Clinical characteristics of a large cohort of US participants enrolled in the National Amyotrophic Lateral Sclerosis (ALS) Registry, 2010–2015

Figures & data

Table 1 Demographic characteristics among US adults with ALS who responded to the National ALS Registry’s Clinical Survey Module (19 October 2010–31 December 2015).

Characteristic	Clinical survey participants		All Registry participants^a
	N =1758	%	N = 6352	%
Age at diagnosis
18–39	76	4.3	408	6.4
40–49	247	14.1	981	15.5
50–59	511	29.1	1922	30.3
60–69	580	33	2033	32.1
70–79	288	16.4	869	13.7
80+	55	3.1	131	2
Missing	1	0	8	0.1
Gender
Male	1071	60.9	3841	60.5
Female	687	39.1	2511	39.5
Race
Nonwhite	78	4.5	293	4.6
White	1658	95.5	5809	91.5
Missing	22	1.3	250	3.9
Census region
Midwest	532	30.4	1790	28.2
South	578	33.1	2200	34.5
West	389	22.2	1357	21.4
East	242	13.8	957	15.1
Other/missing	17	0.1	48	0.8

^aRegistry enrollees who completed one or more of the 17 available surveys.

Table 2 Initial site of onset among 1758^a US adults with ALS who responded to the National ALS Registry’s Clinical Survey Module (19 October 2010–31 December 2015).

Initial site of onset^b	Male		Female		Total		Chi-square p value
	n	%	n	%	n	%
Limb	695	74.3	466	67.8	1261	71.8	0.023
Arm or hand	397	37.1	216	31.4	613	34.9	0.02
Leg or foot	398	37.2	250	36.4	648	36.9	0.74
Bulbar (speech and/or swallowing muscles)	211	19.7	178	25.9	389	22.1	0.0023
Trunk/global	65	6.1	43	6.4	108	6.1	0.87
Neck, back, or abdominal area	23	2.2	19	2.8	42	2.4	0.41
Breathing muscles	18	1.7	12	1.8	30	1.7	0.92
Total body	24	2.2	12	1.8	36	2	0.48

^a100 participants entered missing or unknown data for initial site of onset.

^bInitial site of onset refers to the first body region where a patient reported a weakness or symptom prior to ALS diagnosis.

The bolded values are the categories, the un-bolded values are the sub categories.

Table 3 Other symptoms experienced among 1758^a US adults with ALS who responded to the National ALS Registry’s Clinical Survey Module (19 October 2010–31 December 2015).

Other symptoms^b	Male		Female		Total		Chi-square p value
(Have you ever experienced the following?)	n	%	n	%	n	%
Pneumonia	59	5.6	59	8.7	118	6.7	0.043
Falls	263	25.1	184	27.2	447	25.4	0.37
Blood clot	40	3.8	29	4.3	69	3.9	0.47
Cramps	611	57.9	386	57.3	997	56.7	0.31
Twitching (fasciculations)	609	57.8	380	56.4	989	56.3	0.45
Problems with speech (dysarthria)	334	33.4	247	38.6	581	33	0.023
Difficulty controlling bowels	127	12.1	96	14.3	223	12.7	0.42

^aParticipants may have entered missing or unknown data for initial site of onset. No more than 2.2% are unknown or missing.

^bInitial site of onset refers to the first body region where a patient reported a weakness or symptom prior to ALS diagnosis. The following symptoms could have occurred before or after an ALS diagnosis and regardless of site of onset.

^cStatistically significant at p < 0.05.

Figure 1 Median time from ALS diagnosis to symptoms onset among adults with ALS, 19 October 2010–31 December 2015.

Figure 2 Median time from ALS diagnosis to symptoms onset stratified on body part weakness among adults with ALS, 19 October 2010–31 December 2015.

Table 4 Use of Interventions among 1758^a US Adults with ALS who responded to the National ALS Registry’s Clinical Survey Module (19 October 2010–31 December 2015).

Interventions^b	Male		Female		Total		Chi-square p value^c
	n	%	n	%	n	%
Wheelchair or scooter	355	33.7	222	32.8	577	32.8	0.43
Noninvasive breathing equipment	309	29.5	218	32.3	527	30.0	0.43
Tracheotomy	26	2.5	19	2.8	45	2.6	0.82
Communication device	161	15.4	120	17.8	281	16.0	0.09
Riluzole
Used in the past	135	12.8	91	13.4	226	12.9	0.87
Currently using	523	49.5	326	48.1	849	48.3	0.94
Hospice	54	5.1	37	5.5	91	5.2	0.5

^aParticipants may have entered missing or unknown data for initial site of onset. Unknown or missing data ranges from <2 to 8.2%.

^bIncludes pharmacological and non-pharmacological interventions.

^cStatistically significant at p < 0.05.