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

Assessment and Diagn...

...for cognitive and behavioral impairment sho...

...ests of executive function may be cons...

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


...ufficient evidence to support or ref...


...2. Algorithm for Nutrition Manage...


Selecting a Treatment Regimen

...ng a Treatment Regimen...

...d be offered to slow disease progression. (A)30...

...patients developing fatigue while taking rilu...

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


...patients who have medically refractory...

...radiation therapy to the salivary glands may b...


...ectIf approved by the FDA, and if...


...ramps/Spasticity

...nsufficient data to support or ref...

...here are insufficient data to suppor...


...rition Management...

...ts with impaired oral food intake, ent...

...there are insufficient data to su...

...e considered for prolonging survival. (B)3001...

...there are insufficient data to support or r...

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

...n E should NOT be considered as treatment, (B)300...

...he equivocal evidence regarding low-dos...


...e Management...

...idisciplinary clinic referral should be c...

...e considered to enhance quality of life. (C)30016...


...piratory Managemen...

...ssessment:...

...ced vital capacity (FVC) and maximal ins...

...octurnal oximetry may be considered to detect hy...

...sal pressure (SNP) may be considered to det...

Treatment...

...vasive ventilation should be considered...

...nvasive ventilation may be considered to...

...ntilation may be considered at the...

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

...insufflation/exsufflation (MIE) may be conside...

...e are insufficient data to support o...


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


...Approved Drugs for ALSHaving troub...