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F Cathala

Publications and source records attributed to F Cathala.

At least 55 records · Page 3Linked to original sources

Serum antibodies to neurofilament antigens in patients with neurological and other diseases and in healthy controls.

A total of 529 sera from patients with a wide variety of neurological and non-neurological diseases, and 101 sera from healthy control subjects, were examined by indirect immunofluorescence for the presence of autoantibodies to neurofilament antigens. Antibodies were found in approximately 50% of sera from patients with spongiform encephalopathies, 15-30% of sera from patients with other neurological and non-neurological diseases, and 7% of sera from healthy controls. The antigenic stimulus to these autoantibodies in very diverse disease processes is unknown, but as presently assayed, they are not of sufficient specificity to be useful as an aid to clinical diagnosis.

Alzheimer Disease↗

[Creutzfeldt-Jakob disease of 34 month's development in a 26-year-old woman].

A clinico-pathological case of C. J. disease which occured in a 26 year old patient and evolved over 34 months is reported. Published cases of patients dead before 30 y.o. are briefly reviewed. The average length of evolution was 28 months. Concerning the french cases it appears that this average length was significantly longer when C. J. disease had started before 50 y.o., than when it had begun after 75 y.o. However the general survey of the published cases does not show any correlation between the starting age of the disease and the duration of evolution.

Adult↗

[Familial Alzheimer's disease: a study of HLA markers].

We have studied HLA markers in family with 2 "Probable" and 2 "possible" cases of Alzheimer disease over 3 generations. Three of them (two brothers and the father) present A29 C-B12 DR2 haplotype. It seems that it exists an association between HLA system and Alzheimer disease but we cannot define the character of this genetic linkage; the study of many families and sporadic cases will allow to define it.

Alzheimer Disease↗

[Major OLA histocompatibility complex of sheep. Frequency of factors in sheep affected by trembling (scrapie) or normal sheep].

The OLA histocompatibility complex of sheep was studied in a flock of Ile-de France breed affected with Scrapie. On the whole, gene frequencies of 9 out of 11 factors were increased in the non-affected sheep of this contaminated flock. In these sheep, frequencies of the three following genes appeared to be increased significantly: OLA-A4, OLA-A8, OLA-B6. When these genes were present in the sheep of this flock, the relative risk of Scrapie was estimated at 0.05, 0.10 and 0.25, respectively.

Animals↗

[Creutzfeldt-Jakob disease in France: an epidemiological update (author's transl)].

We report here the results of a prospective epidemiological study of Creutzfeldt-Jakob disease in continental France during the years 1978-80, extending a retrospective study of the decade 1968-1977 previously reported. The new annual mortality figures are 0.53 cases per million people for France as a whole, and 1,01 cases per million people for the paris metropolitan area. The correlation between mortality rate and population density persists in the Parisian area, and is not related to differences in age composition of the density categories. Although the geographic distribution of CJD and Scrapie and completely unrelated, there is a correlation between lamb consumption and CJD mortality rates in different nationwide population categories.

Adult↗

Epidemiologic comparisons between Creutzfeldt-Jakob disease and scrapie in France during the 12-year period 1968-1979.

Systematic investigation of the occurrence of scrapie in France revealed that between 1968 and 1979 the disease has been diagnosed in a total of 145 flocks, in virtually every region where sheep are raised. The geographic distribution of scrapie-affected flocks was unrelated to the residential location of patients dying of Creutzfeldt-Jakob disease (CJD) during this period, arguing against direct contact or indirect vector transmission of the disease to humans. Regional lamb consumption was not correlated with the frequency of CJD; however, lamb consumption among nationwide categories of increasing population density (rural communities, towns, cities, the Paris metropolitan area, and the city of Paris) did correlate with an increasing frequency of CJD. Future accuracy in the reporting of scrapie, together with precise information about commercial distribution routes of lamb and other sheep products, will be required to assess the significance of this observation.

Animals↗

Biologic distinction between sporadic and familial Alzheimer disease by an in vitro cell fusion test.

In vitro cell-fusing activity of brain suspensions prepared from patients with Alzheimer disease occurred in 10 of 17 familial cases (59%), a level similar to that seen in transmissible Creutzfeldt-Jakob disease (CJD), but in only 3 of 17 sporadic cases (17%), a level not statistically different from that in nonneurologic control patients. This biologic distinction between the familial and sporadic forms of Alzheimer disease may be related to the previously reported transmission to primates of a CJD-like disease from two familial cases of Alzheimer disease.

Adult↗

Mycobacterial and fungal skin sensitivity patterns among remote population groups in Papua New Guinea, and in the New Hebrides, Solomon, and Caroline Islands.

Simultaneous intradermal sensitivity testing to Mycobacterium tuberculosis (PPD-S), six different atypical mycobacteria (PPD-A, B, F, G, T, and Y), Coccidiosides immitis (coccidioidin), and Histoplasma capsulatum (histoplasmin) was performed on 560 subjects among the relatively isolated island populations of Ifaluk (Caroline Islands), Loh and Merig (New Hebrides), Anuta (Solomon Islands), and in the Mala (Amdei) and Iwane (Simbari) villages of the Anga linguistic groups in the Marawaka area of the Eastern Highlands Province of Papua New Guinea. At the time these tests were performed (Mala and Iwane villages, and Ifaluk Atoll in 1967, and Loh, Merig, and Anuta Islands in 1972), all island populations had already had a long history of sporadic European contact, whereas the New Guinean villages of Mala and Iwane had remained virtually unexposed to the outside world. Using a dose of 0.001 mg PPD per 0.0 ml (5 TU), and considering induration of at least 10 mm at 48 hours to represent a positive reaction, skin sensitivity to M. tuberculosis was found to be absent among the Anga, and to have an incidence of 11% on Loh, 17% on Merig, 34% on Ifaluk, and 55% on Anuta. Data on the prevalence of tuberculous infection obtained by skin tests reflected the reported prevalence of symptomatic tuberculosis in all groups. The frequency of reactions to 0.0001 mg doses of the atypical mycobacterial antigens corresponded to tuberculin sensitivity in the different groups: none among the Anga, sporadic on Loh and Merig, and common on Ifaluk and Anuta. However, analysis of PPD profiles suggests that actions greater than or equal to 5 mm to a 0.1 ml dose of 1:100 coccidioidin were observed in 26% of the population of Ifaluk, and of 1:100 histoplasmin in 8% of the population of Anuta, and 14-16% of the populations of the two New Guinean villages.

Coccidioides↗

[Creutzfeldt-Jakob disease in the squirrel monkeys].

Four different strains of Creutzfeldt-Jakob disease virus (2 primary and 2 passaged in primates or mice) were inoculated intra-cerebrally into squirrel monkeys implanted with continuously-recording indwelling electrodes. Simultaneous EEC and videotape recordings were made on unrestrained animals. In addition EEG recordings were made of evoked visual potentials on restrained animals. EEG abnormalities appeared in every animal before the first clinical signs (6 to 20 months after inoculation) and included generalized slowing, epileptiform patterns and occasional episodes of pseudo-periodic activity. Abnormal evoked visual potentials and disturbances of consciousness were also noted. All viral strains produced similar disorders and the death of inoculated animals. The relative frequency of epilepsy seen in the CJD-inoculated squirrel monkey contrasts with its irregular occurrence in most other monkey species, and its total absence in the spider monkey. This could be related to the lesser complexity of neo-cortical evolution in the squirrel monkey and a less pronounced development of inhibitory CNS mechanisms under the general control of GABA-ergic neurons.

Animals↗

Familial Creutzfeldt-Jakob disease. Autosomal dominance in 14 members over 3 generations.

Discovery oa a second affected branch of a family with transmitted Creutzfeldt-Jakob disease (CJD), originally reported by Buge et al. in 1978, brings the total number of cases to 14 in 3 generations, with at least 20 members of the next young adult generation presently at risk. Complete segregation of the illness to the descendants of these 2 branches, with no skipped generations, and an overall frequency of CJD in affected sibships of 56%, clearly defines a pattern of autosomal dominance. The disease is indifferent to sex, either in terms of affected members (8 males and 6 females) or of lineage (3 fathers and 3 mothers). Acquisition of CJD virus from a point source contamination is unlikely, and case-to-case transmission, if it occurred, would have required an average minimum incubation period of 17 years.

Creutzfeldt-Jakob Syndrome↗

[Application of a test of cellular fusion in Creutzfeldt-Jakob and Alzheimer diseases (author's transl)].

We have tested the in vitro cellular fusing activity of brain suspensions from the following categories of patients: CJD (53 cases), Alzheimer (23 cases), and non-neurological controls (25 cases). CSF specimens were also available for 26 of the CJD patients. Our results show that 65-75 per cent of CJD brain specimens induce cell fusion but that this activity is only rarely reflected in matched CSF specimens. Among other types of degenerative neurological diseases, a sharp distinction appeared between familial Alzheimer disease, which caused fusion at nearly the same frequency as CJD, and sporadic Alzheimer disease, which was not significantly different from the non-neurological control specimens. The biological and clinical significance of these results are discussed.

Adult↗

Creutzfeldt-Jakob disease in France: I. Retrospective study of the Paris area during the ten-year period 1968-1977.

A systematic search for cases of Creutzfeldt-Jakob disease occurring in the ten-year period 1968 through 1977 was conducted in the city of Paris and surrounding departments which comprise the greater Paris area. Fifty-six cases were found among the total population of approximately 10 million inhabitants, with a maximum incidence of 1.1 cases per year per million people in the densely populated city of Paris and a progressively lower incidence in the less heavily populated suburban and rural areas of the region. There was no temporal or geographic clustering of disease. Attention is drawn to the often rapid onset and short duration of illness (nearly two-thirds of the patients died within five months of onset) and the frequent early occurrence of symptoms such as asthenia, diaphoresis, and disturbances of sleep and appetite.

Adult↗

Creutzfeldt-Jakob disease in France: II. Clinical characteristics of 124 consecutive verified cases during the decade 1968--1977.

One hundred twenty-four consecutive cases of Creutzfeldt-Jakob disease (CJD) in France, verified by biopsy or autopsy between 1968 and 1977, were analyzed with respect to their clinical characteristics. The series comprised equal numbers of men and women, with the most frequent age at onset being 60 to 64 years and the most frequent duration, two to three months. A prodromal illness was observed in more than one-third of the patients. Clinical presentations and symptom frequencies are tabulated, and a multifactor analysis has been performed to obtain those combinations of symptoms and signs which occurred at least as often as the triad of dementia, myoclonus, and a positive electroencephalogram. Two groups of atypical cases are also emphasized: one with sudden, strokelike presentation and rapidly evolving illness of less than 2 months' duration, and the other with a long clinical course of between 2 and 10 years.

Age Factors↗