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Biomedical subjects

C Confavreux

Publications and source records attributed to C Confavreux.

At least 55 records · Page 3Linked to original sources

[MRI in the study of multiple sclerosis].

Magnetic resonance imaging (MRI) has provided considerable insight into the pathological process and disease activity and progression in multiple sclerosis. MRI has become an important tool for the diagnosis of multiple sclerosis, and increasingly for monitoring treatment trial. The growing use of MRI calls for careful consideration in applications so that the technology is not misused. Here we propose a summary of the literature on MRI in application in clinical neurology.

Humans↗

[Intramedullary subependymoma of the cervical spinal cord. Review of the literature a propos of a case].

A case of intramedullary subependymoma (SE) of the cervical spinal cord is reported. A 35 year-old female patient developed paresthesiae in her right upper and lower limbs for approximately two years. Magnetic resonance imaging (MRI) of the cervical spinal cord disclosed an intrisic medullary tumor at the C2-C3 levels, predominantly involving the right hemicord. At surgery, a subpial ependymoma was totally removed using the ultrasonic surgical aspirator with no resulting anomalies of the intraoperative somatosensory evoked responses. The patient made a good recovery. At one-year follow-up, she had resumed her previous occupation and complained only from minor sequelae. Twenty-nine spinal cord SE of the literature are detailed. It is likely that they account for less than two per cent of all spinal cord tumors. A majority of cases involved the cervical cord or the cervicothoracic junction. Signs and symptoms are similar to those of other spinal intramedullary tumors. In 17 cases explored by MRI, no specific pattern could distinguish SE from astrocytomas or ependymomas, except perhaps for a more marked tendency for SE to grow excentrically within the cord or to show an exophytic component. Spinal cord SE show pathological features grossly comparable to those described in intracranial SE with clusters of small glial cells scattered among densely packed fibers. Histogenesis of SE has been a matter of debate over years, whereas it is currently admitted that they may represent a variant of ependymomas. In conclusion, most clinical and imaging features of spinal cord SE are similar to those of other pathological varieties. Radical removal is the most appropriate treatment modality, despite inherent risk of postoperative deterioration and significant sequelae on long term follow-up.

Adult↗

Clinical outcomes assessment in multiple sclerosis.

This article represents initial deliberation of an international task force appointed by the US National Multiple Sclerosis Society to develop recommendations for optimal clinical assessment tools for multiple sclerosis clinical trials. Presented within this article are the key issues identified by the task force during its initial year of deliberation. These include the precise purpose for a clinical assessment tool, the clinical dimensions to be measured in a multidimensional outcome measure, desirable attributes of an optimal clinical outcome measure, the complexities of multidimensional outcome measures, the relative merits of categorical clinical ratings and quantitative functional assessments, and a number of other important design issues that relate to the use of a multidimensional outcome measure. An action plan for analysis of existing data is summarized, as are the plans for more detailed recommendations from the task force.

Clinical Trials as Topic↗

Emerging treatments in multiple sclerosis: azathioprine and mofetil.

Global immunosuppression instead of focused selective or specific immunomodulating strategies may still be relevant in diseases with chronic and broad immune dysregulation such as multiple sclerosis (MS). Among classical or new immunosuppressive drugs, two of them, both inhibiting purine synthesis, show an attractive profile for MS treatment. Azathioprine (AZA) is the most anciently and widely used global immunosuppressive drug in MS. Despite founded initial fears, it can be stated today that AZA is usually well tolerated and compatible with normal daily activities, that it requires minimal monitoring and does not significantly increase the risk of cancer induction after 5 years of continuous usage at the conventional 2.5 mg/kg daily dose. The only two presently available well conducted trials of AZA in ambulatory patients with relapsing-remitting MS show marginally significant beneficial results of AZA treatment on relapse frequency and disability. Some preliminary data on brain MRI are also promising. Mycophenolate mofetil (MMF) affects mainly the desired cell types, with a good safety profile, a rapidly reversible activity, and an absence of mutagenic effect and chromosome breakage. However, it remains to be shown that promising experimental results can be converted into significant clinical results in MS. It is presently demonstrated for AZA and it is presumable for MMF that neither drug is able to cure MS. However, it can be anticipated that either drug in combination with other strategies such as recombinant beta interferon could represent a significant adjunct for the therapeutic control of MS, at least in early ambulatory relapsing-remitting MS. Presently, the choice between the old, no longer 'sexy', but well-known drug as AZA and a young, appealing, but still to be better evaluated drug (notably for the long run) as MMF is a matter of personal, community, industrial and scientific inclination.

Azathioprine↗

Risk of cancer from azathioprine therapy in multiple sclerosis: a case-control study.

An increased risk of cancer has been reported in patients treated with azathioprine. To assess the long-term risk of neoplasia in azathioprine-treated multiple sclerosis (MS) patients, we conducted a case-control study using the Lyon Multiple Sclerosis Database. From the 1,191 MS patients included in the database, we identified patients who developed cancer before December 31, 1991. Each case was then matched to three cancer-free MS controls by gender, date of birth, and date of MS onset. A matched analysis was performed to compare cases and controls for exposure to azathioprine therapy during the same follow-up period. Twenty-three MS patients with cancer were identified: 17 solid tumors, 2 skin carcinomas, 4 hematopoietic cancers. Cases had a mean age of 34.5 years +/- 10.2 (+/- SD) at clinical onset of MS and have been followed up for an average 13.8 years +/- 8.1 before being diagnosed with cancer. Fourteen cases (61%) and 34 controls (49%) had been treated with azathioprine for at least 1 month after being diagnosed with MS (adjusted odds ratio = 1.7; 95% confidence interval [CI], 0.6 to 4.6). When assessing risk associated with different durations of azathioprine therapy compared with no treatment at all, we found that MS patients had an increase in cancer risk of 1.3 (95% CI, 0.4 to 4.0) when treated less than 5 years, of 2.0 (95% CI, 0.4 to 9.1) when treated 5 to 10 years, and of 4.4 (95% CI 0.9 to 20.9) when treated more than 10 years. Similar results were obtained when assessing cancer risk associated with cumulative doses of azathioprine ever taken. This case-control study suggests that the overall long-term risk of cancer from azathioprine is low in MS patients. The results are suggestive of a dose-response relationship with no significant risk during the first years of treatment and a possible increased risk after about 10 years of continuous therapy. Further studies are needed to better assess the risk-benefit ratio of azathioprine in MS.

Adult↗

[Familial epilepsy seizure disclosing long QT syndrome].

The Romano-Ward syndrome shows a congenital prolonged QT on the electrocardiogram. It is often revealed by syncopes, sudden death and, in rare cases, by an epileptic attack. We report the cases of two sisters presenting this syndrome. One of them presented an inaugural epileptic attack. The electrocardiogram returned to normal and the seizures disappeared after a treatment with beta-bloquants.

Adult↗

Double blind placebo controlled multicentre study of ginkgolide B in treatment of acute exacerbations of multiple sclerosis. The Ginkgolide Study Group in multiple sclerosis.

After an open labelled pilot study of ginkgolide B, a potent inhibitor of platelet activating factor, in the treatment of acute exacerbations of multiple sclerosis, a randomised double blind placebo controlled study was undertaken. One hundred and four patients were enrolled in the study: for seven days 43 received placebo, 29 received 240 mg/day ginkgolide B and 32 received 360 mg/day ginkgolide B. There was no statistical difference between the three groups for changes in Rankin, Kurtzke expanded disability status scale (EDSS), and Hauser ambulation index (AI) scores. Although there was a trend in favour of groups treated with ginkgolide for a change of Rankin and AI scores, it is concluded that ginkgolide B is not an effective treatment of exacerbations of multiple sclerosis.

Activities of Daily Living↗

Cholera toxin beta subunit induces the differentiation of human medulloblastoma cell line DEV in a neuronal pathway.

Medulloblastomas are primitive neuroectodermal tumours that are thought to arise from multipotent precursor cells in the cerebellum. Medulloblastoma cells may be undifferentiated or exhibit glial, neuronal or ependymal characteristics, suggesting that they may conserve their ability to differentiate in appropriate circumstances. Medulloblastoma cell lines may thus provide models to study the commitment and differentiation of multipotent CNS progenitor cells. A human medulloblastoma cell line, DEV, has previously been shown to differentiate in an astrocytic pathway after infection by the retrovirus HTLV-1. In this study immunofluorescence flow cytometry shows that cholera toxin beta subunit (CT beta), which binds to the ganglioside GM1, induces a twofold increase in the number of DEV cells differentiating towards a neuronal pathway, as shown by the increased proportion and labelling intensity of cells stained by an anti-neurofilament antibody. Immunocytochemistry shows that after 3 days in culture with CT beta, DEV cells develop processes which stain positive for neurofilaments and MAP-1. This suggests that CT beta induces DEV cells to express a more neuronal phenotype.

Cell Differentiation↗

Establishment and use of multiple sclerosis registers--EDMUS.

EDMUS, the European Database for Multiple Sclerosis, is a clinical database system for multiple sclerosis (MS) that was established on the occasion of the first European Concerted Action for MS, under the auspices of the Commission of the European Communities. Automated functions have been incorporated in the program to preserve consistency between data, generate indexes, provide classifications, and protect a patient's privacy. Retrieval of data, selection of records, and export of selected data to standard software programs are easy. The system is user-friendly, and the user is self-sufficient in all operations. The system has been available since October 1992. By August 31, 1993, it had been implemented in 58 sites. As improvements are made in current operations and new aspects of the disease described, EDMUS is updated. A connection with the North American MS-COSTAR database system for MS is underway, with support of the International Federation of the Multiple Sclerosis Societies. Several international multi-center collaborations are being developed within the framework of a new 3-year European Concerted Action entitled "Establishment and Use of EDMUS as a Database System for Multiple Sclerosis Research in Europe."

Europe↗

Congenital protein C deficiency and superior sagittal sinus thrombosis causing isolated intracranial hypertension.

The first case of a superior sagittal sinus thrombosis causing isolated intracranial hypertension as a result of congenital protein C deficiency is reported. Such a possibility must not be overlooked. Anticoagulation is recommended as a treatment for cerebral venous thrombosis. In the case of congenital protein C deficiency, vitamin K antagonists must be started cautiously due to the risk of skin necrosis.

Cerebral Angiography↗

Dot-blot immunodetection of antibodies against GM1 and other gangliosides on PVDF-P membranes.

A simple and rapid assay for detection of antibodies against GM1 and other gangliosides (GM3, GM2, GD1a, GD1b, GT1b, GD3) is described. Purified gangliosides were applied individually in 1 microliter of methanol to polyvinylidene difluoride (PVDF) membranes. Anti-ganglioside antibodies in human sera were allowed to bind and were revealed with a second antibody coupled to peroxidase. The specificity of antibodies binding to gangliosides was confirmed using established techniques to detect anti-ganglioside antibodies such as immunostaining of gangliosides after high performance thin layer chromatography according to Derrington et al. (1989) and ELISA procedure according to Adams et al. (1991) or using the ability of cholera toxin beta subunit to remove GM1 bound antibodies. The dot-blot assay is the simplest and quickest method to run and it appears to be suitable for large routine screening detection of anti-ganglioside antibodies in sera of patients with neurological diseases.

Animals↗

Brain structures selectively targeted by canine distemper virus in a mouse model infection.

Paramyxoviruses such as measles virus or canine distemper virus are etiological agents for acute and chronic encephalitis (measles inclusion body encephalitis, subacute sclerosing panencephalitis and chronic distemper encephalitis or old dog encephalitis). The mechanisms by which viral injury leads to neurological diseases have not yet been fully elucidated. We have developed an experimental model in mice in order to analyze the spatial and temporal distribution of canine distemper virus in the central nervous system. Cerebral target structures for viral replication were examined for the presence of viral material (proteins and mRNA) during the two stages of the biphasic disease. During the acute stage of infection all target areas could be identified by day 6 with a similar anatomical distribution in all the animals examined, which were either intracranially or intracerebroventricularly infected. Viral mRNA and proteins were selectively localized in certain brain structures such as the thalamus, hypothalamus, substantia nigra (pars compacta), locus ceruleus and raphe nuclei (dorsalis and centralis), and limbic system (hippocampus, septum, entorhinal and cingulate cortex, amygdala). The virus was apparently unable to replicate in cerebellum, striatum, a large part of cortex, or endothelial cells. During the subacute disease, viral material was no longer detectable except in a few structures such as hypothalamus up to 4-6 weeks after inoculation. After this time, all target structures were devoid of any labeling in spite of the occurrence of pathology (obesity, paralysis) during this viral quiescent phase. These results suggest that after the initial viral exposure, expression of viral genes in defined structures might disrupt central homeostasis and finally may lead to neurological or neuroendocrine diseases, even in the absence of the hallmarks of the virus.

Amygdala↗

Multiple sclerosis: a comparison of the latent periods of different populations.

While some research in multiple sclerosis has concentrated on the latent period of this disease in single population groups, none has attempted to carry out significance tests to compare the distributions of the latent periods of two distinct groups. Two comparisons are made here, between the populations attending the Multiple Sclerosis Clinic of the Montreal Neurological Institute and Hôpital Neurologique et Neuro-Chirurgical Pierre-Wertheimer, Lyon. We also compared the latent period separately in males and females. There were no significant differences. The significance tests and power studies were based on a nonparametric model that allows for uncertainty in the ages of disease initiation as well as in the ages of onset.

Adolescent↗