A Glanzmann's thrombasthenia cluster among Iraqi Jews in Israel

A country-wide survey of Glanzmann's thrombasthenia in Israel identified 44 patients. The patients belong to 23 unrelated families, 17 of whom are of Iraqi Jewish origin. The frequency of thrombasthenia among Iraqi Jews in Israel (total population 270,000) is 1:7714 and thus the calculated frequency of the carrier state is 2.3%.     

Genetic risk factors for antiepileptic drug–induced hypersensitivity reactions in Israeli populations

The human leukocyte antigen (HLA) alleles B*15:02 and A*31:01 have been identified as predictive markers of adverse cutaneous effects of carbamazepine and phenytoin in Asian and North European populations, respectively. Our aim was to estimate the distribution of these alleles in Jewish and Arab populations in Israel. The HLA‐B*15:02 and HLA‐A*31:01 carrier rate was estimated based on data from the Hadassah Bone Marrow Registry. Data on Stevens‐Johnson syndrome (SJS)– and toxic epidermal necrolysis (TEN)–related hospitalizations were obtained from the Israeli Ministry of Health (MOH) registries and from four Israeli medical centers. Of 83,705 Jewish and Arab‐Muslim donors, 81 individuals of known origin carried the HLA‐B*15:02. Among them, 66 were Jews of India‐Cochin descent. Of the Cochin Jewish donors, 12.7% were B*15:02 carriers. HLA‐A*31:01 carrier incidence among Arab and Jewish Israeli populations (3.5% and 2.2%, respectively) was within the range reported in other countries. We did not identify SJS‐ or TEN‐related hospitalizations of Jews of Indian descent. Yet, this population should be considered at greater risk for antiepileptic drug–induced SJS and TEN. Until further data on actual risk are available, such patients should be typed for HLA‐B before treatment with carbamazepine or phenytoin.     

Oculopharyngeal MD among Bukhara Jews is due to a founder (GCG)9 mutation in the PABP2 gene

OBJECTIVE: To determine whether all cases of oculopharyngeal muscular dystrophy (OPMD) among Bukhara Jews share the same founder mutation. BACKGROUND: Autosomal dominant OPMD is caused by a (GCG)8-13 repeat expansion in the polyadenylation binding protein 2 (PABP2) gene. The disease has a worldwide distribution but is particularly prevalent in Bukhara Jews and in French Canadians, in whom it was introduced by three sisters in 1648. METHODS: We established the size of the PABP2 mutation in 23 Bukhara Jewish patients belonging to eight unrelated families. In all families, we constructed haplotypes for the carrying chromosomes composed of the alleles for eight chromosome 14q polymorphic markers. RESULTS: All patients share a (GCG)9 PABP2 mutation and a four-marker haplotype. Furthermore, a shared intron single nucleotide polymorphism (SNP) in the PABP2 gene 2.6Kb from the mutation was not observed in 22 families with (GCG)9 mutations from nine different countries. The smaller size of the chromosomal region in linkage disequilibrium around the mutation in Bukhara Jews, as compared with French Canadians, suggests a founder effect that occurred more than 350 years ago. Based on the Luria-Delbrück corrected "genetic clock," we estimate that the mutation appeared or was introduced once in the Bukhara Jewish population between AD 872 and 1512 (mean, AD 1243). CONCLUSION: OPMD among Bukhara Jews is the result of a shared, historically distinct, PABP2 (GCG)9 mutation that likely arose or was introduced in this population at the time they first settled in Bukhara and Samarkand during the 13th or 14th centuries.     

A LRRK2 G2019S mutation carrier from Turkey shares the Japanese haplotype

The leucine-rich repeat kinase 2 (LRRK2) G2019S mutation is recognized as the most common cause of familial autosomal dominant and also sporadic forms of Parkinson disease (PD). A common founder has been described for most Europeans and all North Africans and Jews; besides, two distinct G2019S LRRK2 haplotypes were found in a small proportion of European families and in Japanese PD patients. This study revealed a Turkish patient heterozygous for the G2019S mutation sharing the Japanese haplotype. To the best of our knowledge, it is the first time that the G2019S-associated Japanese haplotype has been reported in a different population.     

Epidemiology of Guillain-Barré syndrome

From 1969 through 1972, a nationwide search for cases of Guillain-Barré syndrome (GBS) is Israel revealed 89 patients. The average annual age-adjusted incidence was 0.75 per 10(5) persons. Overall incidence of the syndrome was similar in Jewish groups of diverse ethnic backgrounds. Arabs had a lower overall incidence than Jews (0.46 per 10(5) persons), perhaps attributable to fewer Arabs at risk in older age groups. Peaks of incidence occurred among individuals over 60 and under 4 years of age when all cases were combined. No clear seasonal or geographic clustering of GBS was evident in Israel during the 4 years of this study. The incidence of GBS in the present study agrees with previous population-based estimates.     

Dystonia gene in Ashkenazi Jewish population is located on chromosome 9q32-34

Idiopathic torsion dystonia (ITD) is a neurological disorder characterized by sustained muscle contractions that appear as twisting movements of the limbs, trunk, and/or neck, which can progress to abnormal postures. Most familial forms of ITD follow autosomal dominant transmission with reduced penetrance. The frequency of ITD in the Ashkenazi Jewish population is five to ten times greater than that in other groups. Recently, a gene for ITD (DYT1) in a non-Jewish kindred was located on chromosome 9q32-34, with tight linkage to the gene encoding gelsolin (GSN). In the present study linkage analysis using DNA polymorphisms is used to locate a gene responsible for susceptibility to ITD in 12 Ashkenazi Jewish families. This dystonia gene exhibits close linkage with the gene encoding argininosuccinate synthetase (ASS), and appears by multipoint analysis to lie in the q32-34 region of chromosome 9, a region that also contains the loci for gelsolin and dopamine-beta-hydroxylase. The same gene may be responsible for ITD both in the non-Jewish kindred mentioned above and in the Ashkenazi Jewish families presented here. However, because there is substantial difference between the penetrance of the dominant allele in these two groups, two different mutations may be operating to produce susceptibility to this disease in the two groups.     

Aseptic meningitis: Frequency among Israeli ethnic groups

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.     

The Libyan Creutzfeldt-Jakob disease focus in Israel: an epidemiologic evaluation

In a country-wide study of Creutzfeldt-Jakob disease (CJD) in Israel, we diagnosed 114 cases, among them 49 Libyan-born, with onset of their disease during the years 1963-1987. After age adjustment, the mean annual incidence rate per million population was 43 among Libyan-born and 0.9 in the rest of the population. Among Jews born in Egypt and Tunisia, neighboring countries of Libya, the adjusted rates were higher than in the other Israelis (3.5 and 2.3 per million, respectively). Among Libyan Jews, there was no association between incidence rate of CJD and age at immigration, ie, duration of exposure to hypothetical infectious factor in Libya. The percent of familial cases among Libyan Jews (41 to 47%) is one of the highest ever published. Genetic factors seem to be important for the high incidence of CJD among Libyan Jews.     

The frequency of multiple sclerosis in jewish and arab populations in greater jerusalem

A comparison of the incidence rate (IR) and the prevalence rate (PR) of multiple sclerosis (MS) in subgroups of the same ethnic origin, but born and living in different geographical areas, may delineate the relationship between environmental and genetic risk factors for MS. Previous epidemiological studies of MS in Israel did not include the Arab population and used diagnostic criteria that did not include MRI findings. Therefore, we studied the age-adjusted IR and PR of MS in a more recent sample in different population groups, including Arabs, of Greater Jerusalem. We found that the PR of MS in Israeli Jews is higher than previously described. Furthermore, the PR was significantly lower among immigrant Jews from Asia/Africa (A/A) than among native-born Jews of Asian/African origin (I-A/A). Since these groups have similar genetic susceptibilities to MS, the higher PR in the latter is probably due to environmental factors. Our study does not support the effect of latitude on the risk of developing MS since no difference in the PR was found between immigrant Jews from Europe/America (E/A) and native-born Jews of European/American origin (I-E/A). Among Arabs, the PR was similar to that among A/A. Therefore, we hypothesized similarity in environmental etiologic factors for MS between the countries of origin of A/A immigrants and of Arabs communities in Greater Jerusalem. The IR of I-E/A was higher than that of I-A/A and Arabs, although this difference did not reach statistical significance.     

Intrafamilial correlation in idiopathic torsion dystonia.

Age of onset and severity of idiopathic torsion dystonia (ITD) were studied in 100 British families containing 107 index cases and 79 secondary cases. Analysis of variance of these clinical features did not suggest that ITD is genetically heterogenous, and they were similar in Jewish and non-Jewish patients. Intrafamilial correlation for age of onset was low, particularly between parents and their offspring, suggesting that the ITD phenotype may be determined in part by nongenetic factors or an allelic modifying gene.     

Compliance with home rehabilitation therapy by parents of children with disabilities in Jews and Bedouin in Israel

Among key points in making progress and succeeding with a therapeutic programme for children with disabilities is parental compliance with the regime for their child. The purpose of this study was to evaluate factors influencing compliance with home therapy in the Jewish and Bedouin populations. Data were collected by structured questionnaires. A total of 193 families participated (84% response rate) with children who ranged in age from 6 months to 6 years (mean age at first visit to the centre was 9.5 years in Jews and 16.1 years in Bedouin). Compliance was significantly lower among the Bedouin. Multivariate regression analysis showed that the strongest contributory factor in lack of compliance was being Bedouin. The second factor was intensity of questioning destiny, indicating that parents with these feelings may be less likely to comply with therapeutic regimes. Other factors which were associated with compliance were parents' education and socioeconomic status: lower levels on these dimensions corresponded with lower parental compliance. These results were illuminated by a trial intervention programme for Bedouin families which involved telephone contact, translation facilities, and detailed explanations during visits to the centre. Intervention increased the compliance rate of the Bedouin appointments with specialists to 76% (91 of 120 appointments) thereby reaching similar levels to those of the Jewish group. These preliminary results indicate that the strong association between non-compliance and being Bedouin may be due to factors of communication, and that the Bedouin are receptive to therapeutic interventions when communicated in their own language.     

Epidemiology of suicide in Israel: a nationwide population study

BACKGROUND: In Israel, some population characteristics and lifestyle patterns make epidemiological research on suicide of particular interest. The Israeli population is characterized by ethnic diversity, with a multi-religious, multi-national mosaic of Jews and Arabs. These subgroups also vary in their suicidal behavior. The aims of the present study were to examine the trends in suicide incidence rates in Israel from 1984 to 1994, to identify sub-populations at high risk for suicide and to identify suicide methods associated with increased risk. METHODS: Suicide rate trends in Israel over the period from 1984 to 1994 were examined for four subgroups: Jewish men and women, and Arab men and women. Cases of undetermined external cause (UEC) of death were also considered. Mortality data were obtained from the computerized data files of the Israeli Central Bureau of Statistics, which includes cause of death and sociodemographic variables. Suicide was classified according to the ICD-9 codes. RESULTS: Suicide rates were higher for Jews than for Arabs, and higher for men than for women. The rates among both population and sex groups increased directly with age. A significant increase over the years studied was found for Jewish men, particularly in the 18- to 21-year-old age group. An increase in the use of firearms was noted, mainly in the groups in which total suicide rates increased. CONCLUSIONS: The findings of this study highlight the need for further studies to identify both sub-populations at high risk for suicide, and societal trends such as lifestyles, immigration, military service, and media exposure to violence, as first steps toward planning of intervention programs to reduce suicide rates.     

Incidence and prevalence of amyotrophic lateral sclerosis in Harris County, Texas, 1985-1988

The incidence of amyotrophic lateral sclerosis was determined in Harris County, Texas, for the period 1985 through 1988. Amyotrophic lateral sclerosis cases were ascertained from four sources: area neurologists, hospitals, death certificates, and the Muscular Dystrophy Association. The age-adjusted incidences of 1.27 per 100,000 person-years in males and 1.03 per 100,000 person-years in females were lower than recent rates in the northern US, Canadian, and northern European studies but higher than rates in southern European studies. Comparisons with other recent incidence studies show less uniformity in occurrence of amyotrophic lateral sclerosis, in both the overall rates and in the age- and sex-specific patterns, than was suggested by mortality studies. The incidence of amyotrophic lateral sclerosis among blacks and whites was similar. Hispanic males had incidences similar to white males, although a deficit of female Hispanic cases was found in Harris County. The prevalence of amyotrophic lateral sclerosis peaked in the 65- to 74-year age group at 33 per 100,000 population among males and 19 per 100,000 population for females.     

Hypersexual behavior in hasidic jewish inpatients

Although no published studies explore rates of sexual symptoms among mentally ill Hasidic Jews, anecdotal observations led to the lead author's hypothesis that male Hasidic Jewish psychiatric inpatients often demonstrated hypersexual behavior. We examined whether male Hasidic Jewish psychiatric inpatients had higher reported rates of hypersexual behavior than controls. We reviewed charts of all Hasidic Jews admitted in 1998 to one inpatient psychiatric hospital and age/diagnosis/gender-matched comparisons for evidence of reported hypersexual behavior. Sixty-nine percent of male Hasidic patients and 40% of female Hasidic patients had staff reports of hypersexual behavior compared with 19% of male and 20% of female comparisons. There was statistically significant discordance between male (p <.04) but not female pairs. Increased rates of reported hypersexual behavior in Hasidic male inpatients suggest a link between culture and symptom presentation, which further studies of this unique population group should explore.     

The Relationship Between Background Characteristics and Death Anxiety in Times of War: A Comparison Between Three Generations Arab and Jewish Families in Israel

(a) To compare the levels of death anxiety between the Jewish and Arab population in Israel after the Second Lebanon War and the Casting Lead Operation. (b) To compare the levels of death anxiety between three families’ generations: elderly parents, their adult children and their adult grandchildren and (c) to learn about the relationship between background characteristics and death anxiety in times of war. The sample included 172 trios of elderly parents (ages 65 and up), their middle aged children (ages 41–64) and, their young adult children (ages 20–40) living at the northern and southern of Israel. Three quarters of the participants were Jews and the others were Arabs (including Muslims, Christians and Druze). Participants answered a self-report questioner. Death anxiety levels were measured by the Carmel and Mutran’s instrument (1999). In general, the highest levels of death anxiety were found among the elderly parents (p?<?0.01). Regarding the nationality and the gender variables, the highest levels of death anxiety were found among the adult Jewish daughters’ group (p?<?0.001). Nationality was found to be the most contributive variable for predicting death anxiety levels among the three generations; the sense of mastery was the second contributor, to predict death anxiety levels among the three generations’ participants. Elderly population, Arab population and women, are at high risk to suffer from high levels of death anxiety in times of war in Israel. Special attention should be given to this population groups.     

Incidence and prevalence of motor neuron disease in Coastal and Mountainous Regions,Croatia, 1984–1993. A preliminary survey

In the period 1984–1993, we carried out an incidence and prevalence study of motor neuron disease in Coastal and Mountainous Regions, Croatia. This was the first epidemiological study on motor neuron disease in Croatia. A total of 22 incident cases were identified. The crude annual incidence rate was 0.68 (95% confidence interval 0.43-1.03) per 100,000 person-years; 0.95 (95% confidence interval 0.60-1.43) per 100,000 person-years for men and 0.42 (95% confidence interval 0.26-0.63) per 100,000 person-years for women. The rate adjusted to the European population was 0.63 (95% confidence interval 0.39-0.95) per 100,000 person-years. The age-adjusted incidence in our population is similar to the rates, standardized to the European population, observed in Italy, but lower than recent rates in the US and northern European studies. On 31st December 1993, there were 18 prevalent cases. The prevalence adjusted to the European population was 5.24 (95% confidence interval 3.10-8.28) per 100,000 population.     

Neurocysticercosis in Western Europe: a re-emerging disease?

The objective of the study was to estimate the magnitude of neurocysticercosis in Western Europe and to determine the pattern of disease expression in the region. Review of patients with neurocysticercosis diagnosed in Western Europe from 1970 to 2011. Abstracted data included: demographic profile, clinical manifestations, form of neurocysticercosis, and whether the disease occurred in immigrants, European international travelers, or Europeans who had never been abroad. A total of 779 patients were found. Of these, only 28 were diagnosed before 1985. Countries with more reported patients were Portugal (n?=?384), Spain (n?=?228), France (n?=?80), The United Kingdom (n?=?26), and Italy (n?=?21). Information on citizenship status, clinical manifestations, and forms of the disease was available in only 30?C40?% of patients. Immigrants accounted for 53?% of cases, European travelers for 8?%, and non-traveler Europeans for 39?%. Immigrants/European travelers were most often diagnosed during the new Millennium, presented most often with seizures, and had less frequently inactive (calcified) neurocysticercosis than non-traveler Europeans. The prevalence of neurocysticercosis in Western Europe may be on the rise. The pattern of disease expression is different among immigrants/European travelers than among non-traveler Europeans. It is possible that some patients had acquired the disease as the result of contact with Taenia solium carriers coming from endemic countries. Much remains to be learned on the prevalence of neurocysticercosis in this region.     

Distinct Pattern Of Age-Specific Incidence Of Guillain-Barre Syndrome In Harbin, China

We describe the age- and sex-specific incidence of Guillain-Barré syndrome (GBS) in Harbin, China, based on the information from a prospective survey among a well-defined large population during one year. A network of physicians reported incident patients with a GBS diagnosis from a general population of 5.4 million inhabitants in Harbin, China, during the period from 1 October 1997 to 30 September 1998. Each reported patient was examined by senior neurologists and the GBS diagnosis was validated according to standard diagnostic criteria. All GBS patients were followed-up for six months after onset. Admission registers at all hospitals in Harbin were also checked afterwards for screening patients with a GBS diagnosis who might have been missed. During the study period, 79 patients with a GBS diagnosis were reported. After validation, the GBS diagnosis was confirmed in 70 patients. Another GBS patient was found through the screening of admission registers at hospitals. Among them, 36 GBS patients were residents in Harbin and the other 35 patients were from geographical areas out of Harbin. The GBS incidence, age-adjusted to the European standard population, was 0.66 (95% CI 0.46-0.91) per 100,000 person-years, with a male to female ratio of 1.4. The highest GBS incidence was found in the youngest age-group and the incidence among the elderly was remarkably lower than those reported from other populations in Western countries. Possible explanations for the distinct pattern of age-specific incidence of GBS are discussed. Further studies are needed.     

Distribution of treated mental illness in the neighborhoods of Jerusalem

The authors compared the rates of treated mental illness among ethnically advantaged Ashkenazic Jews and ethnically disadvantaged Sephardic Jews in four areas of Jerusalem defined by socioeconomic status. They found that the rates of illness were higher in areas of lower socioeconomic status, that the Ashkenazim had higher rates of illness than the Sephardim in the areas of lower status, and that there was a comparatively high rate of illness among the Ashkenazim in the very religious Jewish neighborhoods. They interpret these findings on the basis of the theories of social causation and social selection, including genetic transmission.