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In 2009, a pathologist with sporadic Creutzfeldt-Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt Jakob Disease Surveillance network (EuroCJD). Responses from registries in 21 countries revealed that of 8,321 registered cases, 65 physicians or dentists, two of whom were pathologists, and another 137 healthcare workers had been identified with sCJD. Five countries reported 15 physicians and 68 other health professionals among 2,968 controls or non-cases, suggesting no relative excess of sCJD among healthcare professionals. A literature review revealed: (i) 12 case or small case-series reports of 66 health professionals with sCJD, and (ii) five analytical studies on health-related occupation and sCJD, where statistically significant findings were solely observed for persons working at physicians' offices (odds ratio: 4.6 (95 CI: 1.2-17.6)). We conclude that a wide spectrum of medical specialities and health professions are represented in sCJD cases and that the data analysed do not support any overall increased occupational risk for health professionals. Nevertheless, there may be a specific risk in some professions associated with direct contact with high human-infectivity tissue.

There have been few population-based studies of acute transverse myelitis (ATM). Therefore, incidence and population selectivity of this disorder in different regions is not well known. Data on all Jewish patients with ATM throughout Israel were collected for the period 1955 through 1975. Based on 62 patients who satisfied rigid diagnostic criteria, the average annual incidence rate was 1.34 per million population. No significant difference in incidence was noted between European/American-born and Afro/Asian-born populations. There was no significant seasonal or annual fluctuation in frequency. In 37% of the patients, a history of infection prior to ATM was reported, more commonly among younger patients. ATM rarely evolved into multiple sclerosis. More than one-third of the patients with ATM made a good recovery; in another one-third recovery was only fair; 14 patients failed to improve and 3 died. If other population-based studies of ATM were undertaken, comparison with our results might shed further light on the causes of this disorder.

A country-wide search for idiopathic torsion dystonia (ITD) in Israel between 1969 and 1975 revealed 42 patients (41 Jewish and 1 Druze Arab). Prevalence of ITD per million population, age-adjusted to the United States population in 1970, was 10.8 in the total Jewish population (22.0 among Jews of European extraction contrasted with 1.5 among Jews with Afro-Asian forebears). Among Europeans, the highest prevalence was among Jews from Eastern Europe. The average age-adjusted annual incidence rates per million population were 0.43 in the total Jewish population, 0.98 in the Europeans, and 0.11 in the Afro-Asians. Among the 40 patients for whom familial data were available, the majority of cases (26) were sporadic. The other 14 belonged to four unrelated European families, all of Russian-Polish origin. The pattern of inheritance in these four families fits an autosomal dominant model with incomplete penetrance.

Idiopathic torsion dystonia (ITD) has long been considered to be genetically determined, but the pattern of inheritance has been unclear. It has been suggested that inheritance may differ in Jews and non-Jews. In the present study, data gathered in a nationwide survey of ITD in Israel were analysed. Between 1969 and 1980, 47 patients were collected, of whom 40 were of European origin. In these European Jews, the ITD frequency was about 1:23 000 live births, which was five-fold greater than in Jews of Afro-Asian origin. Assuming that all cases fit the same genetic model, an X linked or a simple autosomal recessive model of inheritance did not agree well with our data. An autosomal dominant model with low penetrance could have accounted for our observations and would yield an ITD gene frequency in European Jews of 3 to 4:100 000. In view of the increased ages of their fathers, the isolated cases may have included some new mutations. Multifactorial inheritance was also possible. However, it may be inappropriate to assume that all cases have the same genetic basis, or even that all are inherited.

The relative frequency of aseptic meningoencephalitis (AME) was compared in populations of diverse origin, A countrywide search of Israel during 1969-1970 disclosed 1350 cases who fit strict diagnostic criteria. The average annual incidence was 21.6 per 100000 population. The total incidence was similar in Afro-Asian, Euro-American and Israeli Jewish groups but among Israeli Arabs, the incidence was apparently lower. Age-specific incidence showed a peak in infants under one year of age among Arabs and Afro-Asian Jews whereas Euro-Americans and Israeli Jews had a peak incidence at 5-9 years. Larger family size among Arabs and Afro-Asian Jews might account for the higher incidence in infants. Age-specific incidence may be a better index than total incidence of important differences in AME among various ethnic groups.

A nationwide epidemiologic study of presenile dementia of the Alzheimer type (PDAT) with onset through age 60 years was carried out in Israel. The Israeli National Neurologic Disease Register and clinical records of all patients discharged from hospitals between 1974 and 1983 with a neurologic or psychiatric diagnosis suggestive of dementia were reviewed. A total of 71 Jewish patients with onset of PDAT between 1974 and 1978 was ascertained. The age at onset in these patients ranged from 43 to 60 years. The median survival was 8.1 years, with slightly longer survival if onset occurred before age 55 years, even after correction for expected mortality according to age and sex. The average annual incidence rate per 100,000 population at risk was 2.4 in the population aged 40 through 60 years. Although the incidence rates were slightly greater for women, the difference between the rates by sex was not statistically significant. The age- and sex-adjusted incidence of PDAT per 100,000 population was significantly higher in those born in Europe or America (2.9) than in those born in Africa or Asia (1.4). No significant difference in survival was found between these two groups. The curve of the incidence rates by age for PDAT in Israel is continuous with that for senile dementia of the Alzheimer type collected by similar methods elsewhere, which suggests that one disease process may account for both conditions.

The association of A3, B7, Dw2 and DR2 histocompatibility (HLA) markers with multiple sclerosis (MS) is well established among Northern Europeans and Caucasoids in the United States. We showed previously that A3 and B7 were not increased among Israelis with MS, and in a preliminary study Dw2 as well. An association of A3 and B7 is also lacking in Italians, Jordanian and Japanese MS patients. In Black American MS patients, the B7 frequency is slightly increased but Dw2 is still significantly associated with MS. For the HLA-DR antigen series DR2 is shown to have a stronger association to MS than A3 and B7. Conceivably, this antigen could be associated with MS even in populations where an association with A3 or B7 is lacking. Therefore, a study of HLA-A, B, C, DR and D antigens was carried out in Israel. No significant excess or deficiency of HLA antigens was found in MS. Possible explanations for these results are as follows: (1) the relevant HLA-D alleles in the Jewish population were not detected by the homozygous typing cells (HTCs) used, since they were derived primarily from European sources; (2) in contrast to the Caucasoid populations the genetic factor predisposing for MS is not associated with HLA alleles in the Israeli population; (3) MS is an heterogeneous disease and in Israelis, an environmental factor is sufficient to cause the disease.

Available estimates of the frequency with which a patient with optic neuritis develops multiple sclerosis range from as low as 13% to as high as 87%. In an effort to obtain a better estimate, a nation-wide study of optic neuritis was carried out in Israel. Patients who fulfilled strict diagnostic criteria of optic neuritis were identified and examined periodically. Between 1955 and 1964, 105 patients were found and on the basis of these, the average annual age-adjusted incidence of optic neuritis in Israel was 0.56 per 10(5) population compared to 1.2 per 10(5) cases of multiple sclerosis per year, i.e. optic neuritis was about half as frequent as multiple sclerosis each year. As with multiple sclerosis, optic neuritis was more common in European immigrants to Israel than Afro-Asian immigrants. During a follow-up interval which ranged from 3.3 to 15.6 years (mean 9.5 years), at least 27 of the 105 patients developed multiple sclerosis (28%). A life-table analysis showed that after 10 years 32.3 +/- 5.6% of patients with optic neuritis would develop multiple sclerosis and, after 14 years, about half would develop multiple sclerosis. Risk of dissemination was highest in those who were youngest when optic neuritis developed. Neither sex nor ethnic background influenced risk significantly. Results of the present study support earlier work using life-table methods carried out in Hawaii which also showed that between 29 and 39% of patients with optic neuritis will develop multiple sclerosis within 10 years of onset. The life-table method is a better predictor of prognosis than newer laboratory techniques such as spinal fluid studies of IgG, kappa-lambda light chain ratios and serum/CSF IgG ratios.