Amyotrophic lateral sclerosis overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Amyotrophic Lateral Sclerosis is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. As a motor neuron disease, the disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy due to that denervation. The patient may ultimately lose the ability to initiate and control all voluntary movement except of the eyes.

Cognitive function is generally spared except in certain situations such as when ALS is associated with frontotemporal dementia.[1] However, there are reports of more subtle cognitive changes of the frontotemporal type in many patients when detailed neuropsychological testing is employed. Sensory nerves and the autonomic nervous system, which controls functions like sweating, generally remain functional.

References

  1. Phukan J, Pender NP, Hardiman O (2007). "Cognitive impairment in amyotrophic lateral sclerosis". Lancet Neurol. 6 (11): 994–1003. doi:10.1016/S1474-4422(07)70265-X. PMID 17945153.

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