dysfunction were absent. Brain MRI disclosed bilateral T2 weighted hyperintensities in the internal capsule and globus pallidus in one patient. Necropsy studies performed late in the course of ALS have shown degeneration in extrapyramidal sites-for example, the globus pallidus, thalamus, and substantia nigra. Clinically, backward falls and retropulsion may occur early in ALS. This may reflect extrapyramidal involvement.  (+info)

*Amyotrophic lateral sclerosis

... including amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, progressive muscular atrophy, progressive bulbar ... "Amyotrophic lateral sclerosis". Lancet. 377 (9769): 942-55. doi:10.1016/s0140-6736(10)61156-7. PMID 21296405. "Amyotrophic ... Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) and Lou Gehrig's disease, is a specific disease ... Eisen, A. (2002). "Amyotrophic lateral sclerosis: A review". BCMJ. 44 (7): 362-366. Archived from the original on 21 June 2013 ...

*Amyotrophic lateral sclerosis research

Research on amyotrophic lateral sclerosis has focused on animal models of the disease, its mechanisms, ways to diagnose and ... "Rodent models of amyotrophic lateral sclerosis". Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1832 (9): ... Kim, Changsung; Lee, Hee Chul; Sung, Jung-Joon (2014-09-01). "Amyotrophic Lateral Sclerosis - Cell Based Therapy and Novel ... "Public summary of opinion on orphan designation Masitinib mesilate for treatment of amyotrophic lateral sclerosis" (PDF). EMA. ...

*Genetics of amyotrophic lateral sclerosis

... models and biomarkers in amyotrophic lateral sclerosis". Amyotrophic lateral sclerosis & frontotemporal degeneration. 14 (1): ... Al-Saif A, Al-Mohanna F, Bohlega S (2011). "A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis". ... Zou, ZY; Liu, CY; Che, CH; Huang, HP (January 2016). "Toward precision medicine in amyotrophic lateral sclerosis". Annals of ... About 5-10% of cases of amyotrophic lateral sclerosis are directly inherited from a person's parents. Overall, first-degree ...

*Neuroscience of aging

Amyotrophic Lateral Sclerosis Creutzfeldt-Jakob disease Frontotemporal Dementia Dementia with Lewy bodies Corticobasal ... Aging is also associated with many neurological and neurodegenerative disease such as amyotrophic lateral sclerosis, dementia, ... "Amyotrophic lateral sclerosis". Lancet. 377 (9769): 942-55. doi:10.1016/s0140-6736(10)61156-7. PMID 21296405. Belay, Ermias D ...

*Granulocyte colony-stimulating factor

"Preliminary investigation of effect of granulocyte colony stimulating factor on amyotrophic lateral sclerosis". Amyotrophic ... "Granulocyte-colony stimulating factor improves outcome in a mouse model of amyotrophic lateral sclerosis". Brain. 131 (Pt 12): ... phase IIb and several clinical pilot studies are published for other neurological disease such as amyotrophic lateral sclerosis ... Lateral Sclerosis. 10 (5-6): 430-1. doi:10.3109/17482960802588059. PMID 19922135. Acosta SA, Tajiri N, Shinozuka K, Ishikawa H ...

*Neuromyotonia

Amyotrophic lateral sclerosis. N Engl J Med 2001; 344: 1688-700. Hirota N, Eisen A, Weber M. Complex fasciculations and their ... In some rare cases, acquired neuromyotonia has been misdiagnosed as amyotrophic lateral sclerosis (ALS) particularly if ... Similarly, multiple sclerosis has been the initial misdiagnosis in some NMT patients. In order to get an accurate diagnosis see ... origin in amyotrophic lateral sclerosis and Kennedy's disease. Muscle Nerve 2000; 23: 1872-5. http://brain.oxfordjournals.org/ ...

*ZGRF1

"Senataxin mutations and amyotrophic lateral sclerosis". Amyotrophic Lateral Sclerosis. 12 (3): 223-7. doi:10.3109/17482968.2010 ...

*Cyanobacteria

"A cluster of amyotrophic lateral sclerosis in New Hampshire: a possible role for toxic cyanobacteria blooms". Amyotrophic ... Amyotrophic Lateral Sclerosis. 10 Suppl 2: 109-17. doi:10.3109/17482960903286066. PMID 19929742. Main D (2006). "Toxic Algae ... that significant exposure to high levels of cyanobacteria producing toxins such as BMAA can cause amyotrophic lateral sclerosis ... Lateral Sclerosis. 10 Suppl 2: 101-8. doi:10.3109/17482960903278485. PMID 19929741. Cox PA, Richer R, Metcalf JS, Banack SA, ...

*SMN2

Amyotrophic Lateral Sclerosis. 10 (5-6): 436-40. doi:10.3109/17482960902759162. PMID 19922137. Tiziano FD, Pinto AM, Fiori S, ...

*Transferase

Rowland LP, Shneider NA (May 2001). "Amyotrophic lateral sclerosis". The New England Journal of Medicine. 344 (22): 1688-700. ... "Early presymptomatic cholinergic dysfunction in a murine model of amyotrophic lateral sclerosis". Brain and Behavior. 3 (2): ...

*Proteopathy

Ludolph, Albert C.; Brettschneider, Johannes; Weishaupt, Jochen H. (October 2012). "Amyotrophic lateral sclerosis". Current ... and in amyotrophic lateral sclerosis / frontotemporal lobar degeneration (FTLD), certain gene-regulating proteins ... Suggesting a Propagative Cell Death Mechanism in Amyotrophic Lateral Sclerosis". PLoS ONE. 5: e10627. doi:10.1371/journal.pone. ... Prion-like mechanisms in amyotrophic lateral sclerosis". Neurobiology of Disease. 77: 257-265. doi:10.1016/j.nbd.2015.02.009. ...

*PRR32

"Differential gene expression in patients with amyotrophic lateral sclerosis". Amyotrophic Lateral Sclerosis. 12 (4): 250-6. doi ... PRR32 (CXorf64) seems to be involved with a group of genes over-expressed in ALS (Amyotrophic lateral sclerosis), evident from ... An experiment analyzed gene expression pattern in muscles from patients with amyotrophic lateral sclerosis (ALS) and multifocal ... a study aiming to study gene expression patterns in muscles from patients with amyotrophic lateral sclerosis and multifocal ...

*Fasciculation

Poliomyelitis Amyotrophic lateral sclerosis (ALS) Spinal muscular atrophies - including spinal muscular atrophy (SMA), spinal ... Amyotrophic Lateral Sclerosis. 9 (2): 120-1. doi:10.1080/17482960701855864. PMID 18428004. Rivlin RS (1994). "Magnesium ... needle electromyography and clinical observation in the detection of fasciculation in people with amyotrophic lateral sclerosis ...

*Pseudobulbar affect

... multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), Lyme disease, PANDAS in children and adults, and Parkinson's ... A study designed specifically to survey for prevalence found that 49% of patients with amyotrophic lateral sclerosis (ALS) also ... Caroscio JT, Mulvihill MN, Sterling R, Abrams B; Mulvihill; Sterling; Abrams (1987). "Amyotrophic lateral sclerosis". ... "Pathological laughing and crying in amyotrophic lateral sclerosis: an association with prefrontal cognitive dysfunction". ...

*Talampanel

"A phase II trial of talampanel in subjects with amyotrophic lateral sclerosis". Amyotrophic Lateral Sclerosis. 11 (3): 266-271 ... malignant gliomas and amyotrophic lateral sclerosis (ALS). As of May 2010, results from the trial for ALS have been found ...

*Polyunsaturated fat

October 2007). "Nutritional status and risk of amyotrophic lateral sclerosis in Japan". Amyotrophic Lateral Sclerosis. 8 (5): ... "Intake of polyunsaturated fatty acids and vitamin E reduces the risk of developing amyotrophic lateral sclerosis". Journal of ... of polyunsaturated fatty acids is under preliminary research to assess the risk of developing amyotrophic lateral sclerosis ( ...

*Cycasin

Amyotrophic Lateral Sclerosis. 10 (sup2): 7-20. doi:10.3109/17482960903286009. Cox PA, Banack SA, Murch SJ (2003). " ... occurs exclusively in the Chamorro people of the island of Guam that has characteristics of both amyotrophic lateral sclerosis ... The Lytico-bodig disease, also known as lateral sclerosis-parkinsonism-dementia, is a neurodegenerative disease of unknown ... It is clinically characterized by weight loss followed by lateral swaying of the hind quarters, with weakness, ataxia, and ...

*Excitotoxicity

Amyotrophic lateral sclerosis. 20: 85-95. Vyas, KJ; Weiss, JH (2009). "BMAA--an unusual cyanobacterial neurotoxin". Amyotrophic ... such as multiple sclerosis, Alzheimer's disease, amyotrophic lateral sclerosis (ALS), Parkinson's disease, alcoholism or ... has long been identified as a neurotoxin which was first associated with the amyotrophic lateral sclerosis/parkinsonism- ... lateral sclerosis. 10: 50-55. doi:10.3109/17482960903268742. PMID 19929732. Chiu, AS; et al. (2012). "Excitotoxic potential of ...

*Lytico-bodig disease

The disease resembles Amyotrophic Lateral Sclerosis (ALS), Parkinson's disease, and Alzheimer's. First reports of the disease ... It is referred to by neuroscientists as amyotrophic lateral sclerosis-parkinsonism-dementia (ALS-PDC), a term coined by Asao ... 1990). "2-Amino-3-(methylamino)-propanoic acid (BMAA) in cycad flour: an unlikely cause of amyotrophic lateral sclerosis and ... Brody JA, Chen K (1969). "Changing epidemiologic patterns of Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia on Guam". ...

*Hereditary inclusion body myopathy

... and amyotrophic lateral sclerosis ("IBMPFD/ALS"). 2013 review sought to explain the degenerative process in diseases like MSP, ... amyotrophic lateral sclerosis, and frontotemporal lobar degeneration, via the development of pathological granules containing ... Pagetoid amyotrophic lateral sclerosis; Pagetoid neuroskeletal syndrome "ORPHA52430: Inclusion body myopathy with Paget disease ...

*Aloysius Schwartz

Amyotrophic Lateral Sclerosis (ALS). He accepted it with joy, serenity, and courage, and regarded it as a gift from God. In ...

*Pyrimethamine

... has also been found to reduce the expression of SOD1, a key protein involved in amyotrophic lateral sclerosis. ... It is being evaluated in clinical trials as a treatment for amyotrophic lateral sclerosis. "Pyrimethamine". The American ... "Recent progress in the discovery of small molecules for the treatment of amyotrophic lateral sclerosis (ALS)" (PDF). Beilstein ... Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 14 (3): 199-204. doi:10.3109/17482968.2012.724074. PMID 22985433. ...

*List of OMIM disorder codes

FUS Amyotrophic lateral sclerosis 8; 608627; VAPB Amyotrophic lateral sclerosis 9; 611895; ANG Amyotrophic lateral sclerosis, ... TARDBP Amyotrophic lateral sclerosis 11; 612577; FIG4 Amyotrophic lateral sclerosis 4, juvenile; 602433; SETX Amyotrophic ... SOD1 Amyotrophic lateral sclerosis, juvenile; 205100; ALS2 Amyotrophy, hereditary neuralgic; 162100; 40430 Amytrophic lateral ... LYZ Amyotrophic lateral sclerosis 10, with or without FTD; 612069; ...

*Troponin C type 1

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 14 (7-8): 582-5. doi:10.3109/21678421.2013.817587. PMID 23952600. ...

*ALS Functional Rating Scale - Revised

Amyotrophic Lateral Sclerosis (ALS), is a neurodegenerative disease that typically affects adults around 54-67 years of age, ... Hobson, Esther V.; McDermott, Christopher J. "Supportive and symptomatic management of amyotrophic lateral sclerosis". Nature ... Martin, Sarah; Al Khleifat, Ahmad; Al-Chalabi, Ammar (2017-03-28). "What causes amyotrophic lateral sclerosis?". F1000Research ... "Predicting prognosis in amyotrophic lateral sclerosis: a simple algorithm". Journal of Neurology. 262 (6): 1447-1454. doi: ...

*Split hand syndrome

It has been proposed as a relatively specific sign for amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease). It can also ... April 2008). "Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity". ... "Dissociated small hand muscle involvement in amyotrophic lateral sclerosis detected by motor unit number estimates". Muscle ... In medicine, split hand syndrome is a neurological syndrome in which the hand muscles on the side of the thumb (lateral, thenar ...
Microglial cell-associated neuroinflammation is considered as a potential contributor to the pathophysiology of sporadic amyotrophic lateral sclerosis. However, the specific role of microglia in the disease pathogenesis remains to be elucidated. We studied the activation profiles of the microglial cultures exposed to the cerebrospinal fluid from these patients which recapitulates the neurodegeneration seen in sporadic amyotrophic lateral sclerosis. This was done by investigating the morphological and functional changes including the expression levels of prostaglandin E2 (PGE2), cyclooxygenase-2 (COX-2), TNF-α, IL-6, IFN-γ, IL-10, inducible nitric oxide synthase (iNOS), arginase, and trophic factors. We also studied the effect of chitotriosidase, the inflammatory protein found upregulated in the cerebrospinal fluid from amyotrophic lateral sclerosis patients, on these cultures. We report that the cerebrospinal fluid from amyotrophic lateral sclerosis patients could induce an early and potent response
TY - JOUR. T1 - Activation of signal transducer and activator of transcription-3 in the spinal cord of sporadic amyotrophic lateral sclerosis patients. AU - Shibata, Noriyuki. AU - Kakita, Akiyoshi. AU - Takahashi, Hitoshi. AU - Ihara, Yuetsu. AU - Nobukuni, Keigo. AU - Fujimura, Harutoshi. AU - Sakoda, Saburo. AU - Sasaki, Shoichi. AU - Iwata, Makoto. AU - Morikawa, Shunichi. AU - Hirano, Asao. AU - Kobayashi, Makio. PY - 2009/5/1. Y1 - 2009/5/1. N2 - Background: Neuroinflammation has been implicated in the pathomechanism of amyotrophic lateral sclerosis (ALS). It is known that signal transducer and activator of transcription-3 (STAT3) is a proinflammatory transcription factor. However, it remains to be determined whether STAT3 is involved in ALS. Objective: To test the hypothesis that STAT3 may be upregulated, activated, or both in the spinal cord of ALS patients. Methods: We performed immunohistochemical, immunoblot and densitometric analyses of total STAT3 (t-STAT3) or phosphorylated active ...
... (ALS), also referred to as "Lou Gehrigs disease" is a disease of the motor nerve cells in the brain and spinal cord. ALS is caused by progressive loss of motor nerves in these areas and affects approximately 1 out of 100,000 people. The diagnosis of ALS is usually based on clinical features, electrodiagnostic testing (EMG), and exclusion of other health conditions with related symptoms. Most people with amyotrophic lateral sclerosis have a form of the condition that is described as sporadic or noninherited. The cause of sporadic amyotrophic lateral sclerosis is largely unknown but probably involves a combination of genetic and environmental factors. About 10 percent of people with amyotrophic lateral sclerosis have a familial form of the condition, which is caused by an inherited genetic mutation ...
Amyotrophic lateral sclerosis, or known as ALS, motor neuron disease, or upper and lower motor neuron disease, is a severe neurological disorder in brain and spinal cord that is characterized by disorders of voluntary muscle movement such as muscle weakness, permanent disability, until death. It is also called Lou Gehrigs disease because there was a basketball player named Lou Gehrig who died of this disease. Amyotrophic lateral sclerosis is a very uncommon disease who occurs at 5 of 100,000 people in the world. The risk exact causes of amyotrophic lateral sclerosis is still unknown, but generally it is a hereditary disease. Researchers also found that amyotrophic lateral sclerosis is caused by gene mutation, bad immune response, and chemical substance imbalance such as too much glutamate ...
Amyotrophic Lateral Sclerosis (ALS), Read about Amyotrophic Lateral Sclerosis (ALS) symptoms, causes, diagnosis, and treatment. Also read Amyotrophic Lateral Sclerosis (ALS) articles about how to live with Amyotrophic Lateral Sclerosis (ALS), and more.
BACKGROUND: Although an increasing role of genetic susceptibility has been recognized, the role of environmental risk factors in amyotrophic lateral sclerosis (ALS) etiology is largely uncertain; among neurotoxic chemicals, epidemiological and biological plausibility has been provided for pesticides, the heavy metal lead, the metalloid selenium, and other persistent organic pollutants. Selenium involvement in ALS has been suggested on the basis of epidemiological studies, in vitro investigations, and veterinary studies in which selenium induced a selective toxicity against motor neurons. OBJECTIVE: Hypothesizing a multistep pathogenic mechanism (genetic susceptibility and environmental exposure), we aimed to study selenium species in ALS patients carrying disease-associated gene mutations as compared to a series of hospital controls. METHODS: Using advanced analytical techniques, we determined selenium species in cerebrospinal fluid sampled at diagnosis in 9 ALS patients carrying different gene ...
... On-line free medical diagnosis assistant. Ranked list of possible diseases from either several symptoms or a full patient history. A similarity measure between symptoms and diseases is provided.
ObjectiveTo describe a mother who had autopsy-proved amyotrophic lateral sclerosis and her daughter who had clinically diagnosed Creutzfeldt-Jakob disease.Desig
Title: Molecular and Cellular Mechanism of Glutamate Receptors in Relation to Amyotrophic Lateral Sclerosis. VOLUME: 1 ISSUE: 5. Author(s): Yasuo Iwasaki, Ken Ikeda and Masao Kinoshita. Affiliation:Fourth Department of Internal Medicine, Toho University Ohashi Hospital, 2-17-6, Ohashi, Meguro-ku, Tokyo 153-8515, Japan.. Keywords:excitatory amino acids, glutamate, glutamate receptors, excitotoxicity, amyotrophic lateral sclerosis. Abstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder of the central nervous system (CNS) with an unknown etiology. This disorder is characterized clinically by muscular weakness and wasting, and pathologically by selective degeneration of the corticospinal tracts and motor neurons of the brain stem and spinal cord. Median survival following onset is 3 to 5 years. Riluzole, an antiglutamatergic agent has been shown to have modest beneficial effects on survival. Glutamate is the main excitatory neurotransmitter in the CNS and excessive ...
TY - JOUR. T1 - Environmental and Occupational Exposures and Amyotrophic Lateral Sclerosis in New England. AU - Andrew, Angeline S.. AU - Caller, Tracie A.. AU - Tandan, Rup. AU - Duell, Eric J.. AU - Henegan, Patricia L.. AU - Field, Nicholas C.. AU - Bradley, Walter G. AU - Stommel, Elijah W.. PY - 2017/2/1. Y1 - 2017/2/1. N2 - Background: Recent data provide support for the concept that potentially modifiable exposures are responsible for sporadic amyotrophic lateral sclerosis (ALS). Objective: To evaluate environmental and occupational exposures as risk factors for sporadic ALS. Methods: We performed a case-control study of ALS among residents of New England, USA. The analysis compared questionnaire responses from 295 patients with a confirmed ALS diagnosis to those of 225 controls without neurodegenerative illness. Results: Self-reported job-or hobby-related exposure to one or more chemicals, such as pesticides, solvents, or heavy metals, increased the risk of ALS (adjusted OR 2.51; 95% CI ...
Treatment for Amyotrophic Lateral Sclerosis. Find Doctors Near You, Book Appointment, Consult Online, View Doctor Fees, Address, Phone Numbers and Reviews. Doctors for Amyotrophic Lateral Sclerosis | Lybrate
Another name for Amyotrophic Lateral Sclerosis is Amyotrophic Lateral Sclerosis. The evaluation of amyotrophic lateral sclerosis begins with a history ...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative syndrome of unknown etiology that most commonly affects people in middle and high age. The hallmark of ALS is a progressive and simultaneous loss of upper and lower motor neurons in the central nervous system that leads to a progressive muscle atrophy, paralysis and death usually by respiratory failure. ALS is not a pure motor neuronal syndrome; it extends beyond the motor system and affects extramotor areas of the brain as well. The majority of the patients suffer from a sporadic ALS disease (SALS) while in at least ten percent the disease appears in a familial form (FALS). Mutations in the gene encoding the antioxidant enzyme superoxide dismutase-1 (SOD1) are the most common cause of FALS. More than 165 SOD1 mutations have been described, and these confer the enzyme a cytotoxic gain of function. Evidence suggests that the toxicity results from structural instability which makes the mutated enzyme prone to misfold and form ...
ALS is characterized by oxidative damage in the brain and cerebrospinal fluid, which is exerted by pro-oxidative activity of iron. Such activity of iron can be drastically increased in the presence of inappropriate iron ligands that catalyze redox cycling of iron, thereby promoting hydroxyl radical generation. The aim of our study was to determine the relative level of inappropriate iron ligands in the cerebrospinal fluid of ALS patients. To determine the levels of inappropriate iron ligands and redox activity of iron in cerebrospinal fluid (10 samples from ALS patients and 10 controls), we applied electron paramagnetic resonance spectroscopy. We have shown that cerebrospinal fluid of ALS patients comprises twofold increased level of inappropriate iron ligands, proportionally increasing iron redox activity and hydroxyl radical production compared to controls. In conclusion, our results strongly support the pro-oxidative/detrimental role of inappropriately chelated iron in ALS pathophysiology. ...
TY - JOUR. T1 - Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. AU - Takeuchi, Hideyuki. AU - Niwa, Jun Ichi. AU - Hishikawa, Nozomi. AU - Ishigaki, Shinsuke. AU - Tanaka, Fumiaki. AU - Doyu, Manabu. AU - Sobue, Gen. PY - 2004/4. Y1 - 2004/4. N2 - Dorfin is a RING-finger type ubiquitin ligase for mutant superoxide dismutase 1 (SOD1) that enhances its degradation. Mutant SOD1s cause familial amyotrophic lateral sclerosis (FALS) through the gain of unelucidated toxic properties. We previously showed that the accumulation of mutant SOD1 in the mitochondria triggered the release of cytochrome c, followed by the activation of the caspase cascade and induction of neuronal cell death. In the present study, therefore, we investigated whether Dorfin can modulate the level of mutant SOD1 in the mitochondria and subsequent caspase activation. We showed that Dorfin significantly reduced the ...
Objective:. The goal of this study is to see whether patterns of cerebral cortex dysfunction differ in Primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS). The function of several regions of the cerebral cortex will be assessed by clinical, physiological, and neuropsychological tests. Magnetic resonance imaging will be carried out in a subset of patients to explore the correlation between functional and anatomical measures of these cortical areas.. Study Population:. 30 patients with Primary lateral sclerosis who meet the diagnostic criteria for PLS proposed by Pringle and 30 patients with ALS who fulfill the revised El Escorial criteria for probable or definite ALS.. 30 healthy volunteers will be studied to provide training and practice in using the rating scales and to provide age-matched controls for EEG and imaging studies.. Design:. A screening examination will be carried out under protocol 01-N-0145 to determine eligibility. Patients and caregivers will return for two ...
Recently, rare mutations in the TARDBP gene have been identified in familial and sporadic amyotrophic lateral sclerosis (ALS) patients. The purpose of this study was to characterize the genetic variability of the TARDBP gene in a cohort of Sardinian ALS patients. The coding region of the gene was analyzed in 97 unrelated patients previously tested negative for superoxide dismutase (SOD1) mutations. The p.Ala382Thr (c.1144G,A) mutation was found in 30 patients (30.9%). The mutation was predominant in familial ALS patients (FALS) as it was represented in 24 of 30 FALS cases (80%) (p , 0.0003). Six cases were apparently sporadic (9% of sporadic ALS patients). No further mutation of TARDBP was found in our cohort of ALS patients. Patients carrying the mutation showed spinal site of onset in 24 cases (80%), an average age at onset of 54.7 ± 11.1 years, not significantly different from patients not harboring TARDBP mutations (56.7 ± 9.6) and a female:male gender ratio of 1:1.1. The haplotype ...
Result Median survival with combined type onset (two regions simultaneously) was shorter (18 months) than with bulbar onset (26 months, p=0.01). The interval from onset to involvement of the second region correlated significantly with survival, independent of particular combinations. 5 year survival rate was 21% for lower limb onset, 18% for upper limb onset and 16% for bulbar onset. No patient with a rapid spread pattern (two regions within 3 months from onset) survived ,5 years. Early manifestations of bulbar symptoms within 1 year were associated with worse survival (p,0.001) although no significant difference in survival was seen between groups with and without bulbar symptoms (p=0.51). In terms of cumulative occurrence, symptoms spread longitudinally to adjacent regions. Bulbar function remained preserved in 27%, lower limb function in 10% and upper limb function in 2.7%. ...
Regulation of endosomal motility and degradation by amyotrophic lateral sclerosis 2/alsin : Dysfunction of alsin, particularly its putative Rab5 guanine-nucleotide-exchange factor activity, has been linked to one form of juvenile onset recessive familial amyotrophic lateral sclerosis (ALS2). Multiple lines of alsin knockout ( ALS2 -/- ) mice have been generated to model this disease. However, it remains elusive whether the Rab5-dependent endocytosis is altered in ALS2 -/- neurons. To
OBJECTIVE: To explore the value of diffusion tensor imaging applied to those specific cerebral white matter tracts consistently involved pathologically in amyotrophic lateral sclerosis as a source of prognostic biomarkers. DESIGN: Baseline clinical assessment and 3-T diffusion tensor imaging, repeated after approximately 6 months.Tract-based spatial statistics were used to assess voxel wise correlations of just the baseline diffusion tensor imaging indices with the progression rate (change in disability score/time interval) within the corticospinal tract and corpus callosum. PATIENTS: The study involved 21 patients with amyotrophic lateral sclerosis and 3 patients with primary lateral sclerosis. RESULTS: Correlation was observed between fractional anisotropy and progression rate for a region of the corticospinal tract spanning the posterior limb of the internal capsule, with a left hemisphere emphasis. Posterior limb of the internal capsule fractional anisotropy showed potential to distinguish those
Probable helicase senataxin is an enzyme that in humans is encoded by the SETX gene. This gene encodes a protein named for its homology to the Sen1p protein of fungi which has RNA helicase activity encoded by a domain at the C-terminal end of the protein. The protein encoded by this gene contains a DNA/RNA helicase domain at its C-terminal end which suggests that it may be involved in both DNA and RNA processing. Mutations in this gene have been associated with Ataxia oculomotor apraxia type 2 (AOA2) and an autosomal dominant form of juvenile amyotrophic lateral sclerosis (ALS4). GRCh38: Ensembl release 89: ENSG00000107290 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000043535 - Ensembl, May 2017 "Human PubMed Reference:". "Mouse PubMed Reference:". Chance PF, Rabin BA, Ryan SG, Ding Y, Scavina M, Crain B, Griffin JW, Cornblath DR (Apr 1998). "Linkage of the gene for an autosomal dominant form of juvenile amyotrophic lateral sclerosis to chromosome 9q34". Am J Hum Genet. 62 (3): ...
BEVERLY EAVES PERDUE GOVERNOR AMYOTROPHIC LATERAL SCLEROSIS AWARENESS MONTH 2012 BY THE GOVERNOR OF THE STATE OF NORTH CAROLINA A PROCLAMATION WHEREAS, Amyotrophic Lateral Sclerosis (ALS) is better known as Lou Gehrigs disease; and WHEREAS, ALS is a fatal neurodegenerative disease characterized by degeneration of cell bodies of the upper and lower motor neurons in the gray matter of the anterior horns of the spinal cord; and WHEREAS, the initial symptom of ALS is weakness of the skeletal muscles, especially those of the extremities; and WHEREAS, as ALS progresses, the patient experiences difficulty in swallowing, talking and breathing; and WHEREAS, ALS eventually causes muscles to atrophy and the patient becomes a functional quadriplegic; and WHEREAS, because ALS does not affect mental capacity, patients remain alert and aware of loss of motor functions; and WHEREAS, there is no known cause, means of prevention or cure for ALS, and once diagnosed, patients succumb to the illness within two to ...
TY - JOUR. T1 - Clinicopathologic variability of the GRN A9D mutation, including amyotrophic lateral sclerosis. AU - Cannon, Ashley. AU - Fujioka, Shinsuke. AU - Rutherford, Nicola J.. AU - Ferman, Tanis Jill. AU - Broderick, Daniel F.. AU - Boylan, Kevin B.. AU - Graff Radford, Neill R. AU - Uitti, Ryan J.. AU - Rademakers, Rosa V. AU - Wszolek, Zbigniew K. AU - Dickson, Dennis W. PY - 2013/5/7. Y1 - 2013/5/7. N2 - Objective: We examined the clinical and pathologic phenotypes of GRN mutation carriers with the pathogenic A9D (g.26C,A) missense mutation. Methods: Three patients with GRN A9D mutations were evaluated clinically and came to autopsy with subsequent neuropathologic examination. Results: The clinical diagnoses of patients with GRN A9D mutations were amyotrophic lateral sclerosis, atypical extrapyramidal disorder, and behavioral variant frontotemporal dementia. Immunohistochemistry for TAR DNA-binding protein 43 (TDP-43) revealed variability in morphology and distribution of pathology. ...
We have found strong evidence of a genetic association of two single nucleotide polymorphisms on chromosome 9 with sporadic ALS, in line with findings from previous independent GWAS of ALS and linkage studies of ALS-frontotemporal dementia. Our findings together with these earlier findings suggest that genetic variation at this locus on chromosome 9 causes sporadic ALS and familial ALS-frontotemporal dementia. Resequencing studies and then functional analysis should be done to identify the defective gene.. ...
The early motor manifestations of sporadic amyotrophic lateral sclerosis (ALS), while rarely documented, reflect failure of adaptive complex motor skills. The development of these skills correlates with progressive evolution of a direct corticomotoneuronal system that is unique to primates and markedly enhanced in humans. The failure of this system in ALS may translate into the split hand presentation, gait disturbance, split leg syndrome and bulbar symptomatology related to vocalisation and breathing, and possibly diffuse fasciculation, characteristic of ALS. Clinical neurophysiology of the brain employing transcranial magnetic stimulation has convincingly demonstrated a presymptomatic reduction or absence of short interval intracortical inhibition, accompanied by increased intracortical facilitation, indicating cortical hyperexcitability. The hallmark of the TDP-43 pathological signature of sporadic ALS is restricted to cortical areas as well as to subcortical nuclei that are under the direct ...
Amyotrophic lateral sclerosis as a system failure is a concept supported by the finding of consistent extramotor as well as motor cerebral pathology. The functional correlates of the structural changes detected using advanced magnetic resonance imaging techniques such as diffusion tensor imaging and voxel-based morphometry have not been extensively studied. A group of 25 patients with amyotrophic lateral sclerosis was compared to healthy control subjects using a multi-modal neuroimaging approach comprising T(1)-weighted, diffusion-weighted and resting-state functional magnetic resonance imaging. Using probabilistic tractography, a grey matter connection network was defined based upon the prominent corticospinal tract and corpus callosum involvement demonstrated by white matter tract-based spatial statistics. This amyotrophic lateral sclerosis-specific network included motor, premotor and supplementary motor cortices, pars opercularis and motor-related thalamic nuclei. A novel analysis protocol, using
Amyotrophic lateral sclerosis as a system failure is a concept supported by the finding of consistent extramotor as well as motor cerebral pathology. The functional correlates of the structural changes detected using advanced magnetic resonance imaging techniques such as diffusion tensor imaging and voxel-based morphometry have not been extensively studied. A group of 25 patients with amyotrophic lateral sclerosis was compared to healthy control subjects using a multi-modal neuroimaging approach comprising T(1)-weighted, diffusion-weighted and resting-state functional magnetic resonance imaging. Using probabilistic tractography, a grey matter connection network was defined based upon the prominent corticospinal tract and corpus callosum involvement demonstrated by white matter tract-based spatial statistics. This amyotrophic lateral sclerosis-specific network included motor, premotor and supplementary motor cortices, pars opercularis and motor-related thalamic nuclei. A novel analysis protocol, using
BACKGROUND. The genetic contribution to sporadic amyotrophic lateral sclerosis (ALS) has not been fully elucidated. There are increasing efforts to characterise the role of copy number variants (CNVs) in human diseases; two previous studies concluded that CNVs may influence risk of sporadic ALS, with multiple rare CNVs more important than common CNVs. A little-explored issue surrounding genome-wide CNV association studies is that of post-calling filtering and merging of raw CNV calls. We undertook simulations to define filter thresholds and considered optimal ways of merging overlapping CNV calls for association testing, taking into consideration possibly overlapping or nested, but distinct, CNVs and boundary estimation uncertainty.. METHODOLOGY AND PRINCIPAL FINDINGS. In this study we screened Illumina 300K SNP genotyping data from 730 ALS cases and 789 controls for copy number variation. Following quality control filters using thresholds defined by simulation, a total of 11321 CNV calls were ...
Amyotrophic lateral sclerosis (ALS) is a disease in which the motor neurons die in a progressive manner, leading to paralysis and muscle wasting. ALS is always fatal, usually through respiratory failure when the disease reaches muscles needed for breathing. Most cases are sporadic, but approximately 5-10% are familial. The first gene to be linked to familial ALS encodes the antioxidant enzyme superoxide dismutase-1 (SOD1). Today, more than 160 different mutations in SOD1 have been found in ALS patients. The mutant SOD1 proteins cause ALS by gain of a toxic property that should be common to all. Aggregates of SOD1 in motor neurons are hallmarks of ALS patients and transgenic models carrying mutant SOD1s, suggesting that misfolding, oligomerization, and aggregation of the protein may be involved in the pathogenesis. SOD1 is normally a very stable enzyme, but the structure has several components that make SOD1 sensitive to misfolding. The aim of the work in this thesis was to study misfolded SOD1 ...
Amyotrophic lateral sclerosis (ALS) is one of the progressive neurodegenerative disorders, affecting upper and lower motor neurons in the cerebral cortex, brainstem and spinal cord. Hence, the signs of damage motor neurons are both at the peripheral (eg. atrophy), and central (eg. spasticity) level. There is no effective treatment for ALS and the majority of patients die within 5 years after diagnosis, usually due to respiratory failure. Numerous studies on murine models revealed that mesenchymal stem cells (MSCs) successfully improve the clinical and pathological features of ALS. The goal of this nonrandomized, open label study is to investigate the safety and tolerability of autologous bone marrow-derived mesenchymal stem cell transplantation into the individuals with diagnosed amyotrophic lateral sclerosis. This clinical trial is conducted to test the therapeutic (neuroprotective and paracrine) effect of autologous bone marrow-derived mesenchymal stem cells (BM-MSCs). All patients enrolled ...
Amyotrophic lateral sclerosis (ALS) is a devastating neurological syndrome in which motor neurons degenerate relentlessly. Although the site of onset and the rate of spread have been studied extensively, little is known about whether focal as opposed to diffuse disease affects prognosis. We therefore tested the hypothesis that regionality of disease burden is a prognostic factor in ALS. We analysed clinical data from two large multicentre, longitudinal trials. Regionality was defined as the difference in progression rates in three domains as measured by the revised ALS Functional Rating Scale, omitting the respiratory domain from analysis. We used death by trial end as the outcome variable and tested this by logistic regression against predictor variables including regionality and overall rate of disease progression. There were 561 patients. Regionality of disease was independently associated with significantly higher chance of death by study end (odds ratio most diffuse against most focal category 0
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrigs disease, is a rare disease with extreme between-subject variability, especially with respect to rate of disease progression. This makes modelling a subjects disease progression, which is measured by the ALS Functional Rating Scale (ALSFRS), very difficult. Consider the problem of predicting a subjects ALSFRS score at 9 or 12 months after a given time-point. We obtained ALS subject data from the Pooled Resource Open-Access ALS Clinical Trials Database, a collection of data from various ALS clinical trials. Due to the typical linearity of the ALSFRS, we consider several Bayesian hierarchical linear models. These include a mixture model (to account for the two potential classes of
Title:Metallothionein is a Potential Therapeutic Strategy for Amyotrophic Lateral Sclerosis. VOLUME: 23 ISSUE: 33. Author(s):Shin-ichi Ono*. Affiliation:Laboratory of Clinical Medicine, School of Pharmacy, Nihon University, 7-1, 7-chome, Narashinodai, Funabashi, Chiba 274-8555. Keywords:Metallothionein, intracellular Cu homeostasis, SOD1 mutation, cysteine111, Cu chaperons, Cu secretion.. Abstract:Lou Gehrigs disease, a synonym of amyotrophic lateral sclerosis, is an adult-onset lethal neurodegenerative disorder. Irrespective of extensive efforts to elucidate the pathogenesis of the disease and searches for therapies, no favorable pharmacotherapeutic strategies have yet to be proposed. In a popular rodent model of ALS, G93A SOD1 strain of mouse, intracellular copper conditions were geared toward copper accumulation inside cells, resulting in an acceleration of oxidative stress and apoptotic process. Disruption of intracellular copper homeostasis was common to transgenic mice expressing human ...
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease resulting in death, usually from respiratory failure, within 2-3 years of symptom onset. Non-invasive ventilation (NIV) is a treatment that when given to patients in respiratory failure leads to improved survival and quality of life. Diaphragm pacing (DP), using the NeuRx/4(®) diaphragm pacing system (DPS)™ (Synapse Biomedical, Oberlin, OH, USA), is a new technique that may offer additional or alternative benefits to patients with ALS who are in respiratory failure. OBJECTIVE: The Diaphragm Pacing in patients with Amyotrophic Lateral Sclerosis (DiPALS) trial evaluated the effect of DP on survival over the study duration in patients with ALS with respiratory failure. DESIGN: The DiPALS trial was a multicentre, parallel-group, open-label, randomised controlled trial incorporating health economic analyses and a qualitative longitudinal substudy. PARTICIPANTS: Eligible participants had a diagnosis of ALS (ALS ...
Amyotrophic lateral sclerosis definition is - a rare progressive degenerative fatal disease affecting the motor neurons, usually beginning in middle age, and characterized especially by increasing and spreading muscular weakness and atrophy -abbreviation ALS-called also Lou Gehrigs disease.
Amyotrophic lateral sclerosis Definition Amyotrophic lateral sclerosis (ALS) is a disease that breaks down tissues in the nervous system [1] (a neurodegenerative disease) of unknown cause that affects the nerves responsible for movement.
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Author: Lill, C. M. et al.; Genre: Journal Article; Published in Print: 2011; Keywords: Amyotrophic Lateral Sclerosis/*genetics/physiopathology; *Databases, Factual; Genetic Predisposition to Disease; Genotype; Humans; Mutation; Phenotype; Software; User-Computer Interface; Title: Keeping up with genetic discoveries in amyotrophic lateral sclerosis: the ALSoD and ALSGene databases
The pipeline guide provides a snapshot of the global therapeutic landscape of Amyotrophic Lateral Sclerosis. The pipeline guide reviews pipeline therapeutics for Amyotrophic Lateral Sclerosis by companies and universities/research institutes based on information derived from company and industry-specific sources.
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Objective: Motor neurons (MNs) die in amyotrophic lateral sclerosis (ALS), a clinically heterogeneous neurodegenerative disease of unknown aetiology. In human or rodent studies, MN loss is preceded by increased excitability. As increased neuronal excitability correlates with structural changes in dendritic arbors and spines, we have examined longitudinal changes in dendritic structure in vulnerable neuron populations in a mouse model of familial ALS. Methods: We used a modified Golgi-Cox staining method to determine the progressive changes in dendritic structure of hippocampal CA1 pyramidal neurons, striatal medium spiny neurons, and resistant (trochlear, IV) or susceptible (hypoglossal, XII; lumbar) MNs from brainstem and spinal cord of mice over-expressing the human SOD1G93A (SOD1) mutation, in comparison to wild-type (WT) mice, at 4 postnatal (P) ages of 8-15, 28-35, 65-75 and 120 days. Results: In SOD1 mice, dendritic changes occur at pre-symptomatic ages in both XII and spinal cord lumbar MNs.
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal neurodegenerative diseases that are related genetically and pathologically. Mutations in the UBQLN2gene, encoding the...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of the motor nervous system. We show using multielectrode array and patch-clamp recordings that hyperexcitability detected by clinical neurophysiological studies of ALS patients is recapitulated in induced pluripotent stem cell-derived motor neurons from ALS patients harboring superoxide dismutase 1 (SOD1), C9orf72, and fused-in-sarcoma mutations. Motor neurons produced from a genetically corrected but otherwise isogenic SOD1(+/+) stem cell line do not display the hyperexcitability phenotype. SOD1(A4V/+) ALS patient-derived motor neurons have reduced delayed-rectifier potassium current amplitudes relative to control-derived motor neurons, a deficit that may underlie their hyperexcitability. The Kv7 channel activator retigabine both blocks the hyperexcitability and improves motor neuron survival in vitro when tested in SOD1 mutant ALS cases. Therefore, electrophysiological characterization of human stem cell-derived neurons ...
A hexanucleotide repeat expansion in the C9orf72 gene is the most common cause of inherited forms of the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Both loss-of-function and gain-of-function mechanisms have been proposed to underlie this disease, but the pathogenic pathways are not fully understood. To better understand the involvement of different cell types in the pathogenesis of ALS, we systematically analyzed the distribution of promoter activity of the mouse ortholog of C9orf72 in the central nervous system. We demonstrate that C9orf72 promoter activity is widespread in both excitatory and inhibitory neurons as well as in oligodendrocytes and oligodendrocyte precursor cells. In contrast, few microglia and astrocytes exhibit detectable C9orf72 promoter activity. Although at a gross level, the distribution of C9orf72 promoter activity largely follows overall cellular density, we found that it is selectively enriched in subsets of neurons and glial cells that degenerate in ...
Researchers have linked newly discovered gene mutations to some cases of the progressive fatal neurological disease amyotrophic lateral sclerosis – ALS, also known as Lou Gehrigs disease. Shedding light on how ALS destroys ...
Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS. Raoul, Cédric; Abbas-Terki, Toufik; Bensadoun, Jean-Charles; Guillot, Sandrine; Haase, Georg; Szulc, Jolanta; Henderson, Christopher E.; Aebischer, Patrick // Nature Medicine;Apr2005, Vol. 11 Issue 4, p423 Mutations in Cu/Zn superoxide dismutase (encoded by SOD1), one of the causes of familial amyotrophic lateral sclerosis (ALS), lead to progressive death of motoneurons through a gain-of-function mechanism. RNA interference (RNAi) mediated by viral vectors allows for long-term reduction in gene... ...
Amyotrophic lateral sclerosis (ALS), a fatal disease causing progressive loss of motor neurons, still has no effective treatment. We developed a phenotypic screen to repurpose existing drugs using ALS motor neuron survival as readout. Motor neurons were generated from induced pluripotent stem cells (iPSCs) derived from an ALS patient with a mutation in superoxide dismutase 1 (SOD1). Results of the screen showed that more than half of the hits targeted the Src/c-Abl signaling pathway. Src/c-Abl inhibitors increased survival of ALS iPSC-derived motor neurons in vitro. Knockdown of Src or c-Abl with small interfering RNAs (siRNAs) also rescued ALS motor neuron degeneration. One of the hits, bosutinib, boosted autophagy, reduced the amount of misfolded mutant SOD1 protein, and attenuated altered expression of mitochondrial genes. Bosutinib also increased survival in vitro of ALS iPSC-derived motor neurons from patients with sporadic ALS or other forms of familial ALS caused by mutations in TAR DNA ...
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal, degenerative disorder of motor neurons. The hallmark of this disease is the selective death of motor neurons in the brain and spinal cord, leading to paralysis of voluntary muscles. Mutant superoxide dismutase 1 (SOD1), as seen in some familial ALS (FALS) cases, is unstable, forming aggregates in the motor neuron cytoplasm, axoplasm and mitochondria. Within mitochondria, mutant SOD1 may interfere with the anti-apoptotic function of Bcl-2, affect mitochondrial import by interfering with the translocation machinery (TOM/TIM), and generate toxic free radicals (ROS). Reactive oxygen species (ROS), produced within mitochondria, inhibit the function of EAAT2, the main glial glutamate transporter protein, responsible for most of the reuptake of synaptically released glutamate. Glutamate excess increases intracellular calcium, which enhances oxidative stress and mitochondrial damage. Mutant SOD1 can also trigger oxidative reactions , which ...
Two forms of amyotrophic lateral sclerosis (ALS) are known, the familial (FALS), due in part to mutations in superoxide dismutase 1 (SOD1), and the sporadic (SALS), which accounts for > 90% of all cases. The cause of SALS is not known, but excitotoxicity due to overactivation of glutamate receptors may mediate the motor neuron degeneration in the spinal cord, which is the hallmark of this disease. Overactivation of calcium-permeable (alpha-amino-3-hydroxy-5-isoxazole propionate receptors lacking the subunit glutamate receptor 2, leading to an increase in calcium cytoplasmic concentration, seems to play an important role in the mechanism of neuronal death. The knowledge of this mechanism, in addition to other factors, provides several possible targets for therapeutic strategies that are reviewed in this article. Some of these strategies have proven to be partially effective in both human mutant superoxide dismutase 1 transgenic rodents (FALS model) and the few existing in vivo models of spinal ...
The purpose of this website is to facilitate the mission of the WFN-ALS. It is a work in progress, which is updated monthly to keep pace with the rapid advances being made in clinical care and basic research by members of this group and our colleagues around the globe.
Embryonic stem (ES) cells can be induced to differentiate into motor neurons (MN). Animal models resembling MN degeneration and paralysis observed in familial amyotrophic lateral sclerosis (ALS) have been previously reported. In this work, we aimed to investigate whether transplanted MN could prevent motor deterioration in transgenic rats expressing a mutant form of human superoxide dismutase 1 (hSOD1G93A) associated with inherited ALS. Mouse ES cells were differentiated to neurons that express green fluorescent protein (GFP) under the promoter of the MN-specific gene hb9, as well as molecular markers indicative of MN identity. Cells were grafted into the lumbar spinal cord of adult wild-type (WT) or hSOD1G93A rats at 10 weeks of age, when transgenic animals are presymptomatic. Grafted cells with MN phenotype can survive for at least 1 week in hSOD1G93A animals. To quantitatively evaluate motor performance of WT and transgenic rats, we carried out weekly rotarod tests starting when the animals ...
Oligodendrocytes associate with axons to establish myelin and provide metabolic support to neurons. In the spinal cord of amyotrophic lateral sclerosis (ALS) mice, oligodendrocytes downregulate transporters that transfer glycolytic substrates to neurons and oligodendrocyte progenitors (NG2(+) cells) exhibit enhanced proliferation and differentiation, although the cause of these changes in oligodendroglia is unknown. We found extensive degeneration of gray matter oligodendrocytes in the spinal cord of SOD1 (G93A) ALS mice prior to disease onset. Although new oligodendrocytes were formed, they failed to mature, resulting in progressive demyelination. Oligodendrocyte dysfunction was also prevalent in human ALS, as gray matter demyelination and reactive changes in NG2(+) cells were observed in motor cortex and spinal cord of ALS patients. Selective removal of mutant SOD1 from oligodendroglia substantially delayed disease onset and prolonged survival in ALS mice, suggesting that ALS-linked genes ...
In July 2015 he joined the Institute of Biomedical Technologies as research scholar but he has been collaborating continuously with it for 8 years.. His research is focused on the intercellular signaling mediated by extracellular vesicles (i.e., exosomes and microvesicles), especially for what concerns death and survival signals in neurodegenerative diseases (Amyotrophic Lateral Sclerosis and Multiple Sclerosis) and cancer.. In 2008 he received his Masters Degree in "Medical Biotechnologies and Molecular Medicine" from the University of Bari where he earned also his Ph.D. in "Biochemistry, Molecular Biology and Bioinformatics" in 2012.. In 2009 he was awarded a 1-year research scholarship from the University of Milan for the project "Analysis and Characterization of Alternative Splicing Isoforms Differentially Expressed in Normal and Tumoral Tissues".. During his doctoral studies (2009-2012) his research lines have been focused mainly on the study of gene expression alterations at transcript ...
Stoica L, Todeasa SH, Cabrera GT, Salameh JS, ElMallah MK, Mueller C, Brown RH, Sena-Esteves M. Adeno-associated virus-delivered artificial microRNA extends survival and delays paralysis in an amyotrophic lateral sclerosis mouse model. Ann Neurol. 2016 Apr; 79(4):687-700 ...
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder with an adult-onset around 54-67 years old, and it belongs to a group of conditions known as Motor Neurone Diseases (MND). Its clinical hallmark is the degeneration of both upper and lower motor neurons, leading to progressive muscle atrophy and weakness, and ultimately to paralysis. Death, often resulting from swallowing problems and respiratory failure, usually occurs within 2-4 years from disease onset, although 5-10% of ALS patients survive over 10 years[1].
... (ALS), also known as motor neuron disease, is characterized by the degeneration of both upper and lower motor neurons, which leads to muscle weakness and eventual paralysis. Until recently, ALS was classified primarily within the neuromuscular domain, although new imaging and neuropathological data have indicated the involvement ... read more of the non-motor neuraxis in disease pathology. In most patients, the mechanisms underlying the development of ALS are poorly understood, although a subset of patients have familial disease and harbour mutations in genes that have various roles in neuronal function. Two possible disease-modifying therapies that can slow disease progression are available for ALS, but patient management is largely mediated by symptomatic therapies, such as the use of muscle relaxants for spasticity and speech therapy for dysarthria. show less ...
In addition to motor neurone degeneration, up to 50% of amyotrophic lateral sclerosis (ALS) patients present with cognitive decline. Understanding the neurobiological changes underlying these...
Background: Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation (LF-RNS) are frequently observed in patients with amyotrophic lateral sclerosis (ALS). The pathological mechanism of this phenomenon remains unknown. This study aimed to illuminate the features of RNS in Chinese patients with ALS. Methods: Clinical and electrophysiological data of 146 probable and definite ALS patients who underwent RNS were retrospectively enrolled and analyzed. LF-RNS (3 Hz) was performed in trapezius, deltoid, abductor digiti minimi (ADM), quadriceps femoris, and tibialis anterior. High-frequency RNS (HF-RNS, 10 Hz) was performed only in ADM. The two-sample t-test and Chi-squared test were used for statistical analysis. Results: Decremental responses to LF-RNS (≥10%) in at least one muscle were detected in 83 (56.8%) of the cases and were most commonly seen in trapezius and deltoid. The incidence of decremental response was higher in patients with upper limb ...
Non-cell autonomous toxicity is one of the potential mechanisms implicated in the etiopathogenesis of amyotrophic lateral sclerosis (ALS). However, the exact role of glial cells in ALS pathology is yet to be fully understood. In a cellular model recapitulating the pathology of sporadic ALS, we have studied the inflammatory response of astroglia following exposure to the cerebrospinal fluid from ALS patients (ALS-CSF). Various inflammatory markers including pro-inflammatory and anti-inflammatory cytokines, COX-2, PGE-2, trophic factors, glutamate, nitric oxide (NO), and reactive oxygen species (ROS) were analyzed in the rat astroglial cultures exposed to ALS-CSF and compared with the disease control or normal controls. We used immunofluorescence, ELISA, and immunoblotting techniques to investigate the protein expression and real-time PCR to study the messenger RNA (mRNA) expression. Glutamate, NO, and ROS were estimated using appropriate biochemical assays. Further, the effect of conditioned medium from
Chinese journal of cell biology 2004,26:635-639 The construction of amyotrophic lateral sclerosis spinal cord model Liu Weigang; Wang Xiaojuan; Xiao Xiangjian Read more: en/upfiles/pdf/The_construction_of_amyotrophic_lateral_sclerosis_spinal_cord_mo
Dominant mutations in superoxide dismutase 1 (SOD1) cause degeneration of motor neurons in a subset of inherited amyotrophic lateral sclerosis (ALS). The pathogenetic process mediated by misfolded and/or aggregated mutant SOD1 polypeptides is hypothesized to be suppressed by protein refolding. This genetic study is aimed to test whether mutant SOD1-mediated ALS pathology recapitulated in mice could be alleviated by overexpressing a longevity-related deacetylase SIRT1 whose substrates include a transcription factor heat shock factor 1 (HSF1), the master regulator of the chaperone system. We established a line of transgenic mice that chronically overexpress SIRT1 in the brain and spinal cord. While inducible HSP70 (HSP70i) was upregulated in the spinal cord of SIRT1 transgenic mice (PrP-Sirt1), no neurological and behavioral alterations were detected. To test hypothetical benefits of SIRT1 overexpression, we crossbred PrP-Sirt1 mice with two lines of ALS model mice: A high expression line that exhibits a
Mitochondrial impairment has been implicated in the pathogenesis of the amyotrophic lateral sclerosis (ALS). Furthermore, mitochondrial-specific polymorphisms were previously related to other neurodegenerative diseases, such as Parkinson, Friedreich and Alzheimer disease. To investigate if specific genetic polymorphisms within the mitochondrial genome (mtDNA) could act as susceptibility factors and contribute to the clinical expression of sporadic ALS (sALS), we have genotyped predefined European mtDNA haplogroups in 222 Italian patients with sALS and 151 matched controls. Individuals classified as haplogroup I demonstrated a significant decrease in risk of ALS versus individuals carrying the most common haplogroup, H (odds ratio 0.08, 95% confidence interval 0.04-0.4, p < 0.01). Further stratification of the dataset by sex, age and site of onset of disease and survival failed to reach significance for association. Our study provides evidence of the contribution of mitochondrial variation to the ...
Bim Links ER Stress and Apoptosis in Cells Expressing Mutant SOD1 Associated with Amyotrophic Lateral Sclerosis Journal Articles Refereed ...
Amyotrophic lateral sclerosis (ALS) is the most common type of adult-onset motor neuron disease. Adult-onset motor neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive degeneration and loss of motor neurons.
Speech intelligibility and its phonetic and acoustic correlates were studied in a group of 10 women with amyotrophic lateral sclerosis (ALS). Intelligibility assessment with a word-identification test indicated that the most disrupted phonetic features pertained to velopharyngeal valving, lingual function for consonant contrasts of place and manner, and syllable shape. An acoustic signature analysis based on trajectories of the first and second formants in selected monosyllabic test words revealed that the mean slope of the second formant (F2) was reduced compared with that of a normal geriatric control group. This F2 slope reduction is interpreted to reflect loss of lingual motoneurons. Acoustic measures of phonatory function for sustained vowel prolongation demonstrated abnormalities in fundamental frequency, perturbations of frequency (jitter) and amplitude (shimmer), and signal-to-noise ratio. The data for women with ALS are compared with data for a normal geriatric control group of women and with
Objectives - To determine the causes and place of death in a cohort of Italian patients with amyotrophic lateral sclerosis (ALS). A better understanding of the likely causes of death in ALS might improve the palliative care at the end-of-life, whereas knowing the place of death will help to verify the need for highly specialized care services, e.g. hospice and nursing home. Patients and methods - Between 2000 and 2008, 182 ALS patients (onset: spinal, 127; bulbar, 55; M/F: 1.6) were followed in a single ALS Tertiary Centre in Palermo, Sicily, Italy until death. Medical data for each individual patient were recorded in a large database throughout the disease course. Information concerning causes and place of death were obtained by consultation with relatives or the family physician. Results - Respiratory failure (terminal respiratory insufficiency, pneumonia) was the most frequent cause of death (81.3%), which included six cases (3.3%) who requested a terminal sedation. Sudden death and death ...
Amyotrophic lateral sclerosis (ALS) is a devastating neurological disease with no effective treatment. Here we report the results of a moderate-scale sequencing study aimed at identifying new genes contributing to predisposition for ALS. We performed whole exome sequencing of 2,874 ALS patients and compared them to 6,405 controls. Several known ALS genes were found to be associated, and the non-canonical IκB kinase family TANK-Binding Kinase 1 (TBK1) was identified as an ALS gene. TBK1 is known to bind to and phosphorylate a number of proteins involved in innate immunity and autophagy, including optineurin (OPTN) and p62 (SQSTM1/sequestosome), both of which have also been implicated in ALS. These observations reveal a key role of the autophagic pathway in ALS and suggest specific targets for therapeutic intervention.. ...
Teva Provides Update on Glatiramer Acetate 40mg for Amyotrophic Lateral Sclerosis (ALS) GA 40 mg was safe and well tolerated in ALS patients; however, study did not meet primary end point
What is ALS? Amyotrophic lateral sclerosis (ALS), often called Lou Gehrig's disease, is a progressive and invariably fatal neurodegenerative disease.
Over the past ten years, there has been an increasing recognition that syndromes of frontotemporal dysfunction (FTD) are a common occurrence in patients with amyotrophic lateral sclerosis (ALS). Such syndromes may be present in as many as 60% of patients with ALS. Conversely, the occurrence of motor neuron dysfunction in patients with clinically pure frontotemporal dementia is increasingly recognized.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that imposes a great burden on the patient, the patients family and society. However, we lack information about the total disease burden at a national level, especially regarding costs before and after diagnosis and the consequences for spouses. We aimed to estimate the factual direct and indirect costs of ALS in a national sample. Using records from the Danish National Patient Registry (1998-2009), 2,394 patients with ALS were identified and subsequently compared with 9,575 randomly chosen control subjects matched for age, gender and geographic area/civil status. Direct costs, including frequencies of primary and sector contacts and procedures, and medication from primary and secondary sectors, were obtained from the Danish Ministry of Health, the Danish Medicines Agency, and the National Health Security. Indirect costs included labour supply and social transfer payments, and were based on income data derived from ...
About Amyotrophic Lateral Sclerosic Association (Lou Gehrigs Disease) Amyotrophic lateral sclerosis (ALS), often referred to as Lou Gehrigs Disease, is a
We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05-21.6; P=1 × 10-4) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10-7). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08-1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in ...
Motor neuron disease, also known as amyotrophic lateral sclerosis (ALS) or Lou Gehrigs disease, is a progressive disease that attacks motor neurons, components of the nervous system that connect the brain with the skeletal muscles. Skeletal muscles are the muscles involved with voluntary movement, like walking and talking. When your brain sends a signal telling a part of your body to move, the motor neurons transmit the command to the skeletal muscles. The muscles respond by contracting. In ALS, the motor neurons deteriorate and eventually die, and though a persons brain is fully functioning and alert, the command to move never reaches the muscle. The patient may want to reach for a glass of water, for example, but is not able to do it because the lines of communication from the brain to the arm and hand muscles have been destroyed. The muscles eventually waste away from disuse, and a person in the late stages of Lou Gehrigs disease is completely paralyzed. Currently, there is no cure for ALS,
Although epidemiologic studies have examined the risk of amyotrophic lateral sclerosis (ALS) in relation to cancer, none have been large population-based studies using incident ALS and adjusting for medical surveillance. Addressing those limitations, all first primary cancer cases from the Surveillance, Epidemiology and End Results (SEER) Program (1992-2005), linked to Medicare claims data were used. Cases were followed from cancer diagnosis until the earliest date of ALS diagnosis, a break in Medicare claims data, death, age 85 or December 31, 2005. A comparison group from a 5% random Medicare sample in the SEER areas who were cancer-free and censored as above, or until a cancer diagnosis were selected. ALS outcomes were derived from medical claims. The proportional hazards models to estimate ALS hazard ratios (HRs), using age as the time scale, adjusting for sex, race and physician visits, and stratifying the baseline hazard on birth year and SEER registry were used. A total of 303 ALS cases were
Applications will end up being through a two-stage procedure - outline proposals to end up being assessed by way of a bespoke panel wholl advise on those to end up being invited to submit complete applications. Related StoriesRXi Pharmaceuticals starts Phase 1/2 scientific trial in ophthalmologyUsing smartphone to identify diabetes marker in salivaResearch sheds brand-new light on the fight tonsil cancer.. Astrocytes may donate to Lou Gehrigs disease Two papers by Columbia and Harvard experts report for the very first time that astrocytes , which carry a mutated gene recognized to cause some instances of amyotrophic lateral sclerosis , induce motor neuron loss of life. This means that that astrocytes might donate to ALS by releasing a toxic factor that damages neurons.Moreover, a lot of individuals with type 2 diabetes will establish retinopathy as their underlying disease progresses. With the global epidemic of type 2 diabetes, this predicament is defined to worsen as over 438 million folks ...
Also known as Lou Gehrigs disease in North America and Motor Neuron disease in some British Commonwealth countries, amyotrophic lateral sclerosis or ALS is a
article{f694784f-8231-4893-a2d2-c04b98a02454, abstract = {Objective: Cigarette smoking has been reported as probable risk factor for Amyotrophic Lateral Sclerosis (ALS), a poorly understood disease in terms of aetiology. The extensive longitudinal data of the European Prospective Investigation into Cancer and Nutrition (EPIC) were used to evaluate age-specific mortality rates from ALS and the role of cigarette smoking on the risk of dying from ALS. Methods: A total of 517,890 healthy subjects were included, resulting in 4,591,325 person-years. ALS cases were ascertained through death certificates. Cox hazard models were built to investigate the role of smoking on the risk of ALS, using packs/years and smoking duration to study dose-response. Results: A total of 118 subjects died from ALS, resulting in a crude mortality rate of 2.69 per 100,000/year. Current smokers at recruitment had an almost two-fold increased risk of dying from ALS compared to never smokers (HR = 1.89, 95% C.I. 1.14-3.14), ...
Amyotrophic lateral sclerosis (ALS;Lou Gehrigs disease) is a fatal neurodegenerative disorder that leads to rapidly progressive paralysis and respiratory failur...
Amyotrophic lateral sclerosis (ALS) is a progressing motor neuron disease characterised by loss of function (LOF) of motor neurons, which are essential for controlling voluntary muscle activity such as walking, breathing and speaking. This condition leads to premature death with a median survival of about two to three years. Disease likely arises from a combination of genetic susceptibility [1-3] and environmental factors [4]. However, our understanding of what these factors are and how they contribute to disease risk, onset and progression remain incomplete.. Likely due to this limited understanding of disease aetiology, there has been limited success in designing any effective treatment for ALS. To date, the most important fundamental insights into the underlying cellular mechanisms have resulted from genetic studies of the known causal mutations [5]. However, highly penetrant identified mutations still only account for up to 10% of cases [6, 7] and thus more work needs to be done. ...
Between 1987 and 2005, the authors conducted a case-control study nested within the entire Swedish population to investigate whether loss of a child due to death is associated with the risk of amyotrophic lateral sclerosis (ALS). The study comprised 2,694 incident ALS cases and five controls per case individually matched by year of birth, gender, and parity. Odds ratios and their corresponding 95% confidence intervals for ALS were estimated by using conditional logistic regression models. Compared with that for parents who never lost a child, the overall odds ratio of ALS for bereaved parents was 0.7 (95% confidence interval (CI): 0.6, 0.8) and decreased to 0.4 (95% CI: 0.2, 0.8) 11-15 years after the loss. The risk reduction was also modified by parental age at the time of loss, with the lowest odds ratio of 0.4 (95% CI: 0.2, 0.9) for parents older than age 75 years. Loss of a child due to malignancy appeared to confer a lower risk of ALS (odds ratio = 0.5, 95% CI: 0.3, 0.8) than loss due to ...
The mission of the Northeast ALS Consortium (NEALS) is to rapidly translate scientific advances into clinical research and new treatments for people with Amyotrophic Lateral Sclerosis (ALS) and motor neuron disease.
The mission of the Northeast ALS Consortium (NEALS) is to rapidly translate scientific advances into clinical research and new treatments for people with Amyotrophic Lateral Sclerosis (ALS) and motor neuron disease.
A neurodegenerative disease, Amyotrophic lateral sclerosis (ALS) has come with an occurrence rate in France similar to multiple sclerosis (two to three new cases per year for every 100,000 residents).
Looking for more information on ALS? This eMedTV segment continues the discussion on amyotrophic lateral sclerosis, with details on common symptoms, possible causes, and how a diagnosis is made.
Amyotrophic lateral sclerosis is a disease of which the underlying cause and pathogenesis are unknown. Cumulatative data clearly indicates an active participation by the immune system in the disease. An increasingly recognized theory suggests a non-cell autonomous mechanism, meaning that multiple cells working together are necessary for the pathogenesis of the disease. Observed immune system alterations could indicate an active participation in this mechanism. Damaged motor neurons are able to activate microglia, astrocytes and the complement system, which further can influence each other and contribute to neurodegeneration. Infiltrating peripheral immune cells appears to correlate with disease progression, but their significance and composition is unclear. The deleterious effects of this collaborating system of cells appear to outweigh the protective aspects, and revealing this interplay might give more insight into the disease. Markers from the classical complement pathway are elevated where ...
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease typically causing death within three years. Understanding the impact of disease on patients using health utility at different stages of ALS would allow meaningful cost-benefit analysis of new potential therapies. A common health-related quality of life measurement, developed and validated for the UK, is the EQ-5D. Using clinical trial data from the LiCALS study, we calculated health utility using the EQ-5D for each Kings ALS clinical stage from 214 patients. We analysed whether health utility, and other health-related measures, significantly changed between each of the clinical stages. Results showed that mean health utility decreased by 0.487 (the scale runs from 1 to - 0.594) between clinical stages 2A and 4. Emotional states, measured using the Hospital Anxiety and Depression Scale (HADS), showed worsening depression and anxiety scores as ALS progressed. Age of onset, disease onset, gender and treatment group were not ...
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease typically causing death within three years. Understanding the impact of disease on patients using health utility at different stages of ALS would allow meaningful cost-benefit analysis of new potential therapies. A common health-related quality of life measurement, developed and validated for the UK, is the EQ-5D. Using clinical trial data from the LiCALS study, we calculated health utility using the EQ-5D for each Kings ALS clinical stage from 214 patients. We analysed whether health utility, and other health-related measures, significantly changed between each of the clinical stages. Results showed that mean health utility decreased by 0.487 (the scale runs from 1 to - 0.594) between clinical stages 2A and 4. Emotional states, measured using the Hospital Anxiety and Depression Scale (HADS), showed worsening depression and anxiety scores as ALS progressed. Age of onset, disease onset, gender and treatment group were not predictors of
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurons that causes progressive paralysis and eventually results in death from respiratory failure. Environmental factors that trigger ALS might result in a pattern of geographical clustering of cases. We tested this hypothesis using the South-East England ALS population register, which covers south-east London, Kent and parts of neighbouring counties. METHODS: The registers catchment area was divided into postcode districts and sectors. The expected rates of ALS (adjusted for age and sex) were compared with the observed rates using a standardised residuals method and the SaTScan programme. RESULTS: There were 406 cases of ALS identified in the catchment area during the study period. Four of the 126 postcode districts, all in Greater London, had residuals |2.5 SDs from the mean. Similarly, there were 15 postcode sectors (out of 420) that had residuals |1.96 SDs from the mean. Nine of these were in Greater London. SaTScan
Amyotrophic lateral sclerosis (ALS) is a highly progressive and debilitating neurodegenerative disease, which usually leads to the death of affected individuals within a few years after the onset of symptoms. ALS is currently incurable and very little is known about its pathophysiology. Finding validated biomarkers will help us to advance our understanding of ALS etiology and find better strategies for early diagnosis and management of the disease. The main aim of the present systematic review is to evaluate the concentration of 11 frequently reported biomarkers for ALS in peripheral blood and CSF of patients diagnosed with ALS compared with controls. This systematic review protocol has been established according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Protocol (PRISMA-P) 2015 guideline. We will include all types of observational studies with human subjects that investigated the concentrations of intended biomarkers (amyloid beta (Aβ-42), tau and phosphorylated tau (p
Curious to know more, I looked for previous reports on this topic but I came out not any wiser. Older research have given conflicting results on the links between MND and cancer. Take this paper published in the International Journal of Cancer titled The risk of amyotrophic lateral sclerosis after cancer in U.S. elderly adults: a population-based prospective study. This found no links at all, as did another paper published in Journal of Neurology titled Prior medical conditions and the risk of amyotrophic lateral sclerosis. On the other hand, other researchers found that people with MND were at a higher risk of cancer. An example is this paper titled The association between cancer and amyotrophic lateral sclerosis, published in Cancer Causes and Control. This reported a link between MND and melanoma, and with tongue cancer. The bulk of the research before now however suggests that there is no link. Take this paper published in Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, and ...
Johns Hopkins scientists say they have evidence from animal studies that a type of central nervous system cell other than motor neurons plays a fundamental role in the development of amyotrophic lateral sclerosis (ALS), a ...
Ice bucket challenges aside, treatment for amyotrophic lateral sclerosis (ALS), a progressive and fatal neurological disease, is limited. However, promising research is ongoing.
Samad Jahandideh, Albert A. Taylor, Danielle Beaulieu, Mike Keymer, Lisa Meng, Amy Bian, Nazem Atassi, Jinsy Andrews & David L. Ennist. Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, on December 20, 2017. This article is accessible on-line by clicking here.. ...
Occupational exposures are suspected of contributing to the risk of amyotrophic lateral sclerosis (ALS), but results of epidemiologic studies have been inconsistent. The authors prospectively assessed the relation between occupation and ALS mortality among more than 1 million participants in the Cancer Prevention Study II of the American Cancer Society. Follow-up from 1989 through 2002 identified 507 ALS deaths among men and 430 among women. Adjusted rate ratios were calculated by using Mantel-Haenszel weights and Cox proportional hazards. Among men, elevated ALS mortality was found for programmers (rate ratio = 4.55, 95% confidence interval: 1.46, 14.2; p = 0.009) and laboratory technicians (rate ratio = 1.96, 95% confidence interval: 1.04, 3.66; p = 0.04). Occupations previously associated with increased risk of ALS for which no increased risk was found included farmers, electricians, and welders, although the numbers of electricians (eight ALS deaths) and welders (two ALS deaths) were small. ...
Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrigs disease is a devastating and rapidly fatal disease with currently only one available, FDA-approved, modestly effective treatment.
Lithium doesnt delay progression of amyotrophic lateral sclerosis (ALS), or Lou Gehrigs disease, and shouldnt be used to treat patients with the disease, researchers say.
Looking for online definition of amyotrophic lateral sclerosis type 8 in the Medical Dictionary? amyotrophic lateral sclerosis type 8 explanation free. What is amyotrophic lateral sclerosis type 8? Meaning of amyotrophic lateral sclerosis type 8 medical term. What does amyotrophic lateral sclerosis type 8 mean?
OBJECTIVE: To assess the transactive response DNA-binding protein 43 (TDP-43) burden in familial forms of Alzheimer disease (FAD) and Down syndrome (DS) to determine whether TDP-43 inclusions are also present. DESIGN: Using standard immunohistochemical techniques, we examined brain tissue samples from 42 subjects with FAD and 14 with DS. RESULTS: We found pathological TDP-43 aggregates in 14.0% of participants (6 of 42 and 2 of 14 participants with FAD and DS, respectively). In both FAD and DS, TDP-43 immunoreactivity did not colocalize with neurofibrillary tangles. Occasionally participants with FAD or DS had TDP-43-positive neuropil threads or dots. Overall, the amygdala was most commonly affected, followed by the hippocampus, with no TDP-43 pathology in neocortical regions. A similar distribution of TDP-43 inclusions is seen in sporadic Alzheimer disease, but it differs from that seen in amyotrophic lateral sclerosis and frontotemporal dementia. CONCLUSIONS: Transactive response DNA-binding ...
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive loss of motor neurons. However, ALS has been recognized to also involve non-motor systems. Subclinical involvement of the autonomic system in ALS has been described. The recently developed C-13-octanoic acid breath test allows the noninvasive measurement of gastric emptying. With this new technique we investigated 18 patients with ALS and 14 healthy volunteers. None of the patients had diabetes mellitus or other disorders known to cause autonomic dysfunction. The participants received a solid standard test meal labeled with C-13-octanoic acid. Breath samples were taken at 15-min intervals for 5 h and were analyzed for (CO2)-C-13 by isotope selective nondispersive infrared spectrometry. Gastric emptying peak time (t(peak)) and emptying half time (t(1/2)) were determined. All healthy volunteers displayed normal gastric emptying with a mean emptying t(1/2) of 138 +/- 34 (range 68-172) min. Gastric ...
Neurochemical biomarkers are urgently sought in ALS. Metabolomic analysis of cerebrospinal fluid (CSF) using proton nuclear magnetic resonance ((1)H-NMR) spectroscopy is a highly sensitive method capable of revealing nervous system cellular pathology. The (1)H-NMR CSF metabolomic signature of ALS was sought in a longitudinal cohort. Six-monthly serial collection was performed in ALS patients across a range of clinical sub-types (n = 41) for up to two years, and in healthy controls at a single time-point (n = 14). A multivariate statistical approach, partial least squares discriminant analysis, was used to determine differences between the NMR spectra from patients and controls. Significantly predictive models were found using those patients with at least one years interval between recruitment and the second sample. Glucose, lactate, citric acid and, unexpectedly, ethanol were the discriminating metabolites elevated in ALS. It is concluded that (1)H-NMR captured the CSF metabolomic signature associated
Workplace exposure to very low frequency electromagnetic fields may be linked to a doubling in risk of developing the most common form of motor neurone disease -- amyotrophic lateral sclerosis, or ALS for short -- suggests research published online in Occupational & Environmental Medicine.
A new toxic entity associated with genetically inherited forms of dementia and motor neuron disease has been identified by scientists at the UCL Institute of Neurology. The toxin is the result of a genetic mutation that leads to the production of RNA molecules which could be responsible for the diseases. The findings are published in the journal Acta Neuropathologica.. Frontotemporal dementia and motor neuron disease are related neurodegenerative diseases that affect approximately 15,000 people in the UK. Frontotemporal dementia causes profound personality and behaviour changes. Motor neuron disease leads to muscle weakness and eventual paralysis.. The most common known cause for both frontotemporal dementia and motor neuron disease is an unusual genetic mutation in the C9orf72 gene. The mutation involves a small string of DNA letters at the beginning of the gene, which expand massively to produce thousands of copies.. The new research, funded by Alzheimers Research UK and the Medical Research ...
Elevated titers of antibodies directed at ganglioside epitopes have been associated with multifocal motor neuropathy (MMN), motor variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), amyotrophic lateral sclerosis (ALS), and other motor neuropathies.Anti-GM1 antibodies were measured in 675 patients: 180 age- and sex-stratified healthy blood bank controls, 182 normal controls who had full neurologic assessment including electromyography, 121 patients with definite ALS, 19 patients with pure sensory neuropathy, and 173 consecutive patient serum samples submitted for GM1 antibody testing. Antibodies to three ganglioside epitopes were determined by ELISA: IgM and IgG anti-monosialo GM1, asialo GM1, and disialo GD1b. Antibody titers for normal subjects and patients with ALS were used to determine normal values and borderline levels below which 99% of normal and 99% of ALS patient titers were found. Clinical evaluation of the next 173 consecutive patients referred for anti-GM1 ...
Mortality rates from motor neuron disease in England and Wales for the years 1959-1979 were studied through death certification data supplied by the Office of Population Censuses and Surveys. The age- and sex-adjusted mortality rate increased over the period from 1.2 per 100 000 per year in 1959-61 to 1.6 in 1977-79, the increase being most apparent in women over age 45 years and men over 60 years. The ratio of the sex-specific mortality rates remained fairly constant at 1.6:1 (male to female). The distribution of motor neuron disease deaths within England and Wales showed more variation between counties and between Hospital Regions than expected, and areas of high motor neuron disease mortality along the south coast and low mortality in the Midlands could be identified. The variation was most marked in those aged over 65 years at death. Examination of occupation, as listed on the death certificates, showed an excess of motor neuron disease deaths in leather workers in all three periods for which data
Pestronk et al. [1] identified a group of patients with a pure motor neuropathy (PMN) that had clinical features similar to motor neuron disease. The syndrome was associated with conduction block and high-titer anti-GM1 antibodies and responded to immunosuppressive therapy. Since then, antibodies directed at the carbohydrate epitopes of complex glycosphingolipids (gangliosides) have been linked to various predominantly peripheral nervous system disorders. [2-4] However, the role of anti-GM1 antibodies in the etiology and pathogenesis of motor neuropathies, such as multifocal motor neuropathy (MMN), motor predominant chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and amyotrophic lateral sclerosis (ALS), has been controversial. [2,3,5,6] Low-affinity antibodies to various gangliosides, including GM1, have been found in serum from normal subjects in up to 100% of samples analyzed. [7] In addition, low-titer anti-GM1 antibodies have also been found in a significant number of ...

Toxins | Free Full-Text | The Cyanobacteria Derived Toxin Beta-N-Methylamino-L-Alanine and Amyotrophic Lateral SclerosisToxins | Free Full-Text | The Cyanobacteria Derived Toxin Beta-N-Methylamino-L-Alanine and Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis). The non-protein amino acid beta-N-methylamino-L-alanine (BMAA) was first associated with the ... high incidence of Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex (ALS/PDC) in Guam, and has been implicated as a ... Amyotrophic lateral sclerosis; Alzheimers disease; cyanobacteria; Beta-N-methylamino-L-alanine; BMAA; epidemiology Amyotrophic ... The Cyanobacteria Derived Toxin Beta-N-Methylamino-L-Alanine and Amyotrophic Lateral Sclerosis. Sandra A. Banack 1,* , Tracie A ...
more infohttp://www.mdpi.com/2072-6651/2/12/2837/xml

Abstract: Biochemical and ultrastructural study of neurofibrillary tangles in amyotrophic lateral sclerosis/parkinsonism...Abstract: Biochemical and ultrastructural study of neurofibrillary tangles in amyotrophic lateral sclerosis/parkinsonism...

Amyotrophic lateral sclerosis/parkinsonism-dementia complex of the Kii peninsula (Kii ALS/PDC) is a neurodegenerative disorder ... Amyotrophic lateral sclerosis/parkinsonism-dementia complex of the Kii peninsula (Kii ALS/PDC) is a neurodegenerative disorder ... Abstract: Biochemical and ultrastructural study of neurofibrillary tangles in amyotrophic lateral sclerosis/parkinsonism- ...
more infohttp://www.prohealth.com/library/showarticle.cfm?libid=5476

Involuntary Laughing, Crying Disorder Pseudobulbar Affect - ABC NewsInvoluntary Laughing, Crying Disorder Pseudobulbar Affect - ABC News

... amyotrophic lateral sclerosis (Lou Gehrigs disease), Parkinsons disease, brain tumors, and traumatic brain injury. ... When his doctor diagnosed multiple sclerosis in 1998, he made adjustments. When the MS left him blind in his left eye in 2000, ...
more infohttp://abcnews.go.com/Health/DepressionTreatment/involuntary-laughing-crying-disorder/story?id=12028237&page=2

Pseudobulbar palsyPseudobulbar palsy

Neurodegenerative diseases: amyotrophic lateral sclerosis, syringobulbia, multiple sclerosis *Autoimmune neuropathies: Guillain ... Neurodegenerative disease: amyotrophic lateral sclerosis, progressive supranuclear palsy, multiple sclerosis *Injury or ... professional/neurologic-disorders/peripheral-nervous-system-and-motor-unit-disorders/amyotrophic-lateral-sclerosis-als-and- ... Rubin M. Amyotrophic Lateral Sclerosis (ALS) and Other Motor Neuron Diseases (MNDs). http://www.msdmanuals.com/ ...
more infohttps://www.amboss.com/us/knowledge/Bulbar_palsy_and_pseudobulbar_palsy

Laughter is no joke - Can laughter kill you? Pathological Laughter - Dr 
Sampurna Roy MDLaughter is no joke - Can laughter kill you? Pathological Laughter - Dr Sampurna Roy MD

Other causes of pathological laughter are multiple sclerosis and amyotrophic lateral sclerosis or motor neuron disease. ...
more infohttp://www.pathopedia-india.com/pathological_laughter.htm

Benign Fasciculation Syndrome from Fluoroquinolone Antibiotics? - Fluoroquinolone Toxicity ResearchBenign Fasciculation Syndrome from Fluoroquinolone Antibiotics? - Fluoroquinolone Toxicity Research

Characteristics of fasciculations in amyotrophic lateral sclerosis and the fasciculation syndrome ↩. * Neurologic side effects ...
more infohttps://fqresearch.org/benign-fasciculation-syndrome?mode=grid

Amyotrophic lateral sclerosis - WikipediaAmyotrophic lateral sclerosis - Wikipedia

Revised criteria for the diagnosis of amyotrophic lateral sclerosis". Amyotrophic Lateral Sclerosis and Other Motor Neuron ... "Edaravone and its clinical development for amyotrophic lateral sclerosis". Amyotrophic Lateral Sclerosis. 18 (sup 1): 5-10. doi ... Amyotrophic Lateral Sclerosis. CRC Press. p. 9. ISBN 978-0824729240. .. *^ Gordon PH (October 2013). "Amyotrophic Lateral ... Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis)". Neurologic ...
more infohttps://en.wikipedia.org/wiki/Lou_Gehrig%27s_disease

Amyotrophic Lateral Sclerosis | SpringerLinkAmyotrophic Lateral Sclerosis | SpringerLink

Psychological and Research Aspects of Amyotrophic Lateral Sclerosis was held in Varese. Italy from the 27th to the 31st March ... Psychological and Research Aspects of Amyotrophic Lateral Sclerosis" was held in Varese. Italy from the 27th to the 31st March ... Immunological Assessment in Sporadic Amyotrophic Lateral Sclerosis (ALS) L. Provinciali, A. R. Giovagnoli, S. Battucci, C. ... Therapeutic Trial of Intrathecal Thyrotropin-Releasing Hormone (TRH) and a TRH-Analogue in Amyotrophic Lateral Sclerosis (ALS) ...
more infohttps://link.springer.com/book/10.1007/978-1-4684-5302-7

CDC - Amyotrophic Lateral Sclerosis: FAQCDC - Amyotrophic Lateral Sclerosis: FAQ

Can persons diagnosed with Primary Lateral Sclerosis (PLS) join the National ALS Registry?. Only people diagnosed with ALS can ... Most persons who are first diagnosed with progressive muscular atrophy, progressive bulbar palsy, or primary lateral sclerosis ... Other MNDs include progressive muscular atrophy, progressive bulbar palsy, and primary lateral sclerosis. ...
more infohttps://www.cdc.gov/als/ALSFAQ.html

CDC - Amyotrophic Lateral Sclerosis: ALSRegistryActCDC - Amyotrophic Lateral Sclerosis: ALSRegistryAct

Amyotrophic Lateral Sclerosis Registry.. Be it enacted by the Senate and House of Representatives of the United States of ... A) develop a system to collect data on amyotrophic lateral sclerosis (referred to in this section as `ALS) and other motor ...
more infohttps://www.cdc.gov/als/ALSRegistryActPublicLaw.html

CDC - Amyotrophic Lateral Sclerosis: LoginCDC - Amyotrophic Lateral Sclerosis: Login

This warning banner provides privacy and security notices consistent with applicable federal laws, directives, and other federal guidance for accessing this Government system, which includes (1) this computer network, (2) all computers connected to this network, and (3) all devices and storage media attached to this network or to a computer on this network. •This system is provided for Government-authorized use only. •Unauthorized or improper use of this system is prohibited and may result in disciplinary action and/or civil and criminal penalties. •Personal use of social media and networking sites on this system is limited as to not interfere with official work duties and is subject to monitoring. •By using this system, you understand and consent to the following: ◦The Government may monitor, record, and audit your system usage, including usage of personal devices and email systems for official duties or to conduct HHS business. Therefore, you have no reasonable expectation of privacy ...
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Amyotrophic Lateral Sclerosis | Encyclopedia.comAmyotrophic Lateral Sclerosis | Encyclopedia.com

Amyotrophic lateral sclerosis Definition Amyotrophic lateral sclerosis (ALS) is a disease that breaks down tissues in the ... AMYOTROPHIC LATERAL SCLEROSIS. DEFINITION. Amyotrophic lateral sclerosis (ALS; pronounced ay-MY-eh-TRO-fik LA-ter-el skler-OH- ... Amyotrophic lateral sclerosis. Definition. Amyotrophic lateral sclerosis (ALS) is a disease that breaks down tissues in the ... Amyotrophic Lateral Sclerosis. Definition. Amyotrophic lateral sclerosis (ALS) is a disease that breaks down tissues in the ...
more infohttps://www.encyclopedia.com/medicine/diseases-and-conditions/pathology/amyotrophic-lateral-sclerosis

Amyotrophic Lateral Sclerosis ALSAmyotrophic Lateral Sclerosis ALS

Amyotrophic lateral sclerosis (ALS):SOD1 mouse helps researchers identify immune component * Amyotrophic Lateral Sclerosis ALS ... Amyotrophic Lateral Sclerosis ALS, Bioinformatics, Computational Biology, Genetics and Genomics, Resource Development and ...
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CDC - Amyotrophic Lateral Sclerosis: Account NotificationCDC - Amyotrophic Lateral Sclerosis: Account Notification

This warning banner provides privacy and security notices consistent with applicable federal laws, directives, and other federal guidance for accessing this Government system, which includes (1) this computer network, (2) all computers connected to this network, and (3) all devices and storage media attached to this network or to a computer on this network. •This system is provided for Government-authorized use only. •Unauthorized or improper use of this system is prohibited and may result in disciplinary action and/or civil and criminal penalties. •Personal use of social media and networking sites on this system is limited as to not interfere with official work duties and is subject to monitoring. •By using this system, you understand and consent to the following: ◦The Government may monitor, record, and audit your system usage, including usage of personal devices and email systems for official duties or to conduct HHS business. Therefore, you have no reasonable expectation of privacy ...
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RhymeZone: amyotrophic lateral sclerosis definitionsRhymeZone: amyotrophic lateral sclerosis definitions

Definitions of amyotrophic lateral sclerosis: *noun: thickening of tissue in the motor tracts of the lateral columns and ... Search for amyotrophic lateral sclerosis at other dictionaries: OneLook, Oxford, American Heritage, Merriam-Webster, Wikipedia ...
more infohttp://www.rhymezone.com/r/d=amyotrophic_lateral_sclerosis

Redox Regulation in Amyotrophic Lateral SclerosisRedox Regulation in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the death of upper and lower motor neurons ... "A systematic review of antioxidant treatment for amyotrophic lateral sclerosis/motor neuron disease," Amyotrophic Lateral ... "Protein disulfide isomerase-immunopositive inclusions in patients with amyotrophic lateral sclerosis," Amyotrophic Lateral ... Redox Regulation in Amyotrophic Lateral Sclerosis. Sonam Parakh. ,1 Damian M. Spencer. ,1 Mark A. Halloran. ,2 Kai Y. Soo. ,1 ...
more infohttps://www.hindawi.com/journals/omcl/2013/408681/

Redox Regulation in Amyotrophic Lateral SclerosisRedox Regulation in Amyotrophic Lateral Sclerosis

... Sonam Parakh,1 Damian M. Spencer,1 Mark A. Halloran,2 Kai Y. Soo,1 and Julie ... Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the death of upper and lower motor neurons ...
more infohttps://www.hindawi.com/journals/omcl/2013/408681/abs/

ATSDR - Amyotrophic Lateral Sclerosis Training ModuleATSDR - Amyotrophic Lateral Sclerosis Training Module

Please visit the ATSDR Amyotrophic Lateral Sclerosis (ALS) Registry Online Training Module CE registration page ... cdc.gov and mention you need assistance with the ATSDR Amyotrophic Lateral Sclerosis (ALS) Registry Online Training Module. ... Amyotrophic Lateral Sclerosis (ALS) Continuing Education Module. ...
more infohttps://www.atsdr.cdc.gov/emes/als/index.html

Amyotrophic lateral sclerosis definition | Drugs.comAmyotrophic lateral sclerosis definition | Drugs.com

Definition of amyotrophic lateral sclerosis. Provided by Stedmans medical dictionary and Drugs.com. Includes medical terms and ... amyotrophic lateral sclerosis. Definition: a fatal degenerative disease involving the corticobulbar, corticospinal, and spinal ... primary lateral sclerosis, in which only upper motor neuron abnormalities are seen; and progressive spinal muscle atrophy, in ...
more infohttps://www.drugs.com/dict/amyotrophic-lateral-sclerosis.html

Amyotrophic lateral sclerosis - WikipediaAmyotrophic lateral sclerosis - Wikipedia

... including amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, progressive muscular atrophy, progressive bulbar ... "Amyotrophic lateral sclerosis". Lancet. 377 (9769): 942-55. doi:10.1016/s0140-6736(10)61156-7. PMID 21296405. "Amyotrophic ... Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) and Lou Gehrigs disease, is a specific disease ... Eisen, A. (2002). "Amyotrophic lateral sclerosis: A review". BCMJ. 44 (7): 362-366. Archived from the original on 21 June 2013 ...
more infohttps://en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis

Amyotrophic lateral sclerosis (ALS): MedlinePlus Medical EncyclopediaAmyotrophic lateral sclerosis (ALS): MedlinePlus Medical Encyclopedia

Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain, brain stem and spinal cord that control ... Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain, brain stem and spinal cord that control ... Amyotrophic lateral sclerosis and other motor neuron diseases. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed ... Amyotrophic lateral sclerosis. Lancet. 2017;390(10107):2084-2098. PMID: 28552366 www.ncbi.nlm.nih.gov/pubmed/28552366. ...
more infohttps://medlineplus.gov/ency/article/000688.htm

The Treatment of Amyotrophic Lateral Sclerosis | SpringerLinkThe Treatment of Amyotrophic Lateral Sclerosis | SpringerLink

Many physicians make no effort at treatment for patients suffering from amyotrophic lateral sclerosis (ALS). This is hard to ... Amyotrophic Lateral Sclerosis Methyl Phenidate Amyotrophic Lateral Sclerosis Patient Motor Neuron Disease Calcium Gluconate ... The Treatment of Amyotrophic Lateral Sclerosis. In: Cosi V., Kato A.C., Parlette W., Pinelli P., Poloni M. (eds) Amyotrophic ... F. H. Norris, K. S. U, E. H. Denys, K. C. Archibald, and C. Lebo, Amyotrophic lateral sclerosis (letter), Mayo Clin.Proc., 53: ...
more infohttps://link.springer.com/chapter/10.1007/978-1-4684-5302-7_29

Amyotrophic Lateral Sclerosis Definition & OverviewAmyotrophic Lateral Sclerosis Definition & Overview

ALS or Amyotrophic Lateral Sclerosis is a progressive nervous system disorder. Gradually, it destroys the nerves in the brain ... See the latest posts about Amyotrophic Lateral Sclerosis Definition & Overview in womens health ... The Latest in Amyotrophic Lateral Sclerosis Definition & Overview. * ALS Ice Bucket Challenge is Increasing Awareness and ... This Amyotrophic Lateral Sclerosis Definition & Overview page on EmpowHER Womens Health works best with javascript enabled in ...
more infohttps://www.empowher.com/condition/amyotrophic-lateral-sclerosis/definition

Discovery aids understanding of amyotrophic lateral sclerosisDiscovery aids understanding of amyotrophic lateral sclerosis

Researchers have discovered a basic molecular mechanism that sheds light on the disease process underlying amyotrophic lateral ... has discovered a basic molecular mechanism that sheds light on the disease process underlying amyotrophic lateral sclerosis ( ... Tags: AIDS, Brain, Cell, Eye, Light, Metabolism, Nucleus, Paralysis, Pathology, Protein, Research, RNA, Sclerosis, Splicing, ... Multiple sclerosis therapies delay progression of disability. *Hearing challenges still reported by high proportion of older ...
more infohttps://www.news-medical.net/news/20180319/Discovery-aids-understanding-of-amyotrophic-lateral-sclerosis.aspx

Amyotrophic Lateral Sclerosis (ALS) Efficacy StudiesAmyotrophic Lateral Sclerosis (ALS) Efficacy Studies

Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrigs disease is a devastating and rapidly fatal disease with currently only one ... Amyotrophic Lateral Sclerosis (ALS) Efficacy Studies. Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrigs disease is a ... Guide: Working with Amyotrophic Lateral Sclerosis (ALS) Mouse Models. This guide summarizes the guidelines for preclinical ...
more infohttps://www.jax.org/jax-mice-and-services/in-vivo-pharmacology/neurobiology-services/amyotrophic-lateral-sclerosis-efficacy-studies
  • Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), or ALS, is a progressive nervous system (neurological) disease that destroys nerve cells and causes disability. (mayoclinic.org)
  • THURSDAY, Feb. 28, 2019 - Professional soccer players may be vulnerable to amyotrophic lateral sclerosis (ALS), a new study suggests. (drugs.com)
  • The report includes a compilation of current active projects in research and development of small molecules, antibodies, proteins, peptides, vaccines and other biologics including cells for treatment of amyotrophic lateral sclerosis (ALS). (slideshare.net)
  • The purpose of this guidance is to assist sponsors in the clinical development of drugs for the treatment of amyotrophic lateral sclerosis (ALS). (fda.gov)
  • A listing of Amyotrophic Lateral Sclerosis (ALS) medical research trials actively recruiting patient volunteers. (centerwatch.com)
  • Research on amyotrophic lateral sclerosis has focused on animal models of the disease, its mechanisms, ways to diagnose and track it, and treatments. (wikipedia.org)
  • As this area degenerates it leads to scarring or hardening ("sclerosis") in the region. (psp.org)