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Table 4 Comparison of auxiliary 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
PSWCs on EEG, % 0/29 (0) 40/133 (30.1) 0.001
Positive CSF 14-3-3 protein, % 3/10 (30.0) 31/70 (44.3) 0.393
Elevated CSF tau protein, % 6/10 (60.0) 8/14 (57.1) 0.899
Positive RT-QuIC, % 2/3 (66.7)
Frontotemporal atrophy, % 26/29 (89.7) 51 (100.0) 4 (3.3) 0.044 < 0.001
Hyperintensity on MRI, % 6/24 (25.0) 0 (0) 113 (83.0) < 0.001 < 0.001
Frontotemporal hypoperfusion or hypometabolism, % 10/11 (90.9) 35/36 (97.2) 19/32 (59.4) 0.417 0.054
  1. PSWCs, periodic sharp wave complexes; RT-QuIC, real-time quaking-induced conversion assay
  2. aGPrDs presenting as FTD vs. FTD
  3. bGPrDs presenting as FTD vs. prion diseases