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From: TSS ()
Date: December 4, 2006 at 8:47 am PST




2.2 Sporadic Creutzfeldt-Jakob Disease

Between 1st January 1970 and 31st December 2005, 1228 cases of sporadic CJD were identified in

the UK, of which 9 cases were alive on 31st December 2005. Two further cases were identified in

Jersey but they were not included in the following UK analyses. Of these UK cases, 925 (75%)

were classified as definite cases with the remainder classed as probable. Figure 2a shows the

number of deaths each year from sporadic CJD for the UK between 1985 and 2005, Figure 2b

shows similar data for England and Wales between 1970 and 2005 and Figure 2c shows the

number of deaths from sporadic CJD in Scotland and Northern Ireland between 1985 and 2005.

In England and Wales the number of deaths identified each year increased from an average of

about 10 per year at the beginning of the 1970s, and rising from about 30 to 50 per year in the

1990s. A similar phenomenon has been observed in other European countries and this probably

largely reflects improved case ascertainment. Over the shorter time period for which data are

available for Scotland and Northern Ireland there is no clear secular trend. Over the period 1990-

2005 the average crude annual mortality rates from sporadic CJD per million population were 0.89

in England, 1.01 in Wales, 0.88 in Scotland and 0.49 in Northern Ireland (Table 1) . When account

is taken of age and sex, the variation in recorded mortality between the different countries is not

statistically significant (p > 0.5).


2.3 Variant Creutzfeldt-Jakob Disease

Up to 31st December 2005, 159 cases of definite or probable vCJD had been identified in the UK

(110 definite, 43 probable who did not undergo post mortem and 6 probable cases still alive).

Seventy-one (45%) of the 159 cases were women. The median age at onset of disease was 26 years

and the median age at death 28 years (compared with 66 years for the median age at onset and 67

years for the median age at death for sporadic CJD). The youngest case was aged 12 years at onset

while the oldest case was aged 74 years. To date, no case of vCJD has been identified in the UK

in individuals born after 1989. The age- and sex-specific mortality rates for vCJD over the time

period 1 May 1995 to 31 December 2005 are shown in Figure 6. The median duration of illness

from the onset of first symptoms to death was 14 months (range 6-39). The median duration of

illness for cases of sporadic CJD was 4 months (range 1 to 74) during the period 1990-2005.


2.4 Iatrogenic Creutzfeldt-Jakob disease

Since 1970, up to 31st December 2005, 58 cases of CJD attributable to iatrogenic exposure have

been identified, 7 in individuals receiving dura mater implants, 50 in individuals who had received

human-derived growth hormone (hGH) and one in a recipient of human gonadotrophin (hGN).

Fifty-six of these individuals have died (Figure 12) and 2 were still alive as at 31st December 2005.

The mean age at death of the hGH/hGN group was 31 years (with a range of 20-46 years) and for

the dura mater cases 42 years (range 27-59 years).

The first identified iatrogenic case was a dura mater recipient who died in 1979. The first hGHrelated

death occurred in 1985. Since 1985 in the UK, human pituitary-derived hormones have

been replaced by synthetic preparations.

A study of the accumulated UK experience with dura mater-related CJD was undertaken and will

be published in 2006.

2.5 Transfusion Medicine Epidemiology Review

The Transfusion Medicine Epidemiology Review (TMER) is a collaborative project between the

UK NCJDSU and UK Blood Services (UKBS). The main purpose is to investigate whether there

is any evidence that CJD or vCJD may have been transmitted via the blood supply.


vCJD cases (definite and probables) are notified to the UKBS by NCJDSU; a search establishes

whether any have acted as donors. Donation records are checked and all components traced

through hospital records. Details of all identified recipients are forwarded to NCJDSU for

subsequent checking.

In the reverse procedure, patients with vCJD reported to have received blood transfusions are

identified by NCJDSU and notified to UKBS. Details of transfusions are traced through hospital

records and relevant blood donors identified. The identity of donors is notified to NCJDSU for

subsequent checking.


The following results are based on vCJD cases who donated or received blood and does not

include data on the ongoing study of sporadic CJD.

Thirty-one vCJD cases were reported to have been blood donors. Four additional cases who were

not reported to have been blood donors were found to be registered with UKBTS. One of these

cases was found to have been a blood donor while the other three cases were registered as a donor

but never made any donations. Twenty-four of the cases have been traced at blood centres,

including the four additional cases mentioned above. Components derived from donations made

by 18 of these individuals were actually issued to hospitals. It has been established that 66

components were transfused to identified recipients. One of these recipients was identified as

developing symptoms of vCJD 6˝ years after receiving a transfusion of red cells donated 3˝

years before the donor developed symptoms of vCJD1. In a further recipient who died from a

non-neurological disorder 5 years after receiving blood from a donor who subsequently developed

vCJD, protease-resistant prion protein (PrPres) was detected in the spleen but not in the brain.

This is the first recorded case in the UK of autopsy detection of presumed preclinical or

subclinical vCJD infection2. In early 2006, a further case of probable vCJD was identified in a

recipient who developed symptoms of vCJD 7 years, 10 months after receiving a transfusion of

red cells from a donor who developed symptoms of vCJD nearly 21 months after donation3. The

diagnosis of vCJD in this donor was confirmed neuropathologically.

In the reverse study, 11 vCJD cases were reported to have received blood transfusions in the past.

A further case received a blood transfusion after onset of illness and is excluded from further

discussion. Checks revealed that of these 11 cases, 2 were not transfused, 2 had transfusions

which pre-dated available records and 7 had records of transfusion which could be traced. These

7 individuals had received 125 components of blood (with one patient given 103 components),

which have been traced to 121 named donors (two of whom had vCJD as described above). The

donors of four components are not traceable.


These findings strongly suggest that vCJD may be transmitted by blood transfusion. The

identification of a second case of vCJD in this small cohort of known recipients of blood from

persons incubating vCJD establishes beyond reasonable doubt that blood transfusion is a

transmission route.


2.6 Study of Progressive Intellectual & Neurological

Deterioration (PIND)

The aim of this project is to use the mechanism of the British Paediatric Surveillance Unit to

identify all cases of progressive intellectual and neurological deterioration in children in the

UK, particularly those with features suggestive of vCJD. All cases are discussed by an Expert

Neurological Advisory Group of seven paediatric neurologists which allocates the cases to a

diagnostic category4-5.

As at 31st December 2005, after almost 9 years surveillance, 2003 patients with suspected

PIND have been reported. The Expert Group has discussed 1415 cases, of which 842 have a

confirmed underlying cause other than vCJD, being categorised into 114 known

neurodegenerative diseases. Among them were six cases of vCJD; four definite and two

probable. Three were reported in 1999, one in 2000 and 2 in mid-2001. One girl was aged

12 at onset - the youngest case of vCJD identified to date.

snip...full text some 40 pages ;


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