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
Asses...
...ng for cognitive and behavioral impairment should...
...of executive function may be considered to dete...
...r, there are insufficient data to support or re...
...e is insufficient evidence to support or r...
...gure 2. Algorithm for Nutrition...
Selecting a Treatment Regimen
...Sele...
...le should be offered to slow disease progression....
...veloping fatigue while taking riluzole, once ris...
...here are insufficient data at this time to...
...patients who have medically refractory sialorr...
and low-dose radiation therapy to the sa...
...r AffectIf approved by the FDA, and...
...amps/Spastic...
...fficient data to support or refute any sp...
...insufficient data to support or refute ex...
...Nutri...
...h impaired oral food intake, enteral nutrition...
...wever, there are insufficient data to supp...
...G should be considered for prolongi...
...er, there are insufficient data to su...
...es of 5-10 g daily, should NOT be g...
...in E should NOT be considered as treatment, (B)300...
...equivocal evidence regarding low-dose vitam...
...ease Management
Specialized multidisciplinary clinic referral s...
...considered to enhance quality of life. (C)3...
...Respirator...
...Assessment:...
...forced vital capacity (FVC) and maximal inspirat...
...l oximetry may be considered to detect hypoventil...
...essure (SNP) may be considered to detec...
...Treatment:...
...sive ventilation should be considered to tr...
...ilation may be considered to enhance quality of li...
...ilation may be considered at the earliest sign...
...nvasive ventilation (TIV) may be co...
...cal insufflation/exsufflation (MIE...
...insufficient data to support or refute...
...There are insufficient data to support or ref...
...1. FDA-Approved Drugs for ALS Dr...