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Table 2 Comparison of baseline characteristics between subtypes in all individuals with ALS–FTD spectrum disorder

From: Novel data-driven subtypes and stages of brain atrophy in the ALS–FTD spectrum

 

S1

n = 116

S2

n = 47

S0

n = 44

Missing data

PS1vsS2

PS0vS1

PS0vsS2

Age at MRI, years

59.9 (9.7)

63.0 (9.5)

60.9 (9.2)

0.0%

0.07

0.59

0.27

Sex, male%

69 (59.5%)

28 (59.6%)

25 (56.8%)

0.0%

0.99

0.76

0.79

Education, years

16.8 (11.3)

15.4 (2.7)

15.1 (2.7)

0.0%

0.80

0.45

0.57

Disease duration, monthsa

29.0 (200.5)

36.7 (200.8)

28.4 (126.9)

0.0%

0.19

0.35

0.08

Diagnostic delay, months

29.1 (24.7)

42.5 (41.8)

19.1 (20.8)

0.0%

0.12

0.002

0.0004

Clinical phenotypes

n = 116

n = 47

n = 44

0.0%

0.06

< 0.0001

< 0.0001

ALS

48 (41.4%)

14 (29.8%)

41 (93.1%)

–

–

–

–

ALS–FTD

26 (22.4%)

19 (40.4%)

2 (4.5%)

–

–

–

–

bvFTD

42 (36.2%)

14 (29.8%)

1 (2.3%)

–

–

–

–

Symptom onset site

n = 110

n = 45

n = 44

3.9%

0.17

< 0.0001

< 0.0001

LMN

45 (40.9%)

16 (35.6%)

31 (70.5%)

–

–

–

–

UMN

14 (12.7%)

2 (4.4%)

11 (25.0%)

–

–

–

–

Cognitive

51 (46.4%)

27 (60.0%)

2 (4.5%)

–

–

–

–

MMSE

25.7 (4.5)

24.3 (5.6)

28.2 (2.2)

6.3%

0.40

0.0001

0.0008

ECAS total

91.0 (28.0)

87.0 (30.2)

113.9 (9.7)

64.3%

0.75

0.002

0.006

ALS specific score

66.8 (22.2)

85.5 (8.3)

85.5 (8.3)

–

0.89

0.001

0.009

ALS non-specific score

24.2 (7.1)

21.8 (7.8)

28.4 (2.5)

–

0.28

< 0.05

0.005

PBAC total

67.3 (14.9)

62.8 (19.1)

81.5 (4.2)

48.8%

0.31

< 0.0001

0.0002

Executive scale

8.0 (4.0)

8.8 (3.5)

12.7 (2.5)

–

0.40

< 0.0001

0.0001

Language scale

16.1 (3.3)

14.2 (4.9)

18.6 (0.6)

–

0.10

< 0.0001

< 0.0001

Visual scale

15.3 (3.7)

15.9 (3.9)

17.6 (0.7)

–

0.21

0.0002

0.05

Memory scale

13.0 (4.7)

10.4 (6.2)

14.6 (3.3)

–

0.13

0.15

0.02

Behavioral scale

14.8 (4.0)

13.5 (4.5)

18.0 (0.0)

–

0.15

< 0.0001

< 0.0001

BNT

24.8 (4.7)

17.3 (8.0)

27.6 (1.9)

37.2%

< 0.0001

0.007

< 0.0001

Animal fluency task

13.1 (6.6)

10.4 (7.1)

19.9 (5.6)

27.5%

0.06

< 0.0001

< 0.0001

Letter fluency task

8.9 (5.3)

8.0 (5.0)

13.1 (4.5)

6.8%

0.42

< 0.0001

< 0.0001

Digit forward span

6.1 (1.4)

6.1 (1.2)

7.1 (1.2)

14.0%

0.77

0.0002

0.0003

Digit backward span

3.9 (1.7)

3.9 (1.6)

4.8 (1.2)

14.5%

0.86

0.001

0.009

Genetic pathogenic variantsb

n = 112

n = 45

n = 23

27.1%

–

–

–

C9orf72

37 (33.0%)

10 (22.2%)

1 (4.4%)

–

0.18

0.004

0.06

GRN

11 (9.8%)

0 (0.0%)

0 (0.0%)

–

0.03

0.21

1.00

MME

0 (0.0%)

0 (0.0%)

1 (4.4%)

–

1.00

0.17

0.34

TBK1

0 (0.0%)

2 (4.4%)

0 (0.0%)

–

0.08

1.00

0.55

TARDBP

0 (0.0%)

2 (4.4%)

0 (0.0%)

–

0.08

1.00

0.55

FTLD/ALS-TDPc

n = 29

n = 15

n = 11

73.4%

0.002

0.0009

< 0.0001

Type A

13 (44.8%)

3 (20.0%)

0 (0.0%)

–

–

–

–

Type B/E

8 (27.6%)

9 (60.0%)

1 (9.1%)

–

–

–

–

Type C

0 (0.0%)

3 (20.0%)

0 (0.0%)

–

–

–

–

Non-specific

8 (27.6%)

0 (0.0%)

10 (90.9%)

–

–

–

–

SuStaIn stage

9.8 (8.1)

12.2 (7.8)

0 (0.0)

0.0%

< 0.05

–

–

  1. Bold values indicate statistically significant results
  2. Data are presented as mean (standard deviation) for the continuous variables, and as number (frequency) for the categorical variables. Missing data indicate the percentage of individuals with missing data
  3. S0 Normal-appearing group, S1 Prefrontal/Somatomotor-predominant subtype, S2 Limbic-predominant subtype, ALS amyotrophic lateral sclerosis, ALS–FTD amyotrophic lateral sclerosis-frontotemporal degeneration, bvFTD behavioral variant frontotemporal degeneration, LMN lower motor neuron, UMN upper motor neuron, MMSE Mini-Mental Status Examination, ECAS Edinburgh Cognitive Assessment Scale, PBAC Philadelphia Brief Assessment of Cognition, BNT Boston naming test, FTLD/ALS-TDP frontotemporal lobar degeneration or amyotrophic lateral sclerosis with TDP-43 inclusions, SuStaIn Subtype and Stage Inference
  4. aData presented as median (range)
  5. bNumber of individuals for genetic screening
  6. cNumber of individuals receiving neuropathological examination
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Translational Neurodegeneration

ISSN: 2047-9158

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