Abstract Details

Title Multivariate Analysis of Survival in an Amyotrophic Lateral Sclerosis Clinic Population

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S35 - Amyotrophic Lateral Sclerosis (5:06 PM-5:18 PM)

Poster/Presentation
Number 009

Objective To determine factors that predict survival in patients with amyotrophic lateral sclerosis.

Background Many factors potentially contribute to survival in ALS, making the prediction of survival for individuals challenging. Although prior studies have identified potential variables that predict survival, many of these studies are of small sample size, and the findings are often not replicated in subsequent studies. Multivariate analysis of clinical, demographic, and genetic data on large cohorts is necessary to better model survival in ALS. We report the baseline clinical, demographic, and genetic data collected in our ongoing clinical registry and their influence on survival.

Design/Methods Age at diagnosis, gender, race, site of onset, time to diagnosis, FVC, and BMI were collected from 1,986 patients seen at Mayo Clinic Florida ALS Center between 1998 and 2019. A subgroup of 948 consented for analysis of C9orf72 repeat expansion. 1,450 patients were confirmed to be deceased at the time of analysis. Survival was determined from reported symptom onset and query of national death databases. Kaplan-Meier survival analysis and Cox regression modeling were used for univariate and multivariate analyses.

Results The median survival was 30.7 months from reported onset and 17.6 months from first diagnosis. While nearly all baseline features were associated with survival in univariate analysis, only age, FVC, BMI, C9orf72 status, and time to diagnosis were independent predictors of survival in multivariate analysis.

Conclusions The median survival of our cohort is consistent with prior series. Contrary to prior studies, bulbar site of onset and gender were not found to be predictors of survival in multivariate analysis. This illustrates the need for multivariate analyses of large cohorts of patients to study the effect of individual factors on survival in ALS.

Authors/Disclosures
Jaimin S. Shah, MD (Mayo Clinic Florida) PRESENTER	No disclosure on file
Marka M. Van Blitterswijk, MD, PhD (Mayo Clinic Florida)	No disclosure on file
Rosa Rademakers, PhD (Mayo Clinic Dept of Neuroscience)	Rosa Rademakers, PhD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Mayo Clinic. Rosa Rademakers, PhD has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arkuda Therapeutics. Rosa Rademakers, PhD has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Alzheimer Fondation. Rosa Rademakers, PhD has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Kisick Family Foundation (FTD grant program). The institution of Rosa Rademakers, PhD has received research support from NIH. The institution of Rosa Rademakers, PhD has received research support from DOD. The institution of Rosa Rademakers, PhD has received research support from Flanders Institute for Biotechnology (VIB). The institution of Rosa Rademakers, PhD has received research support from Flanders Funds for Scientific Research.
Otto Pedraza (Mayo Clinic)	The institution of Otto Pedraza has received research support from Alzheimer's Association. Otto Pedraza has received publishing royalties from a publication relating to health care.
Beth K. Rush, PhD	Dr. Rush has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Bjorn E. Oskarsson, MD, FAAN (Mayo Clinic)	Dr. Oskarsson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. The institution of Dr. Oskarsson has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Oskarsson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AnnJi. The institution of Dr. Oskarsson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Oskarsson has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Tsumura. The institution of Dr. Oskarsson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MediciNova. The institution of Dr. Oskarsson has received research support from Biogen. The institution of Dr. Oskarsson has received research support from Medicinova. The institution of Dr. Oskarsson has received research support from Cytokinetics. The institution of Dr. Oskarsson has received research support from Calico. The institution of Dr. Oskarsson has received research support from Mitsubishi. The institution of Dr. Oskarsson has received research support from Tsumura. The institution of Dr. Oskarsson has received research support from Sanofi. The institution of Dr. Oskarsson has received research support from AZTherapeutics. The institution of Dr. Oskarsson has received research support from Orion. The institution of Dr. Oskarsson has received research support from Esaii.

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