The Care of the Patient with Amyotrophic Lateral Sclerosis: Drug, Nutritional, And Respiratory Therapies
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
...ssment and Diagnosis
Screening for cognitive and behavioral impairmen...
...of executive function may be consider...
...owever, there are insufficient data t...
...is insufficient evidence to support or re...
...igure 2. Algorithm for Nutrition...
Selecting a Treatment Regimen
...a Treatment Regimen...
...should be offered to slow disease progres...
...ts developing fatigue while taking...
...insufficient data at this time to s...
...who have medically refractory sialorrhe...
...low-dose radiation therapy to the sa...
...lbar AffectIf approved by the FDA, a...
...ps/Spasticit...
There are insufficient data to support or re...
...icient data to support or refute exe...
Nutrition Managemen...
...ients with impaired oral food intake,...
...ere are insufficient data to suppo...
...considered for prolonging survival. (B...
...there are insufficient data to support or refut...
...atine, in doses of 5-10 g daily, should NOT b...
...dose vitamin E should NOT be consi...
...the equivocal evidence regarding low-dos...
...sease Managemen...
...ed multidisciplinary clinic referral should be con...
...be considered to enhance quality of life....
...ratory Manageme...
...essment:...
...forced vital capacity (FVC) and m...
...ximetry may be considered to detect hypovent...
Sniff nasal pressure (SNP) may be considered t...
Treatme...
...ive ventilation should be considered to treat res...
...tilation may be considered to enhance qu...
...sive ventilation may be considered at the ea...
...racheostomy invasive ventilation (TIV) may be c...
...anical insufflation/exsufflation (MIE) may be...
...icient data to support or refute high-fre...
...ALSThere are insufficient data to suppor...
...le 1. FDA-Approved Drugs for ALSHaving troubl...