Motor Neuron Disease
| Definition: |
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 involvment 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) |
| Notes: |
GEN or unspecified; prefer specifics |
| Also Called: |
Anterior Horn Cell Disease,Familial Motor Neuron Disease,Lateral Sclerosis,Motor Neuron Disease, Lower,Motor Neuron Disease, Secondary,Motor Neuron Disease, Upper |
| Previously Indexed: |
Motor Neurons (1966-1991),Neuromuscular Diseases (1972-1991) |
Motor Neuron Disease Categories.
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Bulbar Palsy, Progressive - A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked intially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900) |
Motor Neuron Disease Definitions and Terms
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