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Abstract Details

Disease Severity and Disease Trajectory of Amyotrophic Lateral Sclerosis (ALS) Patients at Frist Clinic Visit Measured Prospectively with "ALS Dashboard" - A Six-Domain (Cognition, Affect, Bulbar, Respiratory, Arm, Leg) Staging System - Comparison of Two Cohorts
Anterior Horn
P07 - (-)
088
BACKGROUND: While the time from disease onset to disease diagnosis has been studied in several populations of people with ALS, the disease burden at first clinic visit and at diagnosis has been less carefully analyzed.
DESIGN/METHODS: From 2009-2011 181 patients [101 M; 80 F] were referred to the Carolinas Neuromuscular/ALS-MDA Center. 17 were found not to have ALS. The remaining patients were staged according to the "ALS Dashboard" staging algorithm in cognitive, affect, bulbar, respiratory, arm and leg domains. Age at diagnosis, site of onset, time from onset to diagnosis, vital capacity at diagnosis, ALSFRS-R total and bulbar, arm, leg, respiratory subscores at diagnosis were calculated. Domain-specific stage at diagnosis and at three month intervals after diagnosis were established for each patient. Distribution of domain-specific staging at first clinic visit was compared with a previous cohort from 1998-2001. Statistical analysis was performed with MedcCalc software version 12. 2.1 [www.medcalc.org/].
RESULTS: Cognitive stage ? 3 present at diagnosis was increased in the 2009-2011 cohort compared with the 1998-2001 cohort [23.8%/11.4% p<0.01]. Affect stage ? 3 [0.0/7.5%] was not different. Bulbar stage ? 3 [42.9%/30.0%] and Respiratory stage ? 3 was also increased significantly [57.1%/6.0%]. Arm stage ? 3 [23.8%/20.4%] and Leg stage ? 3 [52.4%/44.5%] was not significantly different.
CONCLUSIONS: "ALS Dashboard" staging changes in the prospectively evaluated cohort was more prominent in the respiratory domain due to changes in standard of practice for these patients.
Authors/Disclosures
Elena Bravver, MD
PRESENTER
No disclosure on file
Mohammed S. Sanjak, PhD (Carolinas Healthcare System) No disclosure on file
Benjamin R. Brooks, MD, FAAN (Clinical Trials Planning LLC) Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Mitsubishi Tanabe Pharma America. Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Medicinova. Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AB Science. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Mitsubishi Tanabe Pharma America. The institution of Dr. Brooks has received research support from Mitsubishi TanabePharma America. Dr. Brooks has received personal compensation in the range of $0-$499 for serving as a Member Annual Surveillance Committee CDC National ALS Registry with Center for Disease Control Agency Toxic Substances Disease Registry. Dr. Brooks has a non-compensated relationship as a Member ALS Quality Measures Subcommittee with 好色先生 that is relevant to AAN interests or activities.
Steven E. Arnold, MD (Massachusetts General Hospital) Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EIP Pharma. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sage Therapeutics. Dr. Arnold has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cortexyme. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Boyle Shaughnessy Law. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Wolf Greenfield. Dr. Arnold has received personal compensation in the range of $500-$4,999 for serving as a Advisory Board Member with Bob's Last Marathon.