The Care of the Patient with Amyotrophic Lateral Sclerosis: Drug, Nutritional, And Respiratory Therapies

Publication Date: October 1, 2009

Key Points

Key Points

  • Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive, paralyzing, neurodegenerative disease. It is characterized by loss of motor neurons in the spinal cord, brainstem, and motor cortex.
  • Most patients with ALS die within 2 to 5 years of onset.
  • The mainstay of ALS management is symptomatic treatment and palliative care.
  • Riluzole has FDA approval for slowing disease progress in ALS.
  • Multidisciplinary clinics are the optimal means to coordinate ALS treatment.

Figure 1. Algorithm for Respiratory Management


Assessment and Diagnosis

...nt and Diagnosis...

...cognitive and behavioral impairment should be...

...g tests of executive function may be consid...

..., there are insufficient data to support...


...insufficient evidence to support or refute a...


...Algorithm for Nutrition Management...


Selecting a Treatment Regimen

...ting a Treatment Regime...

...d be offered to slow disease progressi...

...ients developing fatigue while taking...

...re insufficient data at this time to suppo...


...have medically refractory sialorrhea, botulinu...

...ose radiation therapy to the salivar...


...ffectIf approved by the FDA, and if sid...


...mps/Spasticity...

...are insufficient data to support or re...

...e insufficient data to support or ref...


Nutrition Managemen...

...with impaired oral food intake, enteral...

...ever, there are insufficient data to s...

...considered for prolonging survival. (B)300167...

...e are insufficient data to support or ref...

...doses of 5-10 g daily, should NOT be g...

...amin E should NOT be considered as treatment, (B)3...

...uivocal evidence regarding low-dose vitamin...


...se Management

...pecialized multidisciplinary clini...

...nsidered to enhance quality of life. (...


...tory Management...

...essment:...

...ine forced vital capacity (FVC) and max...

...turnal oximetry may be considered to detect hyp...

...iff nasal pressure (SNP) may be conside...

...atment:...

...sive ventilation should be considered to...

...asive ventilation may be considered to en...

...ve ventilation may be considered a...

...stomy invasive ventilation (TIV) may be considere...

...ufflation/exsufflation (MIE) may be co...

...e insufficient data to support or...


...age ALSThere are insufficient data to support...


...roved Drugs for ALSHaving trouble...