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Table 1 Comparison of baseline characteristics between clinical phenotypes in all individuals with ALS–FTD spectrum disorder

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

 

ALS

n = 103

ALS–FTD

n = 47

bvFTD

n = 57

Missing data*

PALSvsALS−FTD

PALSvsbvFTD

PALS−FTDvsbvFTD

Age at MRI, years

58.9 (10.3)

62.6 (9.6)

62.9 (7.5)

0.0%

0.04

0.005

0.85

Sex, male%

58 (56.3%)

28 (59.6%)

36 (63.2%)

0.0%

0.71

0.40

0.71

Education, years

16.7 (12.0)

15.0 (2.8)

16.1 (2.8)

0.0%

0.72

0.09

0.09

Disease duration, monthsa

23.9 (200.1)

28.4 (126.0)

37.3 (201.2)

0.0%

0.20

0.0004

0.06

Diagnostic delay, months

17.7 (17.6)

36.8 (34.6)

46.5 (33.2)

0.0%

< 0.0001

< 0.0001

0.007

MMSE

27.7 (2.8)

24.1 (5.7)

24.3 (4.9)

6.3%

< 0.0001

< 0.0001

0.87

Genetic pathogenic variantsb

n = 99

n = 45

n = 57

2.9%

C9orf72

7 (7.1%)

10 (22.2%)

31 (54.4%)

 

0.009

< 0.0001

0.001

GRN

0 (0.0%)

0 (0.0%)

11 (19.3%)

 

1.00

< 0.0001

0.002

MME

1 (1.0%)

0 (0.0%)

0 (0.0%)

 

1.00

1.00

1.00

TBK1

0 (0.0%)

1 (2.2%)

1 (1.6%)

 

0.31

0.37

1.00

TARDBP

0 (0.0%)

0 (0.0%)

2 (3.5%)

 

1.00

0.13

0.50

FTLD/ALS-TDPc

n = 21

n = 7

n = 27

73.4%

0.0003

< 0.0001

0.13

Type A

1 (4.8%)

1 (14.3%)

14 (51.9%)

Type B/E

3 (14.3%)

6 (85.7%)

9 (33.3%)

Type C

0 (0.0%)

0 (0.0%)

3 (11.1%)

Non-specific

17 (81.0%)

0 (0.0%)

1 (3.7%)

SuStaIn stage

3.3 (5.2)

12.1 (7.7)

15.6 (7.2)

0.0%

< 0.0001

< 0.0001

0.02

  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
  3. ALS amyotrophic lateral sclerosis, ALS–FTD amyotrophic lateral sclerosis-frontotemporal degeneration, bvFTD behavioral variant frontotemporal degeneration, MMSE Mini-Mental Status Examination, FTLD/ALS-TDP frontotemporal lobar degeneration or amyotrophic lateral sclerosis with TDP-43 inclusions
  4. *The percentage of individuals with missing data
  5. aData presented as median (range)
  6. bNumber of individuals for genetic screening
  7. cNumber of individuals receiving neuropathological examination