Amyotrophic lateral sclerosis natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

The prognosis of Amyotrophic lateral sclerosis is generally poor with the majority of patients dying within 3-5 years of diagnosis.

• The symptoms of Amyotrophic lateral sclerosis usually develop in the fifth decade of life.^[1][2]
• Only 5% of the cases have an onset <30 years of age.^[1][2]

• With the progression of ALS, patients develop the distinctive feature of a combination of UMN and LMN degeneration signs within the same CNS region.

• Common complications of ALS include:^[2]
  • Pulmonary complications:
  • Dyspnea, Orthopnea, Hypoventilation, Pneumonia
  • Cognitive dysfunction
  • Weight loss, which is an indicative of a poor prognosis
  • The main cause of death in ALS is respiratory failure.

• Prognosis is generally poor, and the 30 months survival rate of patients with ALS is approximately 5%.^[3]
• Only 20% of the patients survive between 5 and 10 years after symptoms onset.^[3]
• Reduced survival to the disease is related to the older age of symptom onset, early respiratory muscle dysfunction, and bulbar onset disease. On the other hand, limb-onset disease, younger age at presentation of the disease and longer diagnostic delay are independent predictors of prolonged survival.^[4]

Some ALS subtypes vary according to prognosis. LMN form of ALS, which includes flail-limb variant and PMA, shows a slower progression than other forms of ALS. A prognosis of 2–4 years is seen in the pure bulbar palsy phenotype, which usually affects women older than 65 years of age. In this type of ALS, the disease remains localized to the oropharyngeal musculature and UMN features predominate.^[5]

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