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amyotrophic lateral sclerosis

 

Definition: a fatal degenerative disease involving the corticobulbar, corticospinal, and spinal motor neurons, manifested by progressive weakness and wasting of muscles innervated by the affected neurons; fasciculations and cramps commonly occur. The disorder is 90–95% sporadic in nature (although a number of cases are inherited as an autosomal dominant trait [MIM*105400]), affects adults (typically, older adults), and usually is fatal within 2–5 years of onset. It is in the most common subgroup of motor neuron diseases, and the only one manifested by a combination of upper and lower abnormalities. Variants include: progressive bulbar palsy, in which isolated or predominant lower brainstem motor involvement occurs; primary lateral sclerosis, in which only upper motor neuron abnormalities are seen; and progressive spinal muscle atrophy, in which only lower motor neuron dysfunction is noted.

Synonym(s): Aran-Duchenne disease, Charcot disease, Duchenne-Aran disease, Lou Gehrig disease, motor neuron disease1, progressive muscular atrophy, progressive spinal amyotrophy

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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