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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