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Table 1 PrP immunoreactivity of the retina in 16 cases of Creutzfeldt- Jakob disease (CJD)

From: PrPres deposition in the retina is a common finding of sporadic, familial and iatrogenic Creutzfeldt-Jakob diseases (CJD)

Case number

AAD (y)

Duration (months)

Sex

Initial symptom

Diagnosis

Codon 129

Western blot analysis of PrPres

OPL

IPL

3F4-irs

12F10-irs

3F4-irs

12F10-irs

Case 1

72

18

F

visual acuity, color agnosia

sCJD

MM

Type 1

1

2

2

2

Case 2

67

51

F

amnesia

sCJD

MM

Type 1

1

2

1

2

Case 3

75

17

F

palilalia

sCJD

MM

Type 1

2

1

2

1

Case 4

74

6

M

metamorphopsia

sCJD

MM

Type 1

1

1

0

1

Case 5

73

2

M

amnesia

sCJD

MM

Type 1

2

2

2

2

Case 6

82

5

F

sensory disturbance

sCJD

MM

Type 1

1

2

1

2

Case 7

62

42

M

loss of motivation

sCJD

MM

Type 1

1

2

1

2

Case 8

68

8

M

visuospatial disturbance

sCJD

MM

Type 1

2

2

2

2

Case 9

85

7

F

communication disturbance

sCJD

MM

Type 1

2

2

2

2

Case 10

67

30

M

amnesia

sCJD

MM

Type 2

1

2

1

2

Case 11

62

10

F

dizziness, ataxia

sCJD

MM

Type 1 + 2

2

2

2

2

Case 12

72

21

F

visual hallucination

sCJD

MM

Type 1 > 2

2

2

2

2

Case 13

94

90

F

hearing disturbance

V180I

MV

Type 2 equivalence

2

2

2

2

Case 14

93

46

F

communication disturbance

V180I

MM

Type 2 equivalence

2

2

2

2

Case 15

70

19

M

dementia

M232R

MM

Type 1 equivalence

2

2

2

2

Case 16

74

7

M

dementia

dCJD

MM

Type 1 equivalence

2

2

2

2

Control 1

95

 

F

 

AD+DLB

  

0

0

0

0

Control 2

69

 

M

 

ALS

  

0

0

0

0

Control 3

53

 

F

 

ALS

  

0

0

0

0

Control 4

61

 

M

 

MSA

  

0

0

0

0

  1. Typing of prion protein was performed on the basis of biochemical analysis of the frontal cortex. Diagnosis was performed on the basis of neuropathology and molecular biology analyses
  2. Semi-quantification: 0 = none, 1 = positive and scattered staining, 2 = positive and consistent staining in both outer and inner plexiform layers
  3. Abbreviations: AAD age at death, M male, F female, sCJD sporadic CJD, dCJD cadaveric dura matter graft CJD, AD Alzheimer’s disease, DLB dementia with Lewy body disease, ALS amyotrophic lateral sclerosis, MSA multiple system atrophy, MM methionine/ methionine, VV valine/valine, −irs -immunoreactive deposits