Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK.

Between 1970 and 2003, seven cases of human dura mater-associated Creutzfeldt-Jakob disease (CJD) were identified in the UK. Furthermore, we identified a case of CJD in a porcine dura graft recipient. The mean incubation period of the human dura mater cases was 93 (range 45-177) months. The clinico-pathological features of the cases are described and compared with cases previously reported in the world literature.

Audit of tuberculosis contact tracing procedures in south Gwent.

A survey of part of the work of the tuberculosis contact clinic over a 5-yr period (1984-1988 inclusive) is presented. There were 106 notified index cases and 781 contacts were screened. Following chest radiography, Mantoux tests and BCG vaccinations, only four contacts who were tuberculin-positive required chemoprophylaxis. One asymptomatic adult casual contact was treated as an active case because of shadowing on a chest radiograph. Two other cases of active tuberculosis came to light but not through the routine contact tracing procedures. All cases given treatment were contacts of a single index case and the family case history is described. Because of experience with this family the contact tracing procedures will continue locally but will be monitored prospectively. In view of different findings in other centres it is suggested a further national survey is indicated with special attention to regional variations.

Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970-96.

OBJECTIVE: To identify changes in the occurrence of Creutzfeldt-Jakob disease that might be related to the epidemic of bovine spongiform encephalopathy. DESIGN: Epidemiological surveillance of the United Kingdom population for Creutzfeldt-Jakob disease based on (a) referral of suspected cases by neurologists, neuropathologists, and neurophysiologists and (b) death certificates. SETTING: England and Wales during 1970-84, and whole of the United Kingdom during 1985-96. SUBJECTS: All 662 patients identified as sporadic cases of Creutzfeldt-Jakob disease. MAIN OUTCOME MEASURES: Age distribution of patients, age specific time trends of disease, occupational exposure to cattle, potential exposure to causative agent of bovine spongiform encephalopathy. RESULTS: During 1970-96 there was an increase in the number of sporadic cases of Creutzfeldt-Jakob disease recorded yearly in England and Wales. The greatest increase was among people aged over 70. There was a statistically significant excess of cases among dairy farm workers and their spouses and among people at increased risk of contact with live cattle infected with bovine spongiform encephalopathy. During 1994-6 there were six deaths from sporadic Creutzfeldt-Jakob disease in the United Kingdom in patients aged under 30. CONCLUSIONS: The increase in the incidence of sporadic Creutzfeldt-Jakob disease and the high incidence in dairy farmers in the United Kingdom may be unrelated to bovine spongiform encephalopathy. The most striking change in the pattern of Creutzfeldt-Jakob disease in the United Kingdom after the epidemic of bovine spongiform encephalopathy is provided by the incidence in a group of exceptionally young patients with a consistent and unusual neuropathological profile. The outcome of mouse transmission studies and the future incidence of the disease in the United Kingdom and elsewhere, will be important in judging whether the agent causing bovine spongiform encephalopathy has infected humans.

Transmissible spongiform encephalopathies and human neurodegenerative disease.

There has been a tremendous amount of publicity concerning the transmissible spongiform encephalopathies, and this has led to speculation regarding the possible risk to humans following the emergence of new forms in cattle and cats. We review the data relating to these diseases in the light of new developments, particularly in the fields of molecular biology and epidemiology.

Guillain-Barré syndrome associated with rubella.

A 34 year old male patient with Guillain-Barré syndrome following rubella is described. Diagnosis of the infection was made by the detection of specific IgM and IgG antibody. This is a rare association and has not been previously reported in an adult without a characteristic rash.

Chickenpox pneumonia: an association with pregnancy.

Three pregnant patients with chickenpox pneumonia are described. A review of reports published since 1965 together with these cases shows that 28 of the 46 women (61%) were pregnant, and 21 were in the last trimester. Although the disease is more common and more severe in pregnancy, the proportion of deaths was no greater than usual. In this retrospective analysis there was no evidence that treatment with acyclovir conferred benefit but this may have reflected its use only in very ill patients.

A neurological evaluation of adult phenylketonuria in Northern Ireland.

To investigate the extent of neurological disease in adults with phenylketonuria and also their requirement for specialist medical services, we assessed 27 such patients in Northern Ireland. Most had been diagnosed early and with one exception had dietary relaxation by early adolescence. Abnormal neurological features were elicited in 21/27 cases with significant delay of visual evoked potentials and somatosensory evoked potentials occurring in 63.6 and 13.6% of cases respectively. Periventricular white matter abnormalities were observed in 5/12 patients undergoing magnetic resonance imaging, whilst phenylalanine levels were high (mean 1,226 micromol/l). These data accord with the observation that even early treated patients with phenylketonuria can have significant neurological morbidity in adulthood, although the correlation with current or recent dietary control is unclear and requires further study.

Creutzfeldt-Jakob disease and blood transfusion.

Epidemiological surveillance of Creutzfeldt-Jakob disease (CJD) in the UK identified 21 patients who had received a blood transfusion and 29 who had donated blood, out of a total of 202 definite and probable cases. This frequency of blood transfusion or donation did not differ from that in age and sex matched controls, and the clinical features in patients with a history of blood transfusion were similar to those of classical CJD and clearly distinct from CJD in recipients of human growth hormone. This evidence does not suggest that blood transfusion is a major risk factor for CJD.

A case-control study of sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of clustering.

BACKGROUND: The authors investigated whether cases of sporadic Creutzfeldt-Jakob disease (CJD) had lived closer to one another at some time in life than individuals without sporadic CJD. Such a phenomenon would be compatible with some cases resulting from transmission. METHODS: UK sporadic CJD cases occurring from 1990 to 1998 were identified. Age-, sex- and hospital-matched controls were recruited. Lifetime residential histories were obtained by interview, usually with a proxy respondent. With use of Monte Carlo simulation, the residential proximity of cases during various time periods was compared with that expected in the absence of any clustering, using the information collected on the controls. RESULTS: Two hundred twenty sporadic CJD disease cases and 220 controls were included. Cases lived closer together than might be expected in the absence of any disease-clustering mechanism. This evidence became stronger as the critical period during which residential proximity was required to have occurred extended further into the past. CONCLUSIONS: These findings are consistent with some sporadic Creutzfeldt-Jakob disease (CJD) cases resulting from exposure to a common external factor. The rarity of sporadic CJD suggests that repeated point-source outbreaks of infection are more likely to explain our observations than direct case-to-case transmission. Identifying sources of such outbreaks many years after the event will be extremely difficult.