Amyotrophic Lateral Sclerosis Motor Neuron Disease Definition
Amyotrophic Lateral Sclerosis Motor Neuron Disease: 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)
Synonym: guam form amyotrophic lateral sclerosis, amyotrophic lateral sclerosis with dementia, amyotrophic lateral sclerosis, alss (amyotrophic lateral sclerosis), als (amyotrophic lateral sclerosis), lou gehrigs disease, lou gehrig disease, guam form of amyotrophic lateral sclerosis, gehrigs disease, gehrig disease, dementia with amyotrophic lateral sclerosis, amyotrophic lateral scleroses.