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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