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
Poliomyelitis, Bulbar. Bulbar Polio . Poliomyelitis, Medullary Involvement . Medullary Involvement Poliomyelitis . Polio, Bulbar . Bulbar Poliomyelitis . A form of paralytic poliomyelitis affecting neurons of the MEDULLA OBLONGATA of the brain stem. Clinical features include impaired respiration, HYPERTENSION, alterations of vasomotor control, and dysphagia. Weakness and atrophy of the limbs and trunk due to spinal cord involvement is usually associated. (From Adams et al., Principles of Neurology, 6th ed, p765) . 0.55
Muscular Atrophy, Spinal. Adult Spinal Muscular Atrophy . Adult-Onset Spinal Muscular Atrophy . Amyotrophy, Neurogenic Scapuloperoneal, New England Type . Distal Spinal Muscular Atrophy . Hereditary Motor Neuronopathy . Muscular Atrophy, Adult Spinal . Myelopathic Muscular Atrophy . Myelopathic Muscular Atrophy, Progressive . Progressive Myelopathic Muscular Atrophy . Progressive Proximal Myelopathic Muscular Atrophy . Proximal Myelopathic Muscular Atrophy, Progressive . Scapuloperoneal Spinal Muscular Atrophy . Spinal Amyotrophy . Spinal Muscular Atrophy, Distal . Spinal Muscular Atrophy, Oculopharyngeal . Spinal Muscular Atrophy, Scapuloperoneal . Spinal Muscular Atrophy, Scapuloperoneal Form . Adult Onset Spinal Muscular Atrophy . Amyotrophies, Spinal . Amyotrophy, Spinal . Atrophies, Progressive Muscular . Atrophy, Myelopathic Muscular . Atrophy, Progressive Muscular . Atrophy, Spinal Muscular . Bulbospinal Neuronopathies . Hereditary Motor Neuronopathies . Motor Neuronopathies, Hereditary . Motor Neuronopathy, Hereditary . Muscular Atrophies, Progressive . Muscular Atrophy, Myelopathic . Muscular Atrophy, Progressive . Neuronopathies, Bulbospinal . Neuronopathies, Hereditary Motor . Neuronopathy, Bulbospinal . Neuronopathy, Hereditary Motor . Progressive Muscular Atrophies . Spinal Amyotrophies . Progressive Muscular Atrophy . Spinal Muscular Atrophy . Bulbospinal Neuronopathy . Oculopharyngeal Spinal Muscular Atrophy . Scapuloperoneal Form of Spinal Muscular Atrophy . MYELOPATHIC MUSCULAR ATROPHY . 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) . 0.47
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) . 0.47
Kuru 5929. Encephalopathy, Kuru . Kuru Encephalopathy . A prion disease found exclusively among the Fore linguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773) . 0.42
Anterior Spinal Artery Syndrome. Dissection, Anterior Spinal Artery . Syndrome, Anterior Spinal Artery . Anterior Spinal Artery Dissection . Ischemia or infarction of the spinal cord in the distribution of the anterior spinal artery, which supplies the ventral two-thirds of the spinal cord. This condition is usually associated with ATHEROSCLEROSIS of the aorta and may result from dissection of an AORTIC ANEURYSM or rarely dissection of the anterior spinal artery. Clinical features include weakness and loss of pain and temperature sensation below the level of injury, with relative sparing of position and vibratory sensation. (From Adams et al., Principles of Neurology, 6th ed, pp1249-50) . 0.41
Pick Disease of the Brain. Brain Atrophy, Circumscribed Lobar . Dementia with Lobar Atrophy and Neuronal Cytoplasmic Inclusions . Lobar Atrophy Of Brain . Pick Disease . Pick Disease Of Brain . Pick's Disease . Picks Disease of Brain . Atrophies, Lobar (Brain) . Atrophy, Lobar (Brain) . Disease, Pick . Disease, Pick's . Lobar Atrophies (Brain) . Picks Disease . Circumscribed Lobar Atrophy of the Brain . Lobar Atrophy (Brain) . Lobar Atrophy of the Brain . PICKS DISEASE OF BRAIN . A rare form of DEMENTIA that is sometimes familial. Clinical features include APHASIA; APRAXIA; CONFUSION; ANOMIA; memory loss; and personality deterioration. This pattern is consistent with the pathologic findings of circumscribed atrophy of the poles of the FRONTAL LOBE and TEMPORAL LOBE. Neuronal loss is maximal in the HIPPOCAMPUS, entorhinal cortex, and AMYGDALA. Some ballooned cortical neurons contain argentophylic (Pick) bodies. (From Brain Pathol 1998 Apr;8(2):339-54; Adams et al., Principles of Neurology, 6th ed, pp1057-9) . 0.40
