Prevalence of amyotrophic lateral sclerosis in the United States using established and novel methodologies, 2017

Figures & data

Table 1 Number and percentage of amyotrophic lateral sclerosis (ALS) cases and estimated prevalence, by age group, sex, and race – National ALS Registry, United States, 2017.

		Established algorithm			Capture-recapture methods				Mean estimation
Characteristic	Population^a	No. (%) cases	Prevalence (95% CI)^b	Observed count	Reported % missing^c	Missing cell estimate^c	No. cases, adjusted^d	Prevalence^b,d	No. cases^e	Prevalence^b,e
Total	321,004,407	17,800 (100.0)	5.5 (5.4, 5.6)	17,800	44.1%	14,043	31,843	9.9	24,821	7.7
Age groups (years)
18–39	92,704,957	518 (2.9)	0.6 (0.56, 0.64)	518	51.6%	552	1070	1.2	794	0.9
40–49	40,476,486	1471 (8.3)	3.6 (3.3, 3.8)	1471	51.6%	1568	3039	7.5	2255	5.6
50–59	43,278,449	3339 (18.3)	7.7 (7.3, 8.1)	3339	51.6%	3560	6899	15.9	5119	11.8
60–69	36,721,684	4814 (27.0)	13.1 (12.7, 13.5)	4814	43.2%	3661	8475	23.1	6645	18.1
70–79	21,533,719	4189 (23.5)	19.5 (19.0, 20.0)	4189	34.8%	2236	6425	29.8	5307	24.6
≥80	12,426,454	1428 (8.0)	11.5 (11.1, 11.9)	1428	34.8%	762	2190	17.6	1809	14.6
Unknown	–	2041 (11.5)
Sex
Male	158,018,753	11,034 (62.0)	7.0 (6.9, 7.1)	11,034	47.5%	9983	21,017	13.3	16,026	10.1
Female	162,985,654	6750 (37.9)	4.1 (4.0, 4.2)	6750	45.7%	5681	12,431	7.6	9590	5.9
Unknown	–	16 (0.1)
Race
White	234,370,202	12,929 (72.6)	5.5 (5.4, 5.6)	12,929	33.1%	6397	19,326	8.2	16,127	6.9
Black	40,610,815	1131 (6.4)	2.8 (2.7, 2.9)	1131	37.4%	676	1807	4.4	1469	3.6
Other	–	973 (5.5)		973	37.4%	1554	2527
Unknown		2511 (17.9)		2511	75%	10,166	12,677

^aPopulation based on US Census 2017.

^bCases per 100,000 US population.

^cEstimation based on capture–recapture methodology (31).

^dAdjusted estimation using the previously reported capture–recapture result.

^eMean or midpoint estimation between the established algorithm and the estimation obtained by capture–recapture methods.

Figure 1 Number of ALS cases by sex, race, and overall, adjusted for case undercount using the capture-recapture methodology and missing case estimates – National ALS Registry, United States, 2017.

Table 2 Number and percentage of amyotrophic lateral sclerosis (ALS) cases and estimated prevalence – National ALS Registry, United States, 2010–2016.

	Established algorithm		Capture–recapture method			Mean estimation
Year	No. cases/observed count	Prevalence^a	No. cases, missing^b	No. cases, corrected^b	Prevalence^a, corrected^b	No. cases^c	Prevalence^a,c
2010–2011	12,187	3.9	9614	21,801	7.0	16,994	5.5
2012	14,713	4.7	11,607	26,320	8.4	20,517	6.5
2013	15,908	5.0	12,550	28,458	9.0	22,183	7.0
2014	15,927	5.0	12,565	28,492	8.9	22,209	7.0
2015	16,583	5.2	13,082	29,665	9.2	23,124	7.2
2016	16,424	5.2	12,957	29,381	9.1	22,903	7.1

^aCases per 100,000 population.

^bEstimation based on capture–recapture methodology.

^cMean or midpoint estimation between the established algorithm and the estimation obtained by capture–recapture methods.

Figure 2 Estimated prevalence, adjusted prevalence, and mean prevalence per 100,000 of amyotrophic lateral sclerosis by sex, race, and overall – National ALS Registry, United States, 2017^a. Prevalence (light blue) is the estimation without the application of capture–recapture method. Adjusted prevalence (blue) is the upper-bound estimate using capture–recapture method. Mean prevalence (dark blue) is the midpoint estimation between the established algorithm and the estimation obtained by capture–recapture methods.