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)

  • $1s in the profilin 1 gene cause familial amyotrophic lateral $1" Nature , published online July 15, 2012. (medicalxpress.com)
  • Amyotrophic lateral $1 (ALS) is a genetically heterogeneous disorder that shows a characteristic dichotomy of familial forms typically displaying Mendelian inheritance patterns, and sporadic ALS showing no or less obvious familial aggregation. (mpg.de)
  • Recently, rare $1s in the TARDBP gene have been identified in familial and sporadic amyotrophic lateral $1 (ALS) patients. (unica.it)
  • Background: Recent data provide support for the concept that potentially modifiable exposures are responsible for sporadic amyotrophic lateral $1 (ALS). (elsevier.com)
  • Amyotrophic lateral $1 (ALS) is a disease that breaks down tissues in the nervous system (a neurodegenerative disease) of unknown cause that affects the nerves responsible for movement. (encyclopedia.com)
  • Amyotrophic lateral $1 (ALS) is a fatal neurodegenerative disease of the motor nervous system. (broadinstitute.org)
  • abstract = "AIMS: Amyotrophic lateral $1 (ALS) is a chronic neurodegenerative disease characterised by progressive loss of $1, $1, spasticity, $1 and death usually within 2-5 years of onset. (rug.nl)
  • Primary lateral $1: a rare upper-motor-predominant form of amyotrophic lateral $1 often accompanied by frontotemporal lobar degeneration with $1ated neuronal inclusions? (eurekamag.com)
  • Finally, a survey of the literature based on this patient's clinical and pathological features led us to conclude that the rare clinical syndrome of primary lateral $1 is, in general, a rare upper-motor-predominant form of amyotrophic lateral $1 that is often accompanied by frontotemporal lobar degeneration with $1ated neuronal inclusions. (eurekamag.com)
  • PATIENTS: The study involved 21 patients with amyotrophic lateral $1 and 3 patients with primary lateral $1. (ox.ac.uk)
  • Amyotrophic Lateral $1 Pipeline and Therapeutics Development H1 2017 provides comprehensive information on the therapeutics under development for Amyotrophic Lateral $1, complete with analysis by stage of development, drug target, mechanism of action (MoA), route of administration (RoA) and molecule type. (whatech.com)
  • latest pipeline guide Amyotrophic Lateral $1 - Pipeline Review, H2 2017, provides comprehensive information on the therapeutics under development for Amyotrophic Lateral $1 ($1), complete with analysis by stage of development, drug target, mechanism of action (MoA), route of administration (RoA) and molecule type. (whatech.com)
  • The study was designed to assess the safety, tolerability and efficacy of glatiramer acetate (GA) 40 mg, given once daily as a subcutaneous injection, in reducing disease-related functional deterioration in Amyotrophic Lateral $1 (ALS) patients. (fiercebiotech.com)
  • A group of 25 patients with amyotrophic lateral $1 was compared to healthy control subjects using a multi-modal $1 approach comprising T(1)-weighted, diffusion-weighted and resting-state functional $1. (ox.ac.uk)
  • Objectives - To determine the causes and place of death in a cohort of Italian patients with amyotrophic lateral $1 (ALS). (unipa.it)
  • Researchers have linked newly discovered gene $1s to some cases of the progressive fatal neurological disease amyotrophic lateral $1 ALS, also known as Lou Gehrig's disease. (medicalxpress.com)
  • Dominant $1s in $1 1 (SOD1) cause degeneration of $1 in a subset of inherited amyotrophic lateral $1 (ALS). (biomedcentral.com)
  • Recently, TDP-43 was identified as a key component of $1ated aggregates in amyotrophic lateral $1 (ALS), an adult-onset neurological disorder that leads to the degeneration of $1. (nih.gov)
  • Amyotrophic lateral $1/ALS/ Lou Gehrig's disease belongs to a group of disorders known as $1s, which are characterized by the gradual degeneration and death of $1. (integratedhc.com)
  • Amyotrophic lateral $1, or ALS, is a disease of the nerve cells in the $1, $1 stem and spinal cord that control voluntary muscle movement. (mountsinai.org)
  • Amyotrophic Lateral $1 (ALS) is a nervous system disease that rapidly and progressively destroys motor nerve cells in the $1 and spinal cord. (rxwiki.com)
  • Amyotrophic lateral $1, or ALS, is a disease in which certain nerve cells in the $1 and spinal cord slowly die. (genesishcs.org)
  • One alternative possibility is that increased functional connectivity reflects a progressive loss of inhibitory cortical influence as part of amyotrophic lateral $1 pathogenesis, which might then have relevance to future therapeutic strategies. (ox.ac.uk)
  • The pipeline guide provides a snapshot of the global therapeutic landscape of Amyotrophic Lateral $1. (whatech.com)
  • The Amyotrophic Lateral $1 ($1) pipeline guide also reviews of key players involved in therapeutic development for Amyotrophic Lateral $1 and features dormant and discontinued projects. (whatech.com)
  • OBJECTIVE: To explore the value of diffusion tensor imaging applied to those specific cerebral white matter tracts consistently involved pathologically in amyotrophic lateral $1 as a source of prognostic biomarkers. (ox.ac.uk)
  • Amyotrophic lateral $1 (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). (physio-pedia.com)
  • Amyotrophic lateral $1 as a system failure is a concept supported by the finding of consistent extramotor as well as motor cerebral pathology. (ox.ac.uk)
  • CONCLUSIONS: Posterior limb of the internal capsule fractional anisotropy is a candidate prognostic marker in amyotrophic lateral $1, with potential to identify incident cases with more rapid progression. (ox.ac.uk)
  • SDD cases had higher numbers of $1 and $1l activation than MDD cases, but similar levels of $1 with pTDP-43+ inclusions.CONCLUSIONS: Increased expression of several HSPBs in lateral column $1 suggests that $1 play a role in the pathogenesis of ALS. (rug.nl)
  • Lateral $1 refers to the changes seen in the lateral columns of the spinal cord as upper motor neuron (UMN) $1 in these areas degenerate and are replaced by fibrous $1 ($1). (whatech.com)
  • Amyotrophic lateral $1 ( ALS ) is the most common type of adult-onset $1 (MND). (medscape.com)
  • Integration of structural and functional $1 in amyotrophic lateral $1. (ox.ac.uk)
  • Intrinsic membrane hyperexcitability of amyotrophic lateral $1 patient-derived $1. (broadinstitute.org)
  • A few lower $1 were found to contain cytoplasmic $1 characteristic of amyotrophic lateral $1 (i.e. (eurekamag.com)
  • Amyotrophic lateral $1 (ALS) is a devastating neurological disease with no effective treatment. (unimi.it)
  • Glatiramer acetate is indicated for the reduction of the frequency of relapses in relapsing-remitting multiple $1 (RRMS). (fiercebiotech.com)
  • Dr. Rene Günther, one of the lead authors of the study and head of the research group and specialist outpatient clinic for $1s at the Department of Neurology of the Carl Gustav Carus University Hospital at the Technical University of Dresden, explains: 'Biomarker research plays a crucial role in improving early detection and securing the diagnosis of amyotrophic lateral $1. (news-medical.net)
  • 4,5,7 He stated, "The diagnosis as well as the anatomy and physiology of the condition amyotrophic lateral $1 is one of the most completely understood conditions in the realm of clinical neurology. (ajmc.com)
  • El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral $1. (freemd.com)
  • Prize4Life, a non-profit organization dedicated to accelerating the discovery of a cure for Amyotrophic Lateral $1 (ALS) by offering incentives to drive innovation, today announced that Dr. Seward Rutkove, Chief of the Division of Neuromuscular Disease at Beth Israel Deaconess Medical Center and Associate Professor of Neurology at Harvard Medical School, has received the $1 million dollar Prize4Life award for the discovery of a new ALS biomarker. (highlighthealth.com)
  • How common are behavioural changes in amyotrophic lateral $1? (biomedsearch.com)
  • Dietary intake and function in amyotrophic lateral $1: Are they associated? (eurekalert.org)
  • ALS is a $1, also spelled "motor neurone disease", which is a group of neurological disorders that selectively affect $1, the cells that control voluntary muscles of the body, including amyotrophic lateral $1 (ALS), primary lateral $1, progressive $1, progressive bulbar palsy, $1, and spinal $1. (wikipedia.org)
  • Amyotrophic lateral $1 is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles. (medicinenet.com)
  • Amyotrophic lateral $1 (ALS) is a rare group of neurological diseases that mainly involve the nerve cells ($1) responsible for controlling voluntary muscle movement. (medicinenet.com)
  • This study will examine the effectiveness of Cistanche Total Glycosides(CTG) in treating patients with amyotrophic lateral $1 (ALS) - a fatal neurological degenerative disease that causes adult-onset, progressive $1 loss in the spinal cord, $1 stem and $1. (centerwatch.com)
  • A research team from the Centre for Protein Diagnostics (Prodi) at Ruhr University Bochum (RUB), in collaboration with scientists from Dresden Technical University, Essen University Hospital and University Hospital Göttingen, has developed a diagnostic tool for the rare neurological disease amyotrophic lateral $1 (ALS). (news-medical.net)
  • Amyotrophic lateral $1 (ALS) is a fatal neurological disorder characterized by progressive $1 and respiratory failure. (nih.gov)
  • (mayoclinic.org)
  • Amyotrophic lateral $1 (ALS) is difficult to diagnose early because it may mimic several other neurological diseases. (mayoclinic.org)
  • Amyotrophic lateral $1 (ALS), also known as Lou Gehrig's disease or $1, is a progressive neurological disease that causes the $1 that control voluntary muscles ($1) to degenerate, according to the National Institutes of Health (NIH). (livescience.com)
  • A Meta-Analysis of Observational Studies of the Association Between Chronic Occupational Exposure to Lead and Amyotrophic Lateral $1 " stems from the Canadian government's $15 million effort to better understand neurological health conditions. (scienceblogs.com)
  • Amyotrophic lateral $1 (ALS) is a terminal neurological disorder characterized by progressive degeneration of nerve cells in the spinal cord and $1. (massgeneral.org)
  • Recently, TDP-43 was identified as a key component of $1ated aggregates in amyotrophic lateral $1 (ALS), an adult-onset neurological disorder that leads to the degeneration of $1. (nih.gov)
  • A severe physical disability has a dramatic impact on a person's life, whether it is caused by a neuro-degenerative disease such as amyotrophic lateral $1 (ALS), a $1stem stroke, or a spinal cord injury. (centerwatch.com)
  • Find out more about the degenerative disease- Amyotrophic lateral $1. (medindia.net)
  • Introduction: Amyotrophic lateral $1 (ALS) and Parkinson's disease (PD) are two severe neurodegenerative disorders for which the disease mechanisms are poorly understood and reliable biomarkers are absent. (diva-portal.org)
  • Inflammatory markers in cerebrospinal fluid: independent prognostic biomarkers in amyotrophic lateral $1? (bmj.com)
  • Amyotrophic lateral $1 (ALS) and $1 (FTD) are fatal $1 that are related genetically and pathologically. (mendeley.com)
  • Amyotrophic Lateral $1 (ALS), the most common MND, is a fatal adult-onset neuromuscular disease. (clinicaltrials.gov)
  • Amyotrophic lateral $1 ( ALS ) is the most common type of adult-onset $1 (MND). (medscape.com)
  • SOD1 was considered a regional candidate gene because $1s in human SOD1 can cause amyotrophic lateral $1 (ALS), an adult-onset fatal paralytic neurodegenerative disease with both upper and lower motor neuron involvement. (pnas.org)
  • The purpose of this guidance is to assist sponsors in the clinical development of drugs for the treatment of amyotrophic lateral $1 (ALS). (fda.gov)
  • Amyotrophic lateral $1 (ALS) is a fatal neurodegenerative disease with substantial heterogeneity in its clinical presentation. (nature.com)
  • The DSMB was created as part of the Company's pivotal clinical study evaluating masitinib in the treatment of amyotrophic lateral $1. (cnbc.com)
  • Waragai M: MRI and clinical features in amyotrophic lateral $1. (freemd.com)
  • THURSDAY, Feb. 28, 2019 - Professional soccer players may be vulnerable to amyotrophic lateral $1 (ALS), a new study suggests. (drugs.com)
  • Research on amyotrophic lateral $1 has focused on animal models of the disease, its mechanisms, ways to diagnose and track it, and treatments. (wikipedia.org)
  • Amyotrophic lateral $1 (ALS) is a disease that breaks down tissues in the nervous system (a neurodegenerative disease) of unknown cause that affects the nerves responsible for movement. (encyclopedia.com)
  • Amyotrophic lateral $1 (ALS) is a progressive disease that affects $1, which are specialized nerve cells that control muscle movement. (medlineplus.gov)
  • Amyotrophic lateral $1 (ALS) is a neurodegenerative disease that results from the death of upper and lower $1. (hindawi.com)
  • What is amyotrophic lateral $1 (ALS) (Lou Gehrig disease)? (medscape.com)
  • A disease of the motor tracts of the lateral columns and anterior horns of the spinal cord, causing progressive $1, increased reflexes, fibrillary twitching, and spastic irritability of muscles. (dictionary.com)
  • Amyotrophic Lateral $1 (ALS) is a degenerative neuromuscular disease, progressing inexorably to respiratory failure, the by involvement of respiratory muscles, the commitment with most impact on the prognosis of ALS. (clinicaltrials.gov)
  • Amyotrophic lateral $1 (ALS) is a disease in which the $1 die in a progressive manner, leading to $1 and muscle wasting. (diva-portal.org)
  • Amyotrophic lateral $1 (ALS) is a disease that results in the progressive deterioration and loss of function of the $1 in the $1 and spinal cord, leading to $1. (ajmc.com)
  • 6 In 1874, Charcot named this disease, "amyotrophic lateral $1," which is still known today as Charcot disease in many parts of the world. (ajmc.com)
  • Amyotrophic lateral $1, or ALS, is a disease in which certain nerve cells in the $1 and spinal cord slowly die. (adventisthealthcare.com)
  • Amyotrophic lateral $1 (ALS) is a neurodegenerative disease resulting from the loss of upper $1 (UMN) and lower $1 (LMN). (cap.org)
  • Amyotrophic Lateral $1 (ALS) , is a progressive neurodegenerative disease that affects nerve cells in the $1 and the spinal cord. (psp.org)
  • To determine the magnetic resonance (MR) imaging characteristics of amyotrophic lateral $1 (ALS) and to evaluate possible correlations between the disease severity and the MR imaging findings. (nih.gov)
  • Amyotrophic lateral $1 (ALS) is a fatal and progressive neurodegenerative disease. (frontiersin.org)
  • Amyotrophic lateral $1 (ALS) is a fatal and progressive neurodegenerative disease, which affects $1 in the anterior horn of the spinal cord, the $1stem, and the $1. (frontiersin.org)
  • Brandeis researchers have made a significant advance in the effort to understand amyotrophic lateral $1 (ALS) by successfully reversing the toxicity of the mutated protein in the familial type of the disease. (innovations-report.com)
  • A unique industry-academia partnership will increase the rate at which promising drug compounds can be tested as potential treatments for amyotrophic lateral $1 (ALS), a disease with no known cure that affects 200,000 people worldwide. (mcgill.ca)
  • Amyotrophic lateral $1 (ALS) is a fatal neurodegenerative disease of the motor nervous system. (broadinstitute.org)
  • Amyotrophic lateral $1 (ALS) is a late-onset fatal neurodegenerative disease that causes progressive degeneration of $1 in the $1 and the spinal cord. (nature.com)
  • indeed, the disease is named in part for the pathological findings in the lateral CST. (nature.com)
  • Amyotrophic lateral $1, or ALS, is a disease of the nerve cells in the $1, $1 stem and spinal cord that control voluntary muscle movement. (mountsinai.org)
  • This higher incidence does not appear to be due to familial ALS or en environmental factor, such as an infective agent, but is thought to be due to higher susceptibility to the disease by the islands native population. (yale.edu)
  • $1s include amyotrophic lateral $1 (ALS), primary lateral $1 (PLS), progressive $1 (PMA), progressive bulbar palsy , $1 , and monomelic amyotrophy (MMA). (wikipedia.org)
  • Primary lateral $1 (PLS) involves only upper $1, and progressive $1 (PMA) involves only lower $1. (wikipedia.org)
  • Other MNDs include progressive $1, progressive bulbar palsy, and primary lateral $1. (cdc.gov)
  • The genetics and neuropathology of amyotrophic lateral $1. (cap.org)
  • Deciphering amyotrophic lateral $1: what $1, neuropathology and genetics are telling us about pathogenesis. (cap.org)
  • Amyotrophic Lateral $1 - Anyone know how fast bulbar onset ALS takes? (drugs.com)
  • Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral $1. (clevelandclinic.org)
  • Amyotrophic lateral $1 (ALS) is a fatal neurodegenerative disorder characterized by progressive weakness of the limb, bulbar, and respiratory muscles 1 . (nature.com)
  • Stage-specific $1 effect in amyotrophic lateral $1: a retrospective study. (clevelandclinic.org)
  • Amyotrophic lateral $1 is a rare degenerative disorder that results in progressive wasting and $1 of voluntary muscles. (cnbc.com)
  • Objective The aim of the study was to assess the utility of a novel amyotrophic lateral $1 (ALS) diagnostic index (ALSDI). (neurology.org)
  • The aim of this study is to determine whether serum uric acid levels predict survival in amyotrophic lateral $1 (ALS). (biomedsearch.com)