Outcomes research in amyotrophic lateral sclerosis: lessons learned from the amyotrophic lateral sclerosis clinical assessment, research, and education database.
OBJECTIVE
To examine the care of patients with ALS following the publication of the standardized recommendations for the management of patients with amyotrophic lateral sclerosis (ALS) published in 1999 by the American Academy of Neurology.


METHODS
Specific aspects of ALS patient management have been evaluated serially using a national Amyotrophic Lateral Sclerosis Clinical Assessment, Research, and Education (ALS CARE) database to encourage compliance with these recommendations and to assure continuing quality improvement.


RESULTS
The most recent analysis of 5,600 patients shows interesting epidemiological observations and treatment trends. Proper management of many ALS symptoms has increased substantially since the first publication of the guidelines, and awareness of pseudobulbar affect has increased. Other recommendations are underutilized: Only 9% undergo percutaneous endoscopic gastrostomy, although this procedure was recommended in 22% of patients; and noninvasive positive pressure ventilation was used by only 21% of patients despite being associated with improved 5-year survival rates.


CONCLUSIONS
This observational database has been a useful tool in monitoring compliance with the standard of care for patients with ALS and may have resulted in greater adherence to guidelines.
Miller
Robert G
RG
Forbes Norris MDA/ALS Research Center, California Pacific Medical Center 94115, San Francisco, CA, USA. millerrx@sutterhealth.org
Anderson
Fred
F
Brooks
Benjamin Rix
BR
Mitsumoto
Hiroshi
H
Bradley
Walter G
WG
Ringel
Steven P
SP
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D016208:Databases, Factual; D005260:Female; D047830:Gulf War; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D008991:Monitoring, Physiologic; D009656:North America / Q000453:epidemiology; D017063:Outcome Assessment, Health Care; D017410:Practice Guidelines as Topic; D012106:Research / Q000639:trends*