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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