University of Edinburgh European Centre for Disease Prevention and Control

Creutzfeldt-Jakob Disease

International Surveillance Network formerly EuroCJD

CJD Surveillance Data 1993-2013

The figures for definite and probable CJD/GSS deaths and annual mortality rates are provided where available by a number of collaborating countries. Figures for the number of definite and probable variant CJD cases worldwide are provided for all cases of variant CJD in the European Union, and through collaborations with other non-EU countries.


Sporadic CJD: Definite and probable cases

 Country 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 Total
Australia 17 10 19 25 20 25 25 28 20 18 22 17 26 35 26 31 29 28 35 25 12 493
Austria 6 9 9 9 6 8 6 9 9 7 15 8 14 18 18 8 16 6 17 23 15 236
Belgium - - - - 10 14 13 9 15 21 19 20 13 16 16 6 10 12 13 7 5 219
Canada - 2 3 13 16 22 27 32 27 31 27 42 42 39 35 48 48 35 46 61 37 633
Cyprus - - 1 0 0 0 0 0 1 0 1 1 0 1 1 3 0 0 0 1 0 10
Czech Republic - - - - - - - 2 8 6 8 11 10 10 11 11 15 13 12 12 16 145
Denmark - - - - 11 5 9 5 6 9 10 7 4 8 6 6 13 6 7 10 13 135
Estonia - - - - - - - - - - - 0 1 0 0 0 0 1 0 0 1 3
Finland - - - - 10 5 7 4 9 10 5 6 13 4 5 13 6 7 9 5 12 130
France 35 45 59 68 80 81 92 88 109 107 108 98 82 124 138 105 114 151 111 105 80 1,980
Germany 21 70 80 76 107 115 103 109 129 107 89 123 115 120 127 120 92 109 109 90 n/a 2,011
Greece - - - - 6 4 7 9 8 11 6 7 5 6 7 6 n/a n/a n/a n/a n/a 82
Hungary - - - - 11 3 5 9 17 7 8 8 8 12 13 14 7 14 8 19 16 179
Iceland - - - - 1 0 0 0 0 0 0 0 0 0 1 0 0 0 n/a n/a n/a 2
Ireland - - - 2 2 6 1 3 5 5 2 7 5 4 3 5 2 1 6 6 3 68
Israel 2 1 3 3 4 5 3 3 4 3 8 8 6 5 5 8 9 9 8 1 n/a 98
Italy 27 33 28 51 47 64 74 60 87 76 79 77 108 95 97 92 107 113 120 108 90 1,633
Latvia - - - - - - - - - 0 0 0 0 0 1 1 0 2 1 1 n/a 6
Netherlands 12 18 8 14 18 17 19 10 14 18 12 20 20 22 15 16 11 28 34 26 26 378
Norway - - - - 6 2 2 4 6 3 6 8 4 4 5 4 5 2 4 469 7 76
Poland - - - - - - - - - 6 12 20 14 5 4 10 30 3 26 n/a n/a 130
Portugal - - - - 5 6 5 5 16 6 11 6 10 5 8 4 6 2 13 12 n/a 120
Slovakia 2 2 2 2 3 3 1 2 2 6 2 6 8 3 4 6 5 3 6 3 4 75
Slovenia 0 1 2 0 0 1 3 1 4 5 2 2 2 7 2 3 2 1 1 6 4 49
Spain 21 17 20 27 31 62 50 45 62 54 57 54 72 67 78 62 64 59 58 49 50 1059
Sweden - - - - 10 14 11 14 12 12 11 15 13 16 11 13 11 14 15 14 n/a 206
Switzerland 8 10 10 10 10 8 9 11 19 18 17 16 10 13 16 14 18 5 15 15 17 269
UK 36 54 35 40 59 64 62 50 58 73 79 50 67 68 64 86 80 85 90 93 101 1,394

  

 Sporadic CJD: Annual mortality rates per million

  Country 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 Mean Rate
Australia 0.96 0.56 1.06 1.37 1.09 1.34 1.33 1.47 1.04 0.92 1.12 0.85 1.29 1.71 1.25 1.46 1.34 1.27 1.57 1.1 0.52 1.17
Austria 0.77 1.15 1.15 1.15 0.77 1.03 0.77 1.12 1.12 0.87 1.86 0.99 1.74 2.23 2.23 0.99 1.98 0.75 2.13 2.88 1.88 1.41
Belgium - - - - 0.97 1.37 1.27 0.88 1.46 2.02 1.83 1.92 1.24 1.52 1.51 0.56 0.93 1.11 1.19 0.64 0.45 1.28
Canada 0.07 0.1 0.44 0.53 0.73 0.89 1.04 0.87 0.99 0.85 1.31 1.3 1.2 1.06 1.44 1.42 1.03 1.35 1.74 1.05 1.17
Cyprus - - 1.48 0 0 0 0 0 1.43 0 1.39 1.37 0 1.30 1.28 3.84 0 0 0 1.16 0 0.7
Czech Republic - - - - - - - 0.19 0.78 0.59 0.78 1.08 0.98 0.97 1.07 1.05 1.43 1.23 1.68 1.14 1.52 1
Denmark - - - - 2.09 0.94 1.69 0.94 1.12 1.68 1.86 1.3 0.74 1.47 1.1 1.1 2.36 1.08 1.26 1.79 2.32 1.46
Estonia - - - - - - - - - - - 0 0.74 0 0 0 0 0.75 0 0 0.75 0.22
Finland - - - - 1.92 0.96 1.35 0.77 1.73 1.92 0.96 1.15 2.5 0.77 0.72 2.5 1.13 1.31 1.68 1.07 2.2 1.45
France 0.59 0.76 1 1.14 1.34 1.35 1.53 1.45 1.79 1.74 1.75 1.57 1.31 1.96 2.17 1.65 1.77 2.34 1.7 1.61 1.21 1.51
Germany 0.44 0.85 1 0.93 1.3 1.4 1.25 1.36 1.6 1.3 1.1 1.5 1.4 1.5 1.5 1.5 1.1 1.3 1.33 1.1 n/a 1.24
Greece - - - - 0.55 0.37 0.64 0.83 0.73 1 0.55 0.64 0.46 0.55 0.63 0.54 n/a n/a n/a n/a n/a 0.62
Hungary - - - - 1.1 0.3 0.5 0.9 1.7 0.7 0.8 0.8 0.8 1.2 1.3 1.4 0.7 1.4 0.8 1.9 1.6 1.05
Iceland - - - - 3.7 0 0 0 0 0 0 0 0 0 3.23 0 0 0 n/a n/a n/a 0.53
Ireland - - - 0.55 0.55 1.62 0.27 0.79 1.3 1.28 0.5 1.73 1.21 0.94 0.71 1.18 0.47 0.24 1.31 1.31 1.53 0.97
Israel 0.37 0.19 0.56 0.49 0.66 0.82 0.49 0.49 0.6 0.45 1.2 1.2 0.9 0.68 0.68 1.1 1.18 1.05 1.05 0.13 n/a 0.71
Italy 0.47 0.58 0.49 0.89 0.83 1.11 1.28 1.04 1.51 1.32 1.37 1.33 1.85 1.62 1.64 1.54 1.78 1.88 2 1.8 1.5 1.33
Latvia - - - - - - - - - 0 0 0 0 0 0.43 0.43 0 0.89 0.45 0.49 n/a 0.22
Netherlands 0.79 1.17 0.52 0.9 1.2 1.13 1.24 0.63 0.88 1.13 0.75 1.22 1.22 1.34 0.92 0.98 0.67 1.69 2.04 1.55 1.54 1.12
Norway - - - - 1.37 0.45 0.45 0.89 1.33 0.66 1.32 1.75 0.87 0.86 1.07 0.85 1.04 0.41 0.81 0.8 1.38 0.96
Poland - - - - - - - - - 0.16 0.31 0.52 0.36 0.13 0.08 0.26 0.86 0.08 0.68 n/a n/a 0.34
Portugal - - - - 0.5 0.6 0.5 0.5 1.6 0.6 1.05 0.57 0.95 0.48 0.76 0.38 0.57 0.19 1.23 1.14 n/a 0.73
Slovakia 0.4 0.4 0.4 0.4 0.6 0.6 0.2 0.4 0.4 1.13 0.4 1.17 1.34 0.57 0.75 1.13 0.92 0.55 1.1 0.55 0.73 0.67
Slovenia 0 0.5 1 0 0 0.5 1.5 0.5 2 2.5 1 1 1 3.5 1 1.5 1 0.5 0.5 3 2 1.23
Spain 0.54 0.43 0.51 0.69 0.79 1.57 1.27 1.14 1.57 1.36 1.44 1.36 1.81 1.53 1.76 1.37 1.4 1.28 1.26 1.06 1.08 1.2
Sweden - - - - 1.13 1.58 1.24 1.58 1.35 1.34 1.23 1.66 1.44 1.76 1.2 1.4 1.18 1.49 1.58 1.47 n/a 1.41
Switzerland 1.15 1.42 1.42 1.41 1.41 1.12 1.26 1.53 2.62 2.46 2.31 2.16 1.34 1.73 2.11 1.82 2.31 0.64 1.89 1.87 2.09 1.72
UK 0.62 0.93 0.6 0.69 1.01 1.09 1.06 0.85 0.98 1.23 1.33 0.84 1.11 1.12 1.05 1.4 1.29 1.35 1.42 1.46 1.59 1.1

n/a = not available

 

All Definite And Probable Cases:  Sporadic, Familial/Genetic, FFI, GSS and Iatrogenic Deaths (excluding vCJD)

Country 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 Total
Australia 20 12 21 28 24 27 28 32 23 20 23 21 28 39 28 34 31 29 37 28 12 545
Austria 6 10 10 11 7 8 8 9 10 12 15 11 15 22 19 9 17 6 20 24 15 264
Belgium  - - - - 11 15 13 9 16 22 20 20 13 16 17 6 11 12 13 7 6 227
Canada - 3 3 13 18 24 32 35 30 35 29 44 44 44 39 49 53 38 50 62 38 683
Cyprus - - 1 0 0 0 0 0 1 0 1 1 0 1 1 3 0 0 1 0 10
Czech Republic - - - - - - - 2 8 8 11 11 11 11 13 13 20 21 13 14 19 175
Denmark - - - 11 5 9 5 6 9 10 7 4 8 6 6 13 6 7 10 13 135
Estonia - - - - - - - - - - - 0 1 0 0 0 0 1 0 0 1 3
Finland - - - - 10 5 7 4 9 10 5 6 13 4 5 13 6 8 9 5 12 131
France 55 60 74 88 92 103 105 105 129 124 127 115 97 137 154 122 132 161 116 115 81 2,292
Germany 21 76 89 85 115 125 112 114 144 113 98 130 130 125 133 126 95 114 113 94 n/a 2,152
Greece - - - - 6 4 7 9 8 11 6 7 5 6 7 6 n/a n/a n/a n/a n/a 82
Hungary - - - - 13 3 9 11 19 12 11 12 13 26 21 18 16 19 19 30 28 280
Iceland - - - - 1 0 0 0 0 0 0 0 0 0 1 0 0 0 n/a n/a n/a 2
Ireland - - - 2 3 6 1 3 6 5 2 7 5 5 3 5 2 1 6 6 3 71
Israel 11 10 11 14 13 14 7 13 19 14 14 20 20 15 14 23 31 24 20 15 n/a 322
Italy 36 41 36 59 61 76 92 90 101 91 90 100 133 134 117 105 121 135 143 130 110 2,001
Latvia - - - - - - - - - - 0 0 0 0 1 1 0 2 1 1 n/a 6
Netherlands 12 19 8 16 18 18 20 10 17 18 13 22 23 26 17 18 13 28 37 29 27 409
Norway - - - - 6 2 2 4 6 3 6 8 4 4 5 4 5 2 4 5 7 77
Poland  - - - - - - - - 6 12 20 14 5 4 10 30 3 26 n/a n/a 130
Portugal         5 6 5 5 18 6 11 6 10 5 8 5 6 2 13 12 n/a 123
Slovakia 5 5 6 6 7 6 7 5 10 10 8 13 15 8 13 13 22 18 21 14 12 224
Slovenia 1 2 0 0 1 3 1 4 5 2 2 2 7 2 3 2 1 1 6 4 49
Spain 23 18 24 31 31 66 59 54 69 65 58 61 77 67 88 72 69 66 66 58 59 1,181
Sweden - - - - 10 14 11 14 12 12 11 15 14 17 11 14 11 15 15 16 n/a 212
Switzerland 8 10 10 10 10 8 9 11 19 18 17 16 10 13 16 14 18 5 15 15 17 269
UK 47 63 44 50 71 72 70 54 68 78 90 58 84 78 76 96 90 95 108 109 111 1,612

   

All CJD (excluding vCJD): Annual Mortality Rates Per Million

 Country 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 Mean Rate
Australia 1.13 0.67 1.17 1.54 1.3 1.45 1.49 1.68 1.19 1.03 1.17 1.05 1.39 1.91 1.34 1.6 1.43 1.32 1.66 1.23 0.52 1.3
Austria 0.77 1.28 1.28 1.41 0.9 1.03 1.03 1.12 1.24 1.49 1.86 1.36 1.86 2.73 2.36 1.12 2.11 0.75 2.5 3 1.88 1.58
Belgium - - 1.07 1.47 1.27 0.87 1.55 2.11 1.93 1.92 1.24 1.52 1.61 0.56 1.02 1.11 1.19 0.64 0.54 1.32
Canada - 0.1 0.1 0.44 0.6 0.8 1.05 1.14 0.97 1.12 0.92 1.38 1.36 1.35 1.18 1.47 1.57 1.12 1.44 1.77 1.08 1.26
Cyprus - 1.48 0 0 0 0 0 1.43 0 1.39 1.37 0 1.3 1.28 3.84 0 0 0 1.16 0 0.7
Czech Republic - 0.19 0.78 0.78 1.08 1.08 1.07 1.07 1.26 1.24 1.91 1.99 1.24 1.33 1.81 1.2
Denmark - 2.09 0.94 1.69 0.94 1.12 1.68 1.86 1.3 0.74 1.47 1.1 1.1 2.36 1.08 1.26 1.79 2.32 1.46
Estonia - - 0 0.74 0 0 0 0 0.75 0 0 0.75 0.22
Finland - 1.92 0.96 1.35 0.77 1.73 1.92 0.96 1.15 2.5 0.77 0.72 2.5 1.13 1.49 1.68 0.93 2.2 1.45
France 0.94 1.02 1.25 1.48 1.54 1.72 1.75 1.74 2.13 2.02 2.05 1.85 1.55 2.17 2.42 1.91 2.05 2.49 1.78 1.76 1.23 1.78
Germany 0.44 0.93 1.09 1.04 1.4 1.52 1.37 1.39 1.8 1.4 1.2 1.6 1.6 1.5 1.6 1.5 1.2 1.4 1.38 1.15 n/a 1.33
Greece - 0.55 0.37 0.64 0.83 0.73 1.01 0.55 0.64 0.46 0.55 0.41 0.54 n/a n/a n/a n/a n/a 0.61
Hungary - 1.3 0.3 0.9 1.1 1.9 1.2 1.1 1.2 1.3 2.6 2.1 1.8 1.6 1.9 1.9 3 2.8 1.65
Iceland - 3.7 0 0 0 0 0 0 0 0 0 3.23 0 0 0 n/a n/a n/a 0.53
Ireland - 0.55 0.82 1.62 0.27 0.79 1.56 1.28 0.50 1.73 1.21 1.18 0.71 1.18 0.47 0.22 1.31 1.31 1.53 1.01
Israel 2.2 1.93 2.06 2.54 2.3 2.41 1.17 2.13 3.04 2.2 2.16 3.02 2.97 2.18 2 3.23 3.95 2.86 1.79 1.97 n/a 2.41
Italy 0.63 0.72 0.63 1.03 1.06 1.32 1.6 1.56 1.75 1.58 1.56 1.73 2.27 2.28 1.98 1.76 2.02 2.25 2.38 2.17 1.83 1.63
Latvia - - - 0 0 0 0 0 0.43 0.43 0 0.89 0.45 0.49 n/a 0.22
Netherlands 0.79 1.24 0.52 1.03 1.2 1.2 1.3 0.63 1.07 1.13 0.81 1.34 1.41 1.59 1.04 1.1 0.79 1.69 2.22 1.73 1.6 1.21
Norway - 1.37 0.45 0.45 0.89 1.33 0.66 1.32 1.75 0.87 0.86 1.07 0.85 1.04 0.41 0.81 1 1.38 0.97
Poland - - - 0.16 0.31 0.52 0.36 0.13 0.08 0.26 0.86 0.08 0.68 n/a n/a 0.34
Portugal - 0.5 0.6 0.5 0.5 1.8 0.6 1.05 0.57 0.95 0.47 0.76 0.47 0.57 0.19 1.23 1.14 n/a 0.74
Slovakia 0.94 0.94 1.13 1.13 1.32 1.13 1.32 0.94 1.88 1.88 1.56 2.45 2.83 1.51 2.45 2.45 4.06 3.32 3.88 2.58 2.22 2
Slovenia 0 0.5 1 0 0.5 1.51 0.5 2 2.5 1 1 1 3.5 1 1.5 1 0.5 0.5 3 2 1.17
Spain 0.59 0.46 0.61 0.79 0.79 1.68 1.5 1.37 1.75 1.64 1.46 1.54 1.94 1.53 1.99 1.59 1.51 1.43 1.43 1.26 1.28 1.35
Sweden - 1.13 1.58 1.24 1.58 1.35 1.34 1.23 1.66 1.55 1.87 1.19 1.51 1.18 1.59 1.58 1.68 n/a 1.44
Switzerland 1.15 1.42 1.42 1.41 1.41 1.12 1.26 1.53 2.62 2.46 2.31 2.16 1.34 1.73 2.11 1.82 2.31 0.64 1.89 1.87 2.09 1.72
UK 0.81 1.09 0.76 0.86 1.22 1.23 1.19 0.92 1.15 1.31 1.51 0.97 1.39 1.29 1.25 1.56 1.46 1.51 1.71 1.71 1.74 1.28

n/a = not available

   

vCJD cases Worldwide (at 28 May 2105)

 Country 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 Alive Total
UK 3 10 10 18 15 28 20 17 18 9 5 5 5 2 3

3

5 0 1 0   177
France 0 1 0 0 0 1 1 3 0 2 6 6 3 0 2  0 0 0 1 1   27
Ireland 0 0 0 0 1 0 0 0 0 0 2 1 0 0 0 0 0 0 0 0   4
Italy 0 0 0 0 0 0 0 0 1 0 0 0 0 0 0 0 1 0 0 0   2
USA 0 0 0 0 0 0 0 0 0 1 0 2 0 0 0 0 0 0 0 1   4
Canada 0 0 0 0 0 0 0 1 0 0 0 0 0 0 0 0 1 0 0 0   2
Saudi Arabia 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1 0 0 0 0   1
Japan 0 0 0 0 0 0 0 0 0 1 0 0 0 0 0 0 0 0 0 0   1
Netherlands 0 0 0 0 0 0 0 0 0 0 1 1 0 1 0 0 0 0 0 0   3
Portugal 0 0 0 0 0 0 0 0 0 0 0 0 1 0 1 0 0 0 0 0   2
Spain 0 0 0 0 0 0 0 0 0 0 1 0 1 2 1 0 0 0 0 0   5
Taiwan 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1 0 0 0 0   1

 

Return to top of page

Return to Homepage