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Biomedical subjects

V Bourg

Publications and source records attributed to V Bourg.

15 recordsLinked to original sources

[Turcot's syndrome confirmed by molecular biological tests].

INTRODUCTION: Turcot's syndrome is characterized clinically by the concurrence of a primary brain tumor and a familial adenomatous polyposis or a hereditary nonpolyposis colorectal cancer. OBSERVATION: We report a case of a 45-year-old woman who underwent in 1995 neuro-oncological treatment for an anaplastic astrocytoma (grade III according to the World Health Organization classification). Treatment included complete surgery, radiotherapy, a first-line nitrosourea-based chemotherapy regimen and a second-line platinium salt-based regimen. It was then noted that the patient's brother had colorectal cancer. A genetic study detected a germ-line mutation on the hMSH2 gene specific of HNPCC syndrome (Human Non Polyposis Colorectal Cancer). Colonoscopy was normal. Eight years after the diagnosis, the patient developed a gliomatosis cerebri and died. CONCLUSION: Relevant personal and familial history can provide the clue to the diagnosis of Turcot's syndrome. Molecular diagnosis may contribute to appropriate care of affected patients.

Adaptor Proteins, Signal Transducing↗

Levocarnitine administration in multiple sclerosis patients with immunosuppressive therapy-induced fatigue.

Nutritional factors and comedications are among the postulated causes of fatigue, a highly prevalent symptom in the multiple sclerosis (MS) population, with serious impact on patients' quality of life. Deficiency of carnitine may play a role by reducing energy production through fatty acid oxidation and numerous MS therapies can induce fatigue syndrome. The aim of this prospective open-labelled study was to collect and study serum carnitine levels in MS patients with and without disease-modifying treatment-induced fatigue syndrome. We investigated whether restoration of the carnitine pool might improve treatment-induced fatigue in MS patients. In our study, there was no statistical difference in fatigue frequency between treated and untreated patients (P=0.5). Matched to age, gender and treatments, carnitine levels were lower for MS treated patients compared to untreated MS patients (P <0.05) or controls (P <0.001). Consecutive patients with low plasma carnitine levels who experienced fatigue were substituted. Treatment consisted of oral levocarnitine, 3-6 g daily. All patients achieved normal plasma carnitine levels. For 63% of patients treated with immunosuppressive or immunomodulatory therapies, oral levocarnitine adjunction decreased fatigue intensity, especially in patients treated with cyclophosphamide and interferon beta.

Acetylcarnitine↗

[Recurrent aseptic meningitis associated with primary biliary cirrhosis].

INTRODUCTION: The etiological diagnosis of recurrent aseptic meningitis often requires difficult investigations. We report a case of recurrent aseptic meningitis associated with a primary biliary cirrhosis. CASE REPORT: A 37-year-old woman presented four recurrences of isolated meningitic syndrome with fever. Intravenous steroid treatment was effective. Investigations revealed an old right tympano-labyrinthine fracture with, on MRI, an abnormal enhancement of the posterior wall of the internal auditory canal. Serum tests were also significantly positive for antimitochondria antibodies subtype M2. CONCLUSION: Definitive diagnosis was recurrent puriform aseptic meningitis secondary to an inflammatory non-septic ears-nose-throat focus in a latent autoimmune context of primary biliary cirrhosis. To our knowledge, this is the first case reported in literature.

Adult↗

[Relapsing remitting multiple sclerosis or multiphasic disseminated encephalomyelitis?].

INTRODUCTION: The imaging presentation of some forms of multiple sclerosis may be misleading. In patients with a history of recent infection or vaccination, especially for adolescents or young adults, the differential diagnosis with acute disseminated encephalomyelitis can be difficult. CASE REPORT: We report an unusual clinical and radiological presentation of multiple sclerosis, mimicking acute disseminated encephalomyelitis. We discuss clinical and radiological differential diagnosis, and the outcome after immunosuppressive treatment. CONCLUSION: Distinguishing between acute disseminated encephalomyelitis and the first relapse of multiple sclerosis can be difficult. Brain imaging is a precious tool for differentiating between the two diseases.

Adolescent↗

Long-term outcome of oligodendrogliomas.

BACKGROUND: Favorable prognostic factors for oligodendroglial tumors include age younger than 40 years, low tumor grade, and extent of resection. OBJECTIVE: To assess survival time and prognostic factors of 100 patients with oligodendrogliomas diagnosed between 1995 and 2002. METHODS: The tumors were rated histologically by the WHO classification as low grade (grade II) or anaplastic (grade III). One hundred patients were categorized into three groups: group A: grade II, group B: secondary grade III (low grade with anaplastic transformation during the follow-up), group C: de novo grade III. All patients were symptomatic at presentation and underwent neurosurgical procedure for histologic diagnosis. Follow-up was performed with clinical assessment, brain MRI, and MIBI scintigraphy. RESULTS: There were 66 men and 34 women (mean age at diagnosis 46.7 years). The most common first symptom was partial epileptic seizure (75%). Fifty-six patients had initial gadolinium enhancement (A: 15.6%; B: 36.8% as grade II, 95% as grade III; C: 90%), generally associated with MIBI hypermetabolism (p < 0.0001). Survival rates at 2, 5, and 10 years were A: 88%, 88%, 85%; B: 79%, 64%, 42%; C: 43%, 16%, 15%. CONCLUSIONS: Secondary anaplastic oligodendroglioma patients were younger than patients with de novo anaplastic oligodendrogliomas. Histologic confirmation is mandatory because some low grade oligodendrogliomas had gadolinium enhancement on MRI and some anaplastic did not. Survival time was longer for secondary than for de novo anaplastic oligodendrogliomas without difference in the duration of the malignant phase of the disease.

Adolescent↗

[Neoadjuvant chemotherapy for symptomatic non operable grade II fibrillary astrocytoma in adults].

We collected 6 case-reports of symptomatric non removable low grade fibrillary astrocytoma of adults treated with a procarbazine-CCNU-vincristine chemotherapy regimen. All patients had drug-resistant epilepsy but brain imaging was stable. Total gross resection was rejected because of Volume or tumor location. After 4 to 7 cycles of chemotherapy, 2 patients had partial response and one minor response on brain MRI. All of them were seizure-free. Progression free survival was not reached at 5 Years. Up-front chemotherapy for low-grade astrocytomas may be useful and has to be prospectively evaluated.

Adult↗

[Treatment of progressive multiple sclerosis with monthly pulsed cyclophosphamide-methylprednisolone: predictive factors of treatment response].

INTRODUCTION: Cyclophospamide is used in the treatment of progressive multiple sclerosis. We were looking for predictive indicators of treatment response. MATERIAL AND METHODS: Forty-seven patients with secondary progressive multiple sclerosis and seven others with primary progressive received monthly infusions of cyclophosphamide (750mg/m2) and methylprednisolone (500mg). During the year before cyclophosphamide the EDSS had worsened one point in all patients with or without surimposed relapses. Evaluation was based on EDSS change at 6, 12, 24 months and 5 years. RESULTS: Among secondary progressive patients, 91 per 100 (43/47) were stable or improved at 12 months, 65 per 100 (26/40) at 24 months and 22 per 100 (5/23) at 5 years. Annual relapse rate decreased from 0.81 before treatment to 0.48 during treatment and 0.12 after treatment (p<0.001). At 24 months, efficacy was correlated to a progressive phase lasting less than 5 years (p<0.01) and to a rapid increase of EDSS of at least 2 points the year before treatment (p<0.05). There were no influences of age, EDSS and surimposed relapses at the beginning of treatment, and other immunoactive drugs administrated before cyclophosphamide. There was no significant difference in quality of response to treatment between patients with primary progressive and secondary progressive multiple sclerosis. CONCLUSION: Cyclophosphamide appears to be more efficient in early stage of progressive multiple sclerosis independently of age, relapses or neurological disability scale.

Adjuvants, Immunologic↗

[The contribution of automatic anatomical matching of sequential brain MRI scans in the monitoring of multiple sclerosis lesions].

INTRODUCTION: Magnetic resonance imaging (MRI) has transformed management of patients with multiple sclerosis. The exact contribution of brain MRI remains a subject of debate, but it is generally considered to provide a more specific and more sensitive outcome measure for monitoring purposes and for testing new therapies. The choice of MRI techniques, and measurement reproducibility for multiple sclerosis brain lesions are not defined with precision for routine practice. There are many sources of error when comparing successive images which can be overcome to some extent with repositioning and image processing techniques. METHODS: We evaluated the impact of image repositioning on treatment decision-making for twelve relapsing remitting patients. Brain MRIs were performed every three months for a one-year period. Two neurologists interpreted the non-repositioned and repositioned images giving their analysis of changes in the lesions visualized on the T2 sequences and their therapeutic decisions. RESULTS: For the first neurologist, analysis of the non-repositioned images yielded six patients whose lesions had worsened while for the repositioned images there were only three. For the second neurologist, four patients had more lesions with the non-repositioned images and only three with repositioning. The subjective interpretations were the same for the two neurologists when they used repositioned images. CONCLUSIONS: Comparison by two neurologists of non-repositioned and repositioned MRI, with no other image processing, affected the analysis and in certain cases propositions for treatment.

Adolescent↗

[Epileptic Münchausen syndrome by idiosyncratic of effect antiepileptic drugs].

Between 1996 and 2000, a 36-year-old woman was admitted four times for the presumed diagnosis of status epilepticus. Major skin allergy followed by collapsus occurred each time after intravenous phenytoin therapy. Video-EEG demonstrated non epileptic, psychogenic seizure leading to the diagnosis of Münchausen syndrome by idiosyncratic reaction to antiepileptic drugs.

Adult↗

[Relapsing-remitting inflammatory disease of the central nervous system with normal MRI: multiple sclerosis or phenocopy in a series of 15 patients].

Multiple sclerosis is a demyelinating disease of central nervous system. Although many sub-types and clinical forms are identified, diagnosis is clearly related to the detection of MS lesions on brain MRI. We report data of 15 patients admitted in Nice for suspicion of MS after clinical relapsing-remitting or progressive symptoms. Extensive screening tests (i.e blood sample, CSF, MRI, spectroscopy) were performed at onset and at each relapse. All patients had normal-appearing white matter on spinal cord and brain MRI. Nevertheless, 11 patients can be considered as MS according to McDonald criteria.

Adjuvants, Immunologic↗

[Castleman's disease located in the central nervous system].

A 26-year-old woman complained of trijeminal nevralgy and ocular symptoms revealing a paracavernal tumor which had progressed for three months. Histopathological analysis after partial resection led to the diagnosis of Castleman's disease. Six months later, the patient was considered cured after focal adjuvant radiotherapy. Castleman's disease is a lymphoproliferative disorder. Solitary intracranial involvement is unusual. Unlike multifocal disease, localized Castleman's disease has an excellent prognosis.

Adult↗

[Intravenous immunoglobulins for relapsing-remitting multiple sclerosis after failure of treatment with other immuno-modulators].

Intravenous immunoglobulins are used in the treatment of different autoimmune diseases. Recent trials suggest their efficacy in relapsing remitting multiple sclerosis. We report the results of an efficacy and safety trial using monthly intravenous injections of immunoglobulins for patients with secondary progressive multiple sclerosis. Eighteen patients in clinical progression, who have been previously treated with immunomodulatory or immunosuppressive drugs, were given monthly intravenous immunoglobulin infusions (0.4 g/kg/d for 5 days). At the beginning, the mean EDSS score was 6.77. At the end of the study, an improvement of EDSS was noted in 61.1 p. cent of patients, with less than 1 and 0.75 for secondary and primary progressive diseases respectively. No worsening was reported. Surprisingly, some patients had partial improvement of neurological functions which were considered as sequelae. Indications for intravenous immunoglobulins in the treatment of the multiple sclerosis need to be evaluated.

Adjuvants, Immunologic↗

Nitroso-urea-cisplatin-based chemotherapy associated with valproate: increase of haematologic toxicity.

BACKGROUND: The incidence of haematologic toxicity of valproate (VPA) ranges from 1% to 32% and consists mainly of asymptomatic, dose-dependent thrombopenia. We describe a potentiation of haematologic side-effects of nitroso-urea (NU) when prescribed in association with VPA. PATIENTS AND METHODS: We followed a cohort of 70 patients (58 men, 22 women, mean age: 56 years, range 20-75 years). Patients with high-grade gliomas were treated with up-front chemotherapy regimen consisting of fotemustine (d3: 100 mg/m2), cisplatin (d1-3: 33 mg/m2) and etoposide (d1-3: 75 mg/m2) followed by whole brain radiotherapy at progression. Sixty patients required anti-epileptic drugs (AED) for either a single, well-documented epileptic seizure, or immediatly initiated after neurosurgical procedures. AED included VPA (35 of 60), phenobarbital (PB) (17 of 60), carbamazepine (CBZ) (2 of 60) and phenytoin (PHT) (3 of 60). Two patients had both PB and CBZ and one PB and PHT. RESULTS: Haematologic toxicity (grade 3-4 thrombopenia, neutropenia or both) was observed in 37 of 70 (52.85%) patients. Among them 24 (65%) had VPA. Group C were patients treated with fotemustine alone with or without VPA (23 patients). CONCLUSION: When prescribed in association with a fotemustine-cisplatin regimen, VPA treatment results in a three-fold higher incidence of reversible thrombopenia, neutropenia or both. Haematologic side-effects decrease after AED modification during the continued chemotherapy. This adverse event should be managed with caution.

Adult↗

Intracytoplasmic Th1 and Th2 cytokines in rheumatoid arthritis blood and synovial tissue.

In rheumatoid arthritis (RA), T cells have been proposed either as a main actor or as an epiphenomenon in such a primarily synoviocyte-driven disease. A major issue remains the remarkable paradox between the T cell infiltrate and the relative failure to detect definite markers of their activity. To determine the Th1/Th2 cytokine profile in RA synovium, we used a single cell flow cytometric assay for interleukin-2 (IL-2), interferon-gamma (IFN-gamma), IL-4 and IL-10 in paired peripheral blood (PB) and synovial tissue (ST) lymphocytes from RA and osteoarthritis (OA) patients and PB lymphocytes from healthy controls. Cytokines were undetectable in unstimulated PB and ST lymphocytes. More stimulated PB and ST CD4(+)lymphocytes produced IFN-gamma than IL-4, for all individuals tested. RA PB CD4(+)lymphocytes showed the same Th1 cytokine pattern as normal controls. No increase of such a Th1 profile was observed for ST lymphocytes. A specific recruitment of T CD4(+)lymphocytes in the rheumatoid inflamed synovium could not be concluded on the basis of these results.

Adult↗