Amyotrophic Lateral Sclerosis. ALS (Amyotrophic Lateral Sclerosis) . Amyotrophic Lateral Sclerosis With Dementia . Amyotrophic Lateral Sclerosis, Guam Form . Amyotrophic Lateral Sclerosis, Parkinsonism-Dementia Complex of Guam . Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex 1 . Charcot Disease . Dementia With Amyotrophic Lateral Sclerosis . Gehrig's Disease . Guam Disease . Guam Form of Amyotrophic Lateral Sclerosis . Lou Gehrig's Disease . Lou-Gehrigs Disease . Amyotrophic Lateral Sclerosis Parkinsonism Dementia Complex 1 . Amyotrophic Lateral Sclerosis, Parkinsonism Dementia Complex of Guam . Disease, Guam . Disease, Lou-Gehrigs . Gehrig Disease . Gehrigs Disease . Sclerosis, Amyotrophic Lateral . Lou Gehrig Disease . Motor Neuron Disease, Amyotrophic Lateral Sclerosis . GEHRIG'S DISEASE . LOU GEHRIG'S 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) . 1.00
Motor Neuron Disease. Lower Motor Neuron Disease . Motor Neuron Disease, Familial . Motor Neuron Disease, Secondary . Motor System Disease . Primary Lateral Sclerosis . Secondary Motor Neuron Disease . Upper Motor Neuron Disease . Lateral Scleroses . Lateral Scleroses, Primary . Lateral Sclerosis, Primary . Motor Neuron Diseases . Motor System Diseases . Neuron Disease, Motor . Neuron Diseases, Motor . Primary Lateral Scleroses . Scleroses, Lateral . Scleroses, Primary Lateral . Sclerosis, Lateral . Sclerosis, Primary Lateral . Lateral Sclerosis . Anterior Horn Cell Disease . Familial Motor Neuron Disease . Motor Neuron Disease, Lower . Motor Neuron Disease, Upper . 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) . 0.71
