07/10/2011 - Atualizado em 07/03/2013
Objective: To systematically review evidence bearing on the management of patients with Amyotrophic
Lateral Sclerosis (ALS).
Methods: The authors analyzed studies from 1998 to 2007 to update the 1999 practice parameter.
Topics covered in this section include slowing disease progression, nutrition, and respiratory
management for patients with ALS.
Results: The authors identified 8 Class I studies, 5 Class II studies, and 43 Class III studies in ALS.
Important treatments are available for patients with ALS that are underutilized. Noninvasive ventilation
(NIV), percutaneous endoscopic gastrostomy (PEG), and riluzole are particularly important
and have the best evidence. More studies are needed to examine the best tests of respiratory
function in ALS, as well as the optimal time for starting PEG, the impact of PEG on quality of life
and survival, and the effect of vitamins and supplements on ALS.
Recommendations: Riluzole should be offered to slow disease progression (Level A). PEG should
be considered to stabilize weight and to prolong survival in patients with ALS (Level B). NIV should
be considered to treat respiratory insufficiency in order to lengthen survival (Level B), and may be
considered to slow the decline of forced vital capacity (Level C) and improve quality of life (Level
C). Early initiation of NIV may increase compliance (Level C), and insufflation/exsufflation may be
considered to help clear secretions (Level C).