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N Vandenberghe

Publications and source records attributed to N Vandenberghe.

14 recordsLinked to original sources

Fatigue in multiple sclerosis is related to disability, depression and quality of life.

Fatigue in multiple sclerosis is a frequent and disabling symptom that can interfere in daily functioning. The aim of this study is to demonstrate the relationship between fatigue and disability, disease course, depression and quality of life. We administered French valid versions of the Fatigue Impact Scale (EMIF-SEP), the short form of the Beck depression inventory (13 items) and the SF-36 to 237 out of 312 patients with clinically definite multiple sclerosis with EDSS<or=6.5. The EMIF-SEP is composed of four dimensions (cognitive, physical, social role and psychological) and allows a multidimensional evaluation. Using a multivariate analysis, EMIF-SEP total scores with physical and social role subscales were highly correlated with EDSS (p < 0.0001). Cognitive and psychological dimensions of the EMIF-SEP were not linked to EDSS. EMIF-SEP was not correlated with disease course after adjusting for EDSS. EMIF-SEP scores were significantly associated with depression scores (r = 0.74, p < 0.0001). The multivariate analysis also showed a significant impact of fatigue on each scale of quality of life of the SF-36. These data confirm that fatigue is correlated with disability, but cognitive and psychological dimensions of fatigue remain independent. Fatigue is also associated with depression and quality of life.

Adolescent↗

[Ancillary exams in the diagnosis of amyotrophic lateral sclerosis].

The diagnosis of ALS is established on the basis of the revised El Escorial criteria revealing involvement of the upper and lower motor neuron. The presence of upper motor neuron signs is recognized by physical examination, but is not always easy to demonstrate. For the patient, early diagnosis reduces the uncertainty and the long waiting period before exclusion test can be performed. Early referral to ALS specialty clinics will have a beneficial effect on the patient's quality of life. Early diagnosis of ALS allows early use of drugs, slowing the rate of disease progression. MR proton spectroscopy and diffusion tensor imaging are potentially useful diagnosis tools but require further analysis of reproducibility in studies with more carefully matched patients and standardized techniques. There is no biochemical marker found in serum or cerebrospinal fluid to establish the definite diagnosis of ALS. Detection of Nogo-A in muscle offers an easy tool for detecting the presence of ALS but further studies are needed to determine the specificity, sensitivity and predictive value of such modifications. When applying standard transcranial magnetic stimulation, examination of several territories improves sensitivity and the probability of detection of infraclinical upper motor neuron dysfunction. The cortical silent period seems most sensitive. The triple stimulation technique (TST) is a very sensitive method but needs to be confirmed by other teams. Some teams have stated that the technique is painful and time consuming for the patient. To date, the corticobulbar tract cannot be studied with TST and we have little experience for the lower limb in very few ALS patients.

Amyotrophic Lateral Sclerosis↗

[Where is the role of the genetic investigations in amyotrophic lateral sclerosis?].

About 10 p. cent of amyotrophic lateral sclerosis (ALS) cases are familial. Most of the familial ALS (FALS) cases are clinically homogeneous. Among these families, autosomal dominant, X-linked or autosomal recessive transmission can be observed. Most of the causal mutations have been observed in the SOD1 gene. To date, more than one hundred different mutations have been described, but it remains unclear whether the mutation is always responsible for the phenotype. Penetrance of the mutation depends on age, with almost 90 p. 100 of penetrance at age 70 years. There is no anticipation. Worldwide, the most frequent mutation is A4V with dominant transmission, responsible for a severe, rapid form of the disease. The second most frequent mutation is D90A which is generally transmitted recessively, predominantly in the Scandinavian countries. The phenotype is characterized by a long lasting course (mean: 11 years). Other causal mutations have been described in the Alsine, Apex, NF-H and NAIP genes. Other genes can be considered as risk factors, like SMN2, APO E4, APEX, Dynactine, P-450 D6. Presymptomatic testing for FALS seems difficult because little information can be given to the patient regarding the responsibility of the mutation in the disease, age of onset, and disease trends. The same precautions as for Huntington's disease are needed. Genetic investigations can contribute to better understanding of the pathophysiology of ALS. Other causal genes in the 90 p. 100 of FALS without SOD1 mutation and eventually in the sporadic ALS cases may be disclosed. Genetic investigations also determine the precise role of a given SOD1 mutation because of the large number of potential SOD1 mutations, the variability of the transmission mode and the non-exceptional absence of proven causality for ALS.

Amyotrophic Lateral Sclerosis↗

[The coverage of the social consequences of the disease and the loss of autonomy in amyotrophic lateral sclerosis].

There is no specific social answer to amyotrophic lateral sclerosis, which alters quickly autonomy of the patients. Different financial and human assistances can be set up to compensate the loss of autonomy. To date, to obtain this assistance, delays are often long; and there is no individual evaluation of the situation at home. The creation of reference centres should allow a better coverage focused on an individualized answer with a management of systems based on the nearness.

Amyotrophic Lateral Sclerosis↗

[Creatine deficiency syndromes].

INTRODUCTION: Creatine deficiency syndromes are a newly described group of inborn errors of metabolism affecting creatine metabolism. Three diseases have been described: deficiency of arginine: glycine amidinotransferase (AGAT), deficiency of guanidinoacetate methyltransferase (GAMT) and creatine transporter defect (CRTR). STATE OF ART: These syndromes are characterized by a depletion of creatine/phosphocreatine in the brain. Clinically, most of the patients develop a variable mental retardation and a severe speech delay associated with epilepsy, extra-pyramidal syndrome and behavior disturbances. These diseases are often diagnosed during infancy but a few adult cases have been reported recently. Diagnosis is established by measurement of guanidinoacetate and creatine in biologic fluids and in vivo proton magnetic resonance spectroscopy by the total lack of intra-cerebral creatine/phosphocreatine demonstrating. GAMT and AGAT deficiencies are treatable by oral creatine supplementation whereas patients with CRTR do not respond to the treatment. CONCLUSION: Better knowledge of these syndromes is necessary to optimize diagnosis and patient management of these rare but potentially treatable disorders.

Amidinotransferases↗

Predictive parameters of mitoxantrone effectiveness in the treatment of multiple sclerosis.

INTRODUCTION: In a number of controlled trials it was established that mitoxantrone has a beneficial effect on disease progression in multiple sclerosis (MS) patients with a worsening disease course. The aim of this study was to investigate the use of mitoxantrone in clinical practice, and especially to describe predictive parameters of its effectiveness under these conditions. OBJECTIVES AND METHODS: In a retrospective, open-label mitoxantrone study we analysed 94 MS patients (49% relapsing remitting MS (RRMS), 41% secondary progressive MS and 10% primary progressive MS) who received monthly 20 mg i.v. mitoxantrone and 1 g i.v. methylprednisolone for six months, and selected as a criterion of effectiveness the percentage of patients with an Expanded Disability Status Scale (EDSS) improvement of at least one point (confirmed after one year) after stopping the treatment. A multivariate analysis was undertaken to assess the predictive value of five parameters on mitoxantrone effectiveness: (1) total number of relapses since disease onset and before treatment, (2) number of relapses within the past 24 months before treatment, (3) number of relapses in separate areas within the past 24 months before treatment, (4) active MRI scans (including Gd-enhanced lesions), and (5) clinical course of MS. RESULTS: During the observation period from 1 January 1997 to 30 May 2000 more than 44% of the patients improved by one point or more on the EDSS (confirmed after one year), 39% remained stable and 17% deteriorated. In patients with a RRMS course three or more relapses within the past 24 months preceding treatment, and at least one Gd-enhancing lesion resulted in a strong relative benefit (i.e., relative risk) of mitoxantrone effectiveness. In contrast, total number of relapses since disease onset had no impact on disease evolution and disability progression. Multivariate analysis revealed the number of relapses in separate areas within the past 24 months before treatment as the strongest predictive parameter (P < 0.001). CONCLUSION: Mitoxantrone is effective in improving and stabilizing patients with a worsening MS course in routine clinical practice. Several strong predictive parameters of mitoxantrone effectiveness were investigated among which the number of relapses in separate areas within the past 24 months before treatment was found to be the strongest parameter to predict clinical improvement.

Adult↗

Cerebral venous thrombosis in four patients with multiple sclerosis.

We report four new cases of cerebral venous thrombosis (CVT) occurring in patients with multiple sclerosis (MS). Each patient had undergone lumbar puncture at varying times prior to clinical presentation (4 days to over 1 year). Only two of the patients had received intravenous (i.v.) methylprednisolone 48 h prior to CVT and were under oral contraception, a risk factor for cerebral thrombophlebitis. The other two patients had not undergone recent lumbar puncture, were not taking corticosteroids and did not present vascular risk factors. The patients all had normal routine blood work-ups and none had thrombophilia. All patients dramatically improved with full systemic heparinization. Minor sequelae were noticed in two patients. The pathogenesis underlying the occurrence of CVT in MS patients remains unclear and we discuss the relationship between lumbar puncture, steroid treatment and CVT.

Adult↗

Chaotic self-trapping of a weakly irreversible double Bose condensate.

We analyze the dynamics of a weakly open Bose-Einstein condensate trapped in a double-well potential. Close to the self-trapping bifurcation, numerical simulations of the weakly irreversible one-dimensional Gross Pitaevskii equation reveal chaotic behaviors. A two-mode model is used to derive amplitude equations describing the complex dynamic of the condensate.

Journal Article↗

[Waldenstrom disease revealed by a peripheral motor neuron disease].

BACKGROUND: An association between motor neuron disease and Waldenström disease is rarely reported in the literature. The association with peripheral neuropathy and IgM monoclonal gammapathy is well known. CASE REPORT: In our patient, the malignant monoclonal gammapathy had an unusual antigen pattern. This patient was MAG (myelin-associated glycoprotein) negative but showed a very positive serology for GM1 ganglioside and SGPG (3-sulfated glycuronly paragloboside). DISCUSSION: This is the fifth such case to be reported associated a malignant dyglobulinemia with an MAG-negative IgM gammapathy. It is the first case with positive serology for both the GM1 ganglioside and SGPG.

Aged↗

Use of hatch date for broiler breeder production planning.

Data on 109 batches of broiler breeders belonging to six strains and bred in conventional sheds were analyzed to determine the possible influence of natural day length cycle phase on age at start of laying, at sexual maturity, and at peak egg production. The dependence of these variables on date of hatching was characterized by polynomial regression analysis. The resulting equations constitute simple, novel empirical models that are in keeping with current theory on the effects of photoperiod on sexual maturation, and should facilitate production planning in broiler breeder farms.

Animal Husbandry↗

Progression of lower-extremity arterial occlusive disease in type II diabetes mellitus.

The prevalence of lower-extremity arterial occlusive disease (LEAOD), the progression of LEAOD, and the incidence of new LEAOD were determined by noninvasive method in 410 volunteers between the ages of 50 and 70 yr; 252 individuals had type II (non-insulin-dependent) diabetes, 158 were control subjects. LEAOD was monitored with the ankle/arm systolic blood pressure index in combination with Doppler arterial velocity waveform analysis. LEAOD was much more prevalent in the type II patients (22%, 55 of 252) than in the control subjects (3%, 4 of 158) (P less than .00001). The prevalence of risk factors for LEAOD was much higher in the type II patients, including elevated triglyceride, depressed high-density lipoprotein (HDL) cholesterol, hypertension, smoking, and elevated systolic blood pressure. In type II diabetic patients the incidence of new LEAOD over a 2-yr period (14%, 28 of 197) was lower than the incidence of LEAOD progression (87%, 45 of 52). Type II patients with LEAOD also had a high incidence of mortality (22%, 12 of 55) compared with those without LEAOD (4%, 8 of 197) (P less than .0005). A risk score including smoking history, duration of diabetes, depressed HDL cholesterol, total cholesterol, elevated systolic blood pressure, and low obesity index is related to LEAOD. After accounting for the effect of all of the risk factors, we suggest that type II diabetes contributes additional risk for LEAOD.

Aged↗

[Diagnosis and biological monitoring of 6 neurosyphilis cases: value of cerebrospinal fluid analysis].

Our objective is to assess the relevance of the different laboratory findings in cerebrospinal fluid (CSF) and serum for the diagnosis and survey of active neurosyphilis. A retrospective study of six hospitalized neurosyphilitic patients at Neurological Hospital of Lyon from 1987 to 2002 was carried out. Six males were found, aged from 29 to 72 years. Neurosyphilis can be group in two categories: early (meningeal and meningovascular neurosyphilis) and late (progressive general paralysis and tabes dorsalis). All were tertiary stage and HIV negative. We performed in CSF, white and red cell count, cytology, total protein, glucose levels, in CSF and serum, albumin, total IgG, IgA, IgM for calculation of albumin quotient and IgG, IgA and IgM index. Serological tests for syphilis in CSF and serum are VDRL and TPHA. To increase the reliability of treponema antibody tests, the ratio of serum-to-CSF content of albumin is used to assess intrathecal production of treponema antibodies, especially the treponema pallidum hemagglutination assay (TPHA index). The CSF changes in neurosyphilis included elevated cell count with lymphocytic-plasmocytic cell reaction, increased protein content, strongly positive IgG index, numerous positive IgG oligoclonal bands, positive blood and CSF serology. Serological tests are difficult to interpret. Examination of CSF played a major role in the diagnosis and treatment of all forms of neurosyphilis. The CSF abnormalities improved with clinical improvement, especially in meningeal and vascular neurosyphilis, but the response in paresis and tabes was slower or nonexistent. Pleocytosis and protein are indicators of inflammatory activity in the central nervous system and are used as a clinical guide in the diagnostic, for treatment and re-treatment.

Adult↗