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Does neuroinflammation sustain neurodegeneration in ALS?

ABSTRACT

Amyotrophic lateral sclerosis (ALS), the most prevalent type of motor neuron disease in adults, affecting 4–6 per 100,000, is a fatal neurodegenerative disease. ALS is characterized by the degeneration of both upper motor neurons comprising the corticospinal tract and lower motor neurons arising from the brainstem nuclei and ventral roots of the spinal cord. The only drug approved for ALS—riluzole—provides a modest survival benefit. An improved understanding of the pathophysiology of ALS has potential for the development of more effective therapeutic interventions.