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July 2016

Variability in Amyotrophic Lateral Sclerosis

Author Affiliations
  • 1Department of Neurology, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. 2016;73(7):781-782. doi:10.1001/jamaneurol.2016.0729

Amyotrophic lateral sclerosis (ALS) is a devastating fatal paralytic neurodegenerative disorder that is driven by complex and insufficiently understood pathophysiologic mechanisms. In the clinic, ALS is rarely difficult for the neurologist to recognize; yet considerable interpatient variability exists for what is considered to be a singular disorder. Variability manifests neuroanatomically, wherein the weakness may begin in nearly any voluntary muscle and subsequently migrate throughout the nervous system. Variability is also seen in the rate of disease progression; although the median survival in ALS is approximately 3 years after symptom onset, approximately 20% of patients with ALS live past 5 years, and 5% may live past 10 years.1

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