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Table 2 Comparison of baseline features between gPrD patients with FTD phenotype and FTD/prion disease

From: Genetic prion diseases presenting as frontotemporal dementia: clinical features and diagnostic challenge

Variables

GPrDs presenting as FTD (N = 49)

FTD (N = 51)

Prion diseases (N = 136)

P valuesa

P valuesb

Genetic mutation

49/49 (100.0)

12 (23.5)

17/83 (20.5)

–

–

Female, %

23/49 (46.9)

24 (47.0)

64 (47.1)

0.990

0.988

Age at onset, years, median (IQR)

45.0 (39.5, 53.5)

61.0 (54.0, 67.0)

60.0 (53.0,65.8)

< 0.001

< 0.001

Symptoms duration, years, median (IQR)

5.0 (2.8, 7.0)

–

1.1 (0.5,1.9)

–

< 0.001

Family history, %

40/49 (81.6)

14 (27.5)

18 (13.2)

< 0.001

< 0.001

bvFTD, %

37/49 (75.5)

43 (84.3)

–

0.271

–

PPA, %

9/49 (18.4)

6 (11.7)

–

0.355

–

FTD-ALS, %

2/49 (4.1)

2 (3.9)

–

1.000

–

  1. bvFTD, behavioral variant-frontotemporal dementia; FTD-ALS, frontotemporal dementia-amyotrophic lateral sclerosis; PPA, primary progressive aphasia
  2. aGPrDs presenting as FTD vs. FTD
  3. bGPrDs presenting as FTD vs. prion diseases