Abstract Details

Title Value of FVC and its Rate of Decline for Predicting Survival in ALS

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S5 - ALS and CMT: New Therapeutic Approaches (4:18 PM-4:30 PM)

Poster/Presentation
Number 005

Background ALS is a fatal neurodegenerative disorder of the motor system, leading to death due to progressive neuromuscular respiratory insufficiency in most patients.

Objective

To investigate the value of forced vital capacity (FVC) upon clinical presentation and its rate of decline for predicting survival in ALS.

Design/Methods This retrospective study in our academic center included 153 patients between 2016 and 2019 who were followed in the ALS clinic and for whom complete datasets were available. Initial FVC and its 3-month change were used for survival analysis via Kaplan-Meier method and Cox regression model. We defined two major patient groups based on their initial FVC (“high FVC” if > median, “low FVC” if < median) and subgroups based on their rate of FVC decline: (I) “initial high FVC/fast decline”, (II) “initial high FVC/slow decline”, (III) “initial low FVC/fast decline”, and (IV) “initial low FVC/slow decline”. These subgroups were compared for demographic, disease-related, and survival characteristics.

Results Initial FVC above the median (>85%) was associated with a survival of 39 months, while FVC below the median was associated with a survival of 16 months (p <0.001). Rate of FVC decline was also significantly associated with survival (p <0.001): patients with a 3-month FVC slope ≥ the median slope had a median survival of 41 months, while patients with a 3-month FVC slope below the median slope had a survival of 16 months. Subgroup analysis (p <0.001) showed that patients with “initial low FVC/fast decline” had a median survival of 13 months, while 26 months for patients with “initial low FVC/slow decline” and 47 months for patients with “initial high FVC/slow decline”.

Conclusions Initial FVC and its 3-month decline is a reliable predictor for survival. This simple metric can be utilized by clinicians and their patients to guide further management, as well as by researchers designing clinical trials.

Authors/Disclosures
Muhannad Seyam, MD PRESENTER	Dr. Seyam has nothing to disclose.
Richard Hubbell (Loyola U Chicago)	No disclosure on file
Diantha Howard	No disclosure on file
Joan Skelly (University of Vermont)	No disclosure on file
Waqar Waheed, MD, MBBS	Dr. Waheed has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB.
Rup Tandan, MD, FRCP, FAAN (University of Vermont (UVM)/UVM Medical Center)	Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Apellis. Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amylyx. The institution of Dr. Tandan has received research support from Alexion. The institution of Dr. Tandan has received research support from Apellis. The institution of Dr. Tandan has received research support from Cytokinetics.