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

...ent and Diagnosis...

...ening for cognitive and behavioral...

Screening tests of executive function may be cons...

...here are insufficient data to support...


...nsufficient evidence to support or refut...


...lgorithm for Nutrition Management...


Selecting a Treatment Regimen

...g a Treatment Regimen...

...uzole should be offered to slow disease...

...nts developing fatigue while taking riluz...

...insufficient data at this time to support...


...n patients who have medically refracto...

...e radiation therapy to the salivary glands...


...bulbar AffectIf approved by the FDA, and i...


...ps/Spasticit...

...nsufficient data to support or refut...

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


...ition Management...

...ith impaired oral food intake, enteral nutr...

...here are insufficient data to suppo...

...d be considered for prolonging survival. (B)30016...

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

...ine, in doses of 5-10 g daily, should N...

...ose vitamin E should NOT be considered...

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


...ase Management

...ed multidisciplinary clinic referral shoul...

...e considered to enhance quality of life. (C)3001...


...piratory Managemen...

...sessment:

...ne forced vital capacity (FVC) and maxima...

Nocturnal oximetry may be considered to...

...nasal pressure (SNP) may be considered to...

...atment:...

...invasive ventilation should be considered to trea...

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

...ventilation may be considered at the earliest...

...eostomy invasive ventilation (TIV) may b...

...cal insufflation/exsufflation (MIE) may be con...

There are insufficient data to support or refute...


Late-stage ALSThere are insufficient d...


.... FDA-Approved Drugs for ALSHaving t...