Table 1 Features analyzed in this study with details on overall distribution and by survival group

Diagnostic Delay (Disease Duration)

• Average (9 − 18 months)

• Short (≤ 8 months)

• Long (≥ 19 months)

40%

39%

21%

29%

66%

5%

41%

35%

24%

No

Age at Onset (range)

• 0 − 39

• 40 − 49

• 50 − 59

• 60 − 69

• 70 + 

13%

22%

31%

26%

8%

4%

16%

35%

31%

14%

15%

22%

31%

25%

7%

Sex

• Female

• Male

36%

64%

30%

70%

37%

63%

Site of Onset

• Bulbar

• Limb/Spinal

19%

81%

26%

74%

18%

82%

Riluzole

• No

• Yes

69%

31%

74%

26%

68%

32%

Forced Vital Capacity (FVC)

• Abnormal (< 80%)

• Normal (≥ 80%)

21%

79%

32%

68%

20%

80%

Yes

Body Mass Index (BMI)

• Underweight (≤ 18.4)

• Normal (18.5 − 24.9)

• Overweight (25.0 − 29.9)

• Obesity (≥ 30)

3%

37%

37%

23%

3%

40%

38%

19%

3%

37%

37%

23%

Gastrostomy

• No

• Yes

96%

4%

97%

3%

96%

4%

Regions Involved

Quantity

• 1

• 2

• 3

• 4

13%

28%

34%

25%

10%

25%

36%

29%

14%

28%

33%

25%

Bulbar

• No

• Yes

35%

65%

29%

71%

36%

64%

Upper Limb

19%

81%

20%

80%

19%

81%

Lower Limb

13%

87%

13%

87%

13%

87%

Respiratory

62%

38%

54%

46%

63%

37%

ALSFRS Slopes by Question

Q1 − Speech

• Average (0.05 − 0.13/month)

• Rapid (≥ 0.14/month)

• Slow (≤ 0.04/month)

18%

3%

79%

34%

10%

56%

16%

2%

82%

Q2 − Salivation

12%

2%

86%

26%

7%

67%

10%

1%

89%

Q3 − Swallowing

13%

1%

86%

29%

4%

67%

10%

1%

89%

Q4 − Handwriting

18%

3%

79%

30%

12%

58%

16%

1%

83%

Q5 − Cutting

24%

4%

72%

31%

17%

52%

23%

2%

75%

Q6 − Dressing & Hygiene

30%

5%

65%

42%

21%

37%

28%

3%

69%

Q7 − Turning in Bed

18%

2%

80%

33%

11%

56%

16%

1%

83%

Q8 − Walking

29%

3%

68%

43%

16%

41%

27%

2%

71%

Q9 − Climbing Stairs

37%

11%

52%

36%

35%

29%

38%

8%

54%

Q10 − Respiratory

8%

1%

91%

20%

4%

76%

6%

1%

93%