Prognosis

ALS follows a progressive course without intervals of remissions, relapses, or stabilization, causing progressive disability and ultimately death. The disease is highly variable between affected individuals in terms of clinical presentation and time course. Median survival is 3-5 years, but survival for up to 10 years and even beyond (in approximately 10% to 20% of patients) has been reported.[21][122][123]

Prognostic factors

Prognostic factors associated with more prolonged survival include:

  • Treatment with noninvasive ventilation (NIV)[123]

  • Enteral nutrition[84]

  • Younger age at diagnosis[124][125]​​

  • Limb onset​[124][126]

  • Baseline forced vital capacity (FVC) of >75% (median survival 5 years)[126]

  • Longer time from symptom onset to diagnosis.[125]

Prognostic factors associated with worse outcome include:

  • Older age at diagnosis

  • Bulbar onset

  • Comorbidity with frontotemporal dementia

  • Baseline FVC of <75%

  • Substantial weight loss.[127]

The rate of symptom progression is considered to be an independent prognostic factor.[123]

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