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
...nt and Diagnosis...
...ng for cognitive and behavioral impairment sho...
...reening tests of executive function...
...ver, there are insufficient data to support or...
...is insufficient evidence to support or refu...
...gure 2. Algorithm for Nutrition Ma...
Selecting a Treatment Regimen
...cting a Treatment Regimen
...ould be offered to slow disease progression...
...patients developing fatigue while ta...
...e are insufficient data at this time to support...
...ho have medically refractory sialorrhea, botulinu...
...radiation therapy to the salivary glands may...
Pseudobulbar AffectIf approved by the FDA,...
...ramps/Spasticity...
...ufficient data to support or refute any specif...
...re insufficient data to support or...
Nutrition Manage...
...patients with impaired oral food in...
...re are insufficient data to support or...
...EG should be considered for prolonging survival....
...ever, there are insufficient data to su...
...ne, in doses of 5-10 g daily, should NOT be...
...dose vitamin E should NOT be considered as trea...
...quivocal evidence regarding low-dose vitami...
...sease Management
...pecialized multidisciplinary clinic ref...
...onsidered to enhance quality of li...
...piratory Managem...
...sessment...
...ital capacity (FVC) and maximal inspiratory pre...
...try may be considered to detect hypoven...
...nasal pressure (SNP) may be considere...
...reatment:...
...vasive ventilation should be considered to...
...ive ventilation may be considered to...
...ntilation may be considered at the earlies...
...invasive ventilation (TIV) may be cons...
...nical insufflation/exsufflation (MIE) may be c...
...fficient data to support or refute...
...There are insufficient data to support or r...
...ble 1. FDA-Approved Drugs for ALSHaving trouble...