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Table 1 Cases included in the present study

From: Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

Case

Age (years)

Gender

Neuropathological diagnosis

Mutation in PRNP or other gene

Codon 129 genotype

PrP type

Braak stage NFTa

Disease duration (months)

PMI (hours)

Cause of death

1

51

F

Ctrl

-

-

-

-

-

<24

Traffic accident

2

66

F

Ctrl

-

-

-

-

-

<48

Haemorrhagic shock

3

82

F

Ctrl

-

-

-

-

-

NA

Myocardial infarct

4

52

M

Ctrl

-

-

-

-

-

<24

Suicide

5

70

M

Ctrl

-

-

-

-

-

<48

Asphyxia

6

60

F

Ctrl

-

-

-

-

-

7.30

Infection

7

60

F

Ctrl

-

-

-

-

-

6.50

Metastasized mamma carcinoma

8

55

M

Ctrl

-

-

-

-

-

7.30

Euthanasia with oesophageal carcinoma

9

57

F

Ctrl

-

-

-

-

-

7.40

Metastasized bladder carcinoma

10

59

M

GSS

7-OPRI [59]

MV

1

NA

7

NA

Cerebral pathology

11

57

M

GSS

7-OPRI [59]

VV

1

I

65

<48

Cerebral pathology

12

42

F

GSS

5-OPRI [60]

MM

1/2

0

92

<24

Cerebral pathology

13

52

M

GSS

G131V [61]

MV

Not 1 or 2

III

192

5.45

Cerebral pathology

14

45

F

GSS

Q227X [39]

MV

Not 1 or 2

VI

72

<6

Cerebral pathology

15

57

F

PrP-CAA

Y226X [39] and D178N

MV

NA

0

27

NA

Cerebral pathology

16

36

F

FFI

D178M

MM

2

NA

48

<24

Cerebral pathology

17

61

M

FFI

D178N

MM

2

III

7

<48

Cerebral pathology

18

58

M

FFI [62]

D178N

MM

2

NA

6

<24

Cerebral pathology

19

16

M

vCJD

-

MM

2

0

9

<144

Cerebral pathology

20

26

F

vCJD [63]

-

MM

2

0

20

<24

Cerebral pathology

21

49

F

vCJD

-

MM

2

NA

15

<48

Cerebral pathology

22

54

M

iCJD

-

MM

1

0

4

<72

Cerebral pathology

23

66

M

iCJD

-

MV

1

NA

9

NA

Cerebral pathology

24

58

M

iCJD

-

MV

1

I

4

<24

Cerebral pathology

25

55

F

sCJD

-

MV

2

0-I

16

<120

Cerebral pathology

26

75

F

sCJD

-

MM

1/2

III-IV

12

NA

Cerebral pathology

27

64

F

sCJD

-

MV

2

0

8

<144

Cerebral pathology

28

61

F

sCJD

-

MV

2

0

20

NA

Cerebral pathology

29

68

F

sCJD

-

MV

2

0

26

<216

Cerebral pathology

30

59

F

sCJD

-

MV

2

0

22

<216

Cerebral pathology

31

52

F

sCJD

-

VV

1

I-II

3

<72

Cerebral pathology

32

60

M

sCJD

-

VV

2

0

6

<48

Cerebral pathology

33

79

F

sCJD

-

MV

2

III

12

<24

Cerebral pathology

34

50

F

sCJD

-

MM/MV

1/2

0

4

<48

Cerebral pathology

35

68

M

sCJD

-

MM/MV

1

0

1

<24

Cerebral pathology

36

81

M

sCJD

-

MM/MV

1

I

2

<24

Cerebral pathology

37

62

F

sCJD

-

MM/MV

1/2

I-II

1

<24

Cerebral pathology

38

62

F

sCJD

-

VV

2

I-II

4

<20

Cerebral pathology

39

60

F

sCJD

-

MV

2

I

20

<5

Cerebral pathology

40

62

M

sCJD

-

MV

2

I-II

16

<24

Cerebral pathology

41

77

F

sCJD

-

VV

2

II

5

<48

Cerebral pathology

42

81

M

sCJD

-

MM

2

I-II

36

<24

Cerebral pathology

43

73

F

sCJD

-

MV

2

I

10

<120

Cerebral pathology

44

70

F

sCJD

-

VV

2

I-II

6

<24

Cerebral pathology

45

73

F

sCJD

-

MM/MV

1/2

0

2

<24

Cerebral pathology

46

57

F

sCJD

-

MM/MV

NA

I

24

<192

Cerebral pathology

47

52

F

sCJD

-

MV

2

0

7

<24

Cerebral pathology

48

67

F

sCJD

-

MM

1/2

0

2

<24

Cerebral pathology

49

82

F

sCJD

-

MM

1

III

2

<96

Cerebral pathology

50

62

M

sCJD

-

VV

2

0

5

<24

Cerebral pathology

51

83

F

sCJD

-

MM

1

II

2

<24

Cerebral pathology

52

59

F

sCJD

-

MV

1/2

I

36

<24

Cerebral pathology

53

76

M

sCJD

-

MV

2

I

4

<24

Cerebral pathology

54

63

F

sCJD (p.enceph.) [64]

-

MV

2

0

36

NA

Cerebral pathology

55

64

F

sCJD (p.enceph.) [64]

-

MM

1

0

12

<96

Cerebral pathology

56

47

M

VPSPr [65]

-

VV

Not 1 or 2

I

20

<120

Cerebral pathology

57

65

F

Sporadic AD

-

-

-

VI

48

<24

Cerebral pathology

58

69

M

Sporadic AD

-

-

-

V-VI

48

<48

Cerebral pathology

59

29

F

Familial AD

S170F in PSEN1

-

-

VI

72

<48

Cerebral pathology

60

83

M

Sporadic AD (Hip)

-

-

-

III

120

<12

Cerebral pathology

61

84

F

Sporadic AD (Hip)

-

-

-

VI

84

<12

Cerebral pathology

  1. For this study the frontal lobe was used unless indicated otherwise
  2. Abbreviations: M Male, F Female, Ctrl Control, GSS Gerstmann–Sträussler–Scheinker syndrome, PrP-CAA PrP-Cerebral amyloid angiopathy, FFI Fatal Familial Insomnia, vCJD Variant CJD, iCJD Iatrogenic CJD, sCJD Sporadic CJD, sCJD (p.enceph.) Sporadic CJD panencephalopathic subtype, VPSPr variably protease-sensitive prionopathy, AD Alzheimer’s disease, OPRI Octapeptide repeat insertion, M Methionine, V Valine, PMI Post-mortem interval, Hip Hippocampal sections used instead of frontal sections, NA Not available
  3. aBraak stage for NFT was used to describe the severity of tau pathology. However, since in prion diseases tau pathology can also be secondary to PrPSc amyloidosis instead of Aβ amyloidosis, this staging does not represent real Braak and Braak classification, but rather an indication of the severity of tau pathology, described as if it were an AD patient. Additionally, tau and Aβ pathology in the frontal cortex were assessed by our own immunohistochemical stainings
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Acta Neuropathologica Communications

ISSN: 2051-5960

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