Abstract Details

Title Validation of a staging system in ALS: Comparison of the US and European Populations

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-014

Objective To apply the King’s Staging system retrospectively to ALS subjects followed in two US clinics and validate this staging system.

Background King’s Staging is a validated staging system that provides prognostic information and allows for judicious resource management and effective research design.

Design/Methods We applied the King’s Staging system to 221 ALS subjects from three clinics. We identified the onset of disease (stage 1) and compared the timing and percent of disease duration in each additional stage (stage 2: involvement of second region, stage 3: involvement of third region, stage 4: need for gastrostomy or assisted ventilation) for bulbar and limb onset types. Stage 4 was separated into 4a (need for gastrostomy) and 4b (need for assisted ventilation). The proportion or percentage of time in each stage was determined.

Results

In US subjects, stage 2 occurred 35% through the disease course, stage 3 at 48%, and stage 4 at 61%, with 4a and 4b at 63%. The time to stage 2, 3, 4a and 4b were statistically different from the overall European population (40%, 59%, 76%, 75% respectively). In the US limb-onset population, the time to stage 3, 4a, and 4b were statistically different from the European population (63%, 81%, 73%). In the US bulbar-onset population, the time to stage 4a and 4b were significantly different from the European population (71% and 81% respectively). Median survival for the US population was 36.8 months compared to 48.3 months for the UK population.

Conclusions

The King’s Staging was easily applied to the US population and ALS subjects progressively moved through the stages. The differences that were seen may be a result of differences in data collection (retrospective versus prospective), bias in case selection, and differences in demographic profiles, population characteristics, and rules for implementing interventions.

Authors/Disclosures
Justin Y. Kwan, MD, FAAN (National Institutes of Health) PRESENTER	Dr. Kwan has received research support from National Institutes of Health. Dr. Kwan has received personal compensation in the range of $100,000-$499,999 for serving as a Employee with National Institutes of Health.
Diana Z. Li, MD (Abington Neurological Associates)	Dr. Li has nothing to disclose.
Brooke Lubinski (NeurExpand Brain Center)	No disclosure on file
Anahita F. Deboo, MD (Temple University School of Medicine - Neurology)	Dr. Deboo has nothing to disclose.
	No disclosure on file
Ammar Al-Chalabi, PhD, FRCP DipStat (King'S College London)	The institution of Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for OrionPharma. The institution of Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics Inc. The institution of Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Tanabe Pharma. The institution of Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. The institution of Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Quralis. Dr. Al-Chalabi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for My Name'5 Doddie Foundation. The institution of Dr. Al-Chalabi has received research support from Medical Research Council. The institution of Dr. Al-Chalabi has received research support from NIHR. The institution of Dr. Al-Chalabi has received research support from European Commission. The institution of Dr. Al-Chalabi has received research support from MND Association. The institution of Dr. Al-Chalabi has received research support from My Name'5 Doddie Foundation. Dr. Al-Chalabi has received publishing royalties from a publication relating to health care. Dr. Al-Chalabi has received publishing royalties from a publication relating to health care. Dr. Al-Chalabi has a non-compensated relationship as a SAB Member and Executive Member with TRICALS that is relevant to AAN interests or activities.
Terry D. Heiman-Patterson, MD (Temple University Lewis Katz School of Medicine)	Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MTPA. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Heiman-Patterson has received personal compensation in the range of $0-$499 for serving as a Consultant for novartis. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for biogen. The institution of Dr. Heiman-Patterson has received research support from MTPA. The institution of Dr. Heiman-Patterson has received research support from State of Pennsylvania . The institution of Dr. Heiman-Patterson has received research support from ALS Association. The institution of Dr. Heiman-Patterson has received research support from ALS United. The institution of Dr. Heiman-Patterson has received research support from ALS Hope Foundation.

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