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...

...r cognitive and behavioral impairment shoul...

Screening tests of executive function may be con...

However, there are insufficient data to su...


...is insufficient evidence to support or r...


...2. Algorithm for Nutrition Management...


Selecting a Treatment Regimen

...ng a Treatment Regimen...

...be offered to slow disease progression....

...tients developing fatigue while taking...

...here are insufficient data at this time to suppor...


...who have medically refractory sialorrhea, bot...

...low-dose radiation therapy to the saliv...


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


...amps/Spasticity...

...e insufficient data to support or refute a...

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


...trition Management...

...patients with impaired oral food intake, ente...

...re insufficient data to support or refute sp...

...hould be considered for prolonging...

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

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

...igh-dose vitamin E should NOT be consid...

...vocal evidence regarding low-dose vitamin E permi...


...sease Management...

...idisciplinary clinic referral should be co...

...nd may be considered to enhance quality of...


...ratory Management...

...essment:...

...tal capacity (FVC) and maximal insp...

...nal oximetry may be considered to detect hypov...

...asal pressure (SNP) may be considered to detect hy...

Treatmen...

Noninvasive ventilation should be con...

...ventilation may be considered to enhance q...

...asive ventilation may be considered...

...nvasive ventilation (TIV) may be consider...

...flation/exsufflation (MIE) may be considered to cl...

...ufficient data to support or refute...


...ALSThere are insufficient data to supp...


...proved Drugs for ALSHaving trouble viewing...