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Table 3 Comparison of clinical 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
Clinical features of FTD
 Disinhibition, % 23/40 (57.5) 26 (51.0) 11 (8.1) 0.536 < 0.001
 Apathy, % 27/42 (64.3) 35 (68.7) 28 (20.6) 0.658 < 0.001
 Loss of empathy, % 8/40 (20.0) 28 (54.9) 15 (11.0) 0.001 0.139
 Stereotyped/perseverative behavior, % 16/40 (40.0) 33 (64.7) 10 (7.4) 0.019 < 0.001
 Alterations in food preferences, % 10/40 (25.0) 10 (19.6) 5 (3.7) 0.538 < 0.001
 Executive deficits, % 23/40 (57.5) 27 (52.9) 23 (16.9) 0.664 < 0.001
 Speech disorders, % 23/29 (79.3) 27 (52.9) 84 (61.8) 0.019 0.072
Clinical features of prion diseases
 Cognitive dysfunction, % 38/41 (92.7) 45 (88.2) 104 (76.5) 0.475 0.022
 Parkinsonism, % 28/44 (63.7) 5 (9.8) 63 (46.3) < 0.001 0.046
 Pyramidal signs, % 9/23 (39.1) 4 (7.8) 70 (51.5) 0.001 0.274
 Mutism, % 14/39 (35.9) 0 14 (10.3) < 0.001 < 0.001
 Visual signs, % 5/21 (23.8) 6 (11.8) 48 (35.3) 0.197 0.300
 Seizure, % 8/31 (25.8) 0 9 (6.6) < 0.001 0.001
 Cerebellar signs, % 7/31 (22.6) 5 (5.9) 73 (53.7) 0.112 0.003
 Myoclonus, % 9/40 (22.5) 0 56 (41.2) < 0.001 0.031
  1. aGPrDs presenting as FTD vs. FTD
  2. bGPrDs presenting as FTD vs. prion diseases