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Amyotrophic lateral sclerosis

2023
The scientific landscape surrounding amyotrophic lateral sclerosis has shifted immensely with a number of well-defined ALS disease-causing genes, each with related phenotypical and cellular motor neuron processes that have come to light. Yet in spite of decades of research and clinical investigation, there is still no etiology for sporadic amyotrophic ...
David S, Younger, Robert H, Brown
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Amyotrophic lateral sclerosis

Current Opinion in Neurology, 1980
Therapeutic trials for amyotrophic lateral sclerosis have attracted much attention, but no drug tested has been effective yet. Three major theories of pathogenesis form the basis for these trials: autoimmunity, chronic excitotoxic stimulation due to accumulation of glutamate, and, in the familial form, peroxidation due to subnormal activity of ...
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Amyotrophic lateral sclerosis mimics

Muscle & Nerve, 2022
AbstractAmyotrophic lateral sclerosis (ALS) is the most common adult‐onset motor neuron disorder characterized by progressive degeneration of cortical, bulbar, and spinal motor neurons. When a patient presents with a progressive upper and/or lower motor syndrome, clinicians must pay particular attention to any atypical features in the history and/or ...
Justin, Kwan, Mithila, Vullaganti
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Amyotrophic lateral sclerosis

Current Treatment Options in Neurology, 2000
More than a century after its initial clinicopathologic description, amyotrophic lateral sclerosis (ALS) remains a largely fatal, progressive neurodegenerative disorder for which few efficacious pharmacotherapies with an impact directly on the natural course of the illness exist. The only currently approved therapy, the antiglutamatergic agent riluzole,
, Demaerschalk, , Strong
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Amyotrophic Lateral Sclerosis

Seminars in Neurology, 1998
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease with evidence of both anterior horn cell and corticospinal tract degeneration. The incidence of ALS is 1 to 2.5 cases per 100,000 population and the disease occurs primarily in adult life. The etiology of sporadic ALS remains unknown, although 5 to 10% of cases are familial. The diagnosis of
C E, Jackson, W W, Bryan
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Amyotrophic Lateral Sclerosis: Pathogenesis

Seminars in Neurology, 2001
Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by motor neuron degeneration. A subgroup of familial cases arises from mutations in the gene encoding cytosolic superoxide dismutase (SOD1). This review considers insight now being gained into ALS pathogenesis from the study of mutant SOD1 protein and its possible mechanisms
R H, Brown, W, Robberecht
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Hereditary amyotrophic lateral sclerosis

Journal of the Neurological Sciences, 1981
A Spanish family transmits, as an autosomal dominant trait, a form of amyotrophic lateral sclerosis characterized by an unusually prolonged evolution of the disease in all affected members. Precocity and persistence of muscle cramps, presence of unilateral proximal segmental myoclonus and early abolition of ankle jerks are other clinical features ...
R, Alberca   +2 more
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Hereditary amyotrophic lateral sclerosis

Journal of the Neurological Sciences, 1969
Abstract We report the clinical and histopathological findings in a patient affected by a hereditary form of amyotrophic lateral sclerosis (ALS). The paper includes a clinical description of 11 other members of the same family and comments upon genetic considerations in this family.
A F, Thomson, F A, Alvarez
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Amyotrophic Lateral Sclerosis: Review

Seminars in Neurology, 2015
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease primarily affecting the upper and lower motor neurons. The lifetime risk of developing ALS is estimated at 1:350 for men and 1:500 for women, higher for those who have served in the military. The diagnosis remains clinical with electrodiagnostic support.
Johnny S, Salameh   +2 more
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Amyotrophic Lateral Sclerosis Model

2018
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects upper and lower motor neurons in the brain and the spinal cord. Due to the progressive neurodegeneration, ALS leads to paralysis and death caused by respiratory failure 2-5 years after the onset of symptoms. There is no effective cure available.
Yumiko, Azuma   +3 more
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