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1.
Neurology ; 65(3): 478-80, 2005 Aug 09.
Article in English | MEDLINE | ID: mdl-16087922

ABSTRACT

Dementia with ubiquitinated neuronal inclusions has been described only with frontotemporal dementia (FTD). The authors report a patient with progressive FTD accompanied by prominent impairments in visuospatial cognitive functions. Pathology was characterized by ubiquitin-positive intranuclear and cytoplasmic neuronal inclusions. Cortical pathology was widespread and posteriorly accentuated but spared the hippocampal dentate gyrus.


Subject(s)
Agnosia/pathology , Dementia/pathology , Inclusion Bodies/pathology , Neurons/pathology , Agnosia/etiology , Agnosia/physiopathology , Atrophy/etiology , Atrophy/pathology , Atrophy/physiopathology , Cerebral Cortex/pathology , Cerebral Cortex/physiopathology , Cognition Disorders/etiology , Cognition Disorders/pathology , Cognition Disorders/physiopathology , Dementia/metabolism , Dementia/physiopathology , Disease Progression , Fatal Outcome , Humans , Male , Memory Disorders/etiology , Memory Disorders/pathology , Memory Disorders/physiopathology , Middle Aged , Neurons/metabolism , Psychomotor Performance/physiology , Ubiquitin/metabolism
2.
Philos Trans R Soc Lond B Biol Sci ; 343(1306): 385-90, 1994 Mar 29.
Article in English | MEDLINE | ID: mdl-7913755

ABSTRACT

Various mutations in the prion protein (PrP) gene are associated with Creutzfeldt-Jakob disease (CJD), a transmissible fatal neurodegenerative disorder. Among Libyan Jews, CJD is a familial disease with an incidence about 100 times higher than the worldwide population. CJD in this community segregates with a point mutation at codon 200 of the PrP gene which causes the substitution of lysine for glutamate. This mutation was found in all definitely affected individuals and yields a maximum lod score of 4.85. Some healthy elderly mutation carries above 65 years of age were identified, suggesting partial penetrance. Homozygous patients have the same disease pattern and age of onset as heterozygous patients, which argues that CJD associated with the codon 200 lysine mutation is a true dominant disorder. In the caucasian population, Palmer et al. (1991) reported an association between homozygosity in a polymorphic site at codon 129 of the PrP gene, coding for either valine or methionine, with a tendency to acquire the sporadic or iatrogenic forms of CJD, as well as with disease age of appearance in the genetic type. The incidence of the polymorphism at codon 129 in the control Libyan population is similar to the one found in the caucasian population. In the Libyan CJD patients, the codon 200 mutation is within a Met129-encoding allele. The incidence of the Met allele is significantly higher in the affected pedigrees than in the control Libyan population; however, no difference was detected between CJD patients, codon 200 healthy carriers, and their normal family members.(ABSTRACT TRUNCATED AT 250 WORDS)


Subject(s)
Creutzfeldt-Jakob Syndrome/genetics , Prions/genetics , Adult , Aged , Alleles , Base Sequence , Codon/genetics , DNA Primers/genetics , Female , Genetic Linkage , Genotype , Heterozygote , Homozygote , Humans , Jews/genetics , Libya , Male , Middle Aged , Molecular Sequence Data , Phenotype , Point Mutation , Polymorphism, Genetic , PrPSc Proteins
3.
Isr J Med Sci ; 29(5): 280-4, 1993 May.
Article in English | MEDLINE | ID: mdl-8314687

ABSTRACT

A serological test for chlamydial infection was administered to 281 Jerusalem women in order to determine the rate and influence of Chlamydia on pregnancy outcome. Serological indication of active infection was present in 7.8% of the tested women, while 15.3% were shown to be positive for Chlamydia. Among the ultraorthodox subpopulation of Mea Shearim, serological indication of active infection was present among 5.9% of the women, and 12.3% of this population tested positive. In comparison, women from the secular subpopulation had 12.7% serological indication of active infection and 22.95% tested positive (P < 0.01). There were no statistically significant differences between pregnancy duration, birthweight, incidence of premature uterine contractions, premature rupture of membranes, and postpartum febrile morbidity in the infected and noninfected groups. Women with a previous history of induced abortions showed a significantly higher evidence of past Chlamydia infection (9.3%) when compared with the women who did not have an infection (1.4%) (P < 0.006). Among the ultraorthodox women with positive or active infection, 41% had suffered at least one spontaneous abortion, as compared with 25% of the religious women who had no serological evidence of infection.


Subject(s)
Chlamydia Infections , Pregnancy Complications, Infectious , Pregnancy Outcome , Abortion, Induced/statistics & numerical data , Abortion, Spontaneous/epidemiology , Antibodies, Bacterial/blood , Chlamydia Infections/complications , Chlamydia Infections/epidemiology , Chlamydia Infections/immunology , Chlamydia trachomatis/immunology , Female , Humans , Immunoglobulin A/analysis , Immunoglobulin G/analysis , Israel/epidemiology , Jews/statistics & numerical data , Pregnancy , Pregnancy Complications, Infectious/epidemiology , Pregnancy Complications, Infectious/immunology
4.
J Nurs Adm ; 18(5): 19-22, 1988 May.
Article in English | MEDLINE | ID: mdl-3367226

ABSTRACT

Labor stoppages in health care facilities occur periodically and strike terror into the hearts of those responsible for continuity of patient care and safety. This article describes preparation for and survival during an actual labor stoppage of support services personnel.


Subject(s)
Nursing Service, Hospital/organization & administration , Personnel Administration, Hospital , Strikes, Employee , Hospital Bed Capacity, 500 and over , Humans , New York , Nurse Administrators , Personnel Staffing and Scheduling
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