Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies.

To review the evidence for risk factors of Creutzfeldt-Jakob disease (CJD), we pooled and reanalyzed the raw data of three case-control studies. The pooled data set comprised 178 patients and 333 control subjects. The strength of association between CJD and putative risk factors was assessed by computing the odds ratio as estimate of the relative risk. The risk of CJD was statistically significantly increased for subjects with a family history of CJD (odds ratio = 19.1; 95% CI 1.1 to 348.0). Further, there was a significant association between the risk of CJD and a history of psychotic disease (odds ratio = 9.9; 95% CI 1.1 to 86.1). Although not significantly increased, there was an elevated risk of CJD for subjects with a family history of dementia, a history of poliomyelitis, subjects employed as health professionals, and subjects ever exposed to cows and sheep. No association could be shown with organ meat consumption, including brain. The negative results of this reanalysis reassures the absence of a common risk factor in all CJD patients. However, the ongoing epidemiologic surveillance of CJD in several European countries may provide more evidence to exclude any environmental exposure early in childhood.

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.

Recurrent intra-cranial granulomata presenting as space-occupying lesions in a patient with common variable immunodeficiency.

A patient with profound panhypogammaglobulinaemia due to common variable immunodeficiency developed two discrete intra-cranial space-occupying lesions, 10 years apart, requiring craniotomy. Histological examination revealed the intra-cranial masses to be granulomata of unknown aetiology on both occasions.

Serial studies of evoked potentials and circulating lymphocyte subsets for multiple sclerosis: attempts to monitor progress.

A concurrent change in evoked potential measurements and quantitation of circulating T-suppressor (CD8) lymphocyte subpopulations might indicate increased subclinical disease activity. Eight untreated patients with clinically definite multiple sclerosis were monitored monthly for changes in the numbers of cells positive for CD8 markers, and hence in the ratio of CD4: CD8 positive cells. Such changes were found not to be associated with changes in evoked potentials or clinical status.

Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-84.

Two hundred and sixty seven patients with a definite or probable diagnosis of Creutzfeldt-Jakob disease (CJD) were identified in an attempt to ascertain all cases of the disease in England and Wales in the period 1970-84. No evidence of space-time clustering of dates and places of onset was found for these cases. Two age- and sex-matched controls were selected for 72 of the 122 cases diagnosed in the period 1980-84 and life histories of places of residence were obtained for these cases and the controls. No evidence was found that cases had lived closer to each other than had controls at any time in their lives, except that there was an excess of cases born in London. The study does not provide supportive evidence for case-to-case transmission of CJD being an important component in the aetiology of the disease.

Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors.

An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls.

Sensory perineuritis.

A case of sensory perineuritis is described, affecting individual cutaneous nerves in the extremities and with a chronic inflammatory exudate confined to the perineurium in a sural nerve biopsy. No cause was found. The condition slowly resolved on steroid treatment.

A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-1979. II: Epidemiology.

A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-1979, 31 probable cases and 10 possible cases. Descriptive epidemiological data are presented. The average annual incidence was 0.3 cases/million. An unexpected female excess was found. There was no evidence of space-time clustering of cases and no associations with occupation or past medical treatment were apparent. There was statistically significant variation in incidence rates in different parts of the country but no relationship was discovered between incidence and population density.

Multiple sclerosis : A multielement survey.

Levels of 35 elements have been measured in whole blood and ultrasonically washed scalp hair from multiple sclerosis patients and matched control subjects from Oxford, England. Among the significant differences found, the low levels of vanadium and the high levels of barium in the patients were most marked and consistent. Although the etiological significance of the observations is unknown, the low hair-vanadium levels are in qualitative agreement with a previous Canadian report. Hair- and blood-lead levels in the patients and the controls were virtually identical.

A method of calculating spinal cord transit time from potentials evoked by tibial nerve stimulation in normal subjects and in patients with spinal cord disease.

Somatosensory potentials were evoked by stimulation of the tibial nerve at the ankle and recorded over the spine and scalp in 16 normal subjects and 26 patients with known or suspected spinal cord disease, with the aim of developing a method of measuring spinal sensory conduction velocity using a tolerable number of stimuli, applied unilaterally to alert subjects. In normal subjects N21 was consistently recorded over L1 vertebra and in most subjects a complex, N27/N29/P33, was recorded over the cervical spine referred to the vertex. Constant latencies at different spinal levels and, in one subject, comparison with the latency of the ascending volley indicate that the complex was not derived from the spinal cord but from more rostral structures, and therefore only transit time, rather than velocity, could be measured. In patients with clinically definite multiple sclerosis, even with minimal clinical signs, the N27/N29/P33 complex was always abnormal. Abnormalities in this and other forms of spinal cord disease were commonly absence or distortion of the complex, prolonged transit time being rare. The clinical value of the method is limited by the very low amplitude of the responses.

A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-79. I: Clinical features.

A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-79, and 31 probable cases. The clinical features and the results of investigation are described. Three subgroups of patients were distinguished by clinical characteristics and contrasting course.

Prolonged follow-up of abnormal visual evoked potentials in multiple sclerosis: evidence for delayed recovery.

A case is described in which the latency of the P100 component of the pattern evoked visual response (VEP) was shown in serial recordings to have remained greatly prolonged for three years after an attack of optic neuritis, but had returned to normal after a further three and a half years. A similar pattern was found in one of a group of 21 patients re-examined between 6 and 8 years after abnormal VEP had been recorded. The pathophysiological implications are briefly discussed.

Creutzfeldt-Jakob disease. Failure to detect spiroplasmas by cultivation and serological tests.

Specimens from confirmed cases of Creutzfeldt-Jakob disease (CJD) were examined for evidence of spiroplasma infection. No spiroplasmas or other mycoplasmas were cultivated from brain tissue of 18 cases and no antibodies to several recognised Spiroplasma spp. were detected in sera from 15 patients. These negative cultural and serological results provide no support for published suggestions that CJD brain contains structures morphologically resembling spiroplasmas.

The migrant sensory neuritis of Wartenberg.

Six cases are reported that conform to Wartenberg's description of migrant sensory neuritis. This is a benign relapsing and remitting condition in which pain and subsequent loss of sensation in the distribution of individual cutaneous nerves is induced by movement of the limbs inducing stretch. Sural nerve biopsy in one case showed loss of large myelinated fibres, axonal sprouting and some changes suggestive of ischaemia.