: MeSH: Amyotrophic Lateral Sclerosis

Nervous System Diseases > Neurodegenerative Diseases > Motor Neuron Disease > Amyotrophic Lateral Sclerosis

Nervous System Diseases > Neuromuscular Diseases > Motor Neuron Disease > Amyotrophic Lateral Sclerosis

Nervous System Diseases > Central Nervous System Diseases > Spinal Cord Diseases > Amyotrophic Lateral Sclerosis

Nutritional and Metabolic Diseases > Metabolic Diseases > Proteostasis Deficiencies > TDP-43 Proteinopathies > Amyotrophic Lateral Sclerosis

Nervous System Diseases > Neurodegenerative Diseases > TDP-43 Proteinopathies > Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis

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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)

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