[Subjective quality of life profile in patients with multiple sclerosis]. / Profil de qualité de vie subjective chez les sujets atteints de sclérose en plaques.

OBJECTIVES: This study aimed to assess subjective quality of life in patients with multiple sclerosis regarding the criteria of satisfaction and importance attached to various domains of life. METHOD: Sixty-one subjects took part in this study. The psychological evaluation consisted of a clinical interview and three questionnaires: subjective quality of life profile (PQVS), State-Trait anxiety inventory (STAI), Beck depression inventory (BDI). RESULTS: The results show a low level of general satisfaction of subjective quality of life. Fatigue and pain are the most unsatisfactory domains of life while the relationships with close relations and medical staff are perceived as the most satisfactory and important domains of life. Some differences are observed between men and women regarding the satisfaction of conjugal life and fatigue perception. The subjective quality of life seems mainly correlated to anxiety, depression and to the level of handicap. CONCLUSIONS: Our results emphasize the necessity of taking into account the subjective perception of the disease which can lead in an appropriated treatment. These results underline the place of anxious and depressive disorders associated with multiple sclerosis, which seem to have a certain impact on the subjective quality of life.

[Long-lasting and isolated Uhthoff's phenomenon after effort preceding multiple sclerosis]. / Phénomène d'Uhthoff lié à l'effort, isolé, précédant une sclérose en plaques.

INTRODUCTION: Described in 1890, Uhthoff's symptom corresponds to a transient blurred vision provoked by physical exercise or an increase of temperature. It is a frequent symptom occurring during the course of multiple sclerosis. PATIENTS AND METHOD: We report here four cases of patients presenting isolated Uhthoff phenomenon preceding multiple sclerosis by several years. RESULTS: These four patients presented transient neurological symptoms induced by intensive sporting activity for 1 to 6 years before diagnosis of multiple sclerosis. These symptoms were often visual but sometimes motor or sensorial. All symptoms appeared above a certain threshold specific to each patient, after 15 to 30minutes of intense physical exercise (bike, running or handball) and all disappeared after a few minutes to one hour rest with full recovery to baseline. DISCUSSION: Uhthoff's phenomenon is explained by a conduction block. It is due to axonal demyelization that provokes a reorganization of sodium channels induced by a decrease in a safety factor highly sensitive to temperature, or by release of soluble blocking substances (oxide nitric or cytokines). CONCLUSION: Without being specific, this symptom is strongly suggestive of this disease. Our case reports confirm the existence of "infraclinic multiple sclerosis".

[What has changed for stroke at the beginning of the 21st century]. / Les accidents vasculaires cérébraux : ce qui a changé au début du xxi(e) siècle.

INTRODUCTION: Striking developments in stroke epidemiology, initially based on the results of the Framingham study, have greatly improved our neuroepidemiological knowledge of the disease. STATE OF ART: The development of stroke registries has made it possible to evaluate the descriptive epidemiology of stroke and its evolution. With the increasing use of CT-scan, MRI, and either cardiac or vascular imaging, the diagnosis of stroke and its subtypes has been made easier. Over the last 20 years, a decrease in the incidence and mortality of stroke has been observed in Western countries. In contrast, in Dijon, which has the only population-based stroke registry in France, stable incidence rates have been reported. However, over the same period, age at stroke onset has risen by five years in men and eight years in women, which is probably related to both population aging and improvements in primary prevention and general health. The reported decrease in case-fatality rates suggests better acute management of stroke patients, and explains in part the increase in the prevalence of stroke. In addition, the assessment of vascular risk factors has demonstrated that high blood pressure remains the principal risk factor for both ischemic and hemorrhagic stroke, and that antihypertensive treatment is able to reduce stroke incidence. PERSPECTIVES: Epidemiology studies could make it possible to measure the impact of new therapeutic strategies applied in both primary and secondary prevention. CONCLUSION: Prevention, diagnosis, and acute treatment of stroke have considerably improved, but cerebrovascular diseases together with myocardial infarction remain the leading cause of death. Despite the absence of a rise in the incidence of stroke, its prevalence has increased. This is due to the decrease in case-fatality rates. As a consequence, there is an urgent need to organize health networks around stroke. Moreover, the rise in stroke-free life expectancy is a positive finding that reflects improvements in prevention.

Epidemiology of ischemic stroke from atrial fibrillation in Dijon, France, from 1985 to 2006.

BACKGROUND: Atrial fibrillation (AF) is strongly associated with age, and epidemiologic studies are needed to evaluate the impact of both aging of the population and the use of anticoagulant therapy in patients with AF on the incidence of cardioembolic stroke with AF (CE/AF stroke). METHODS: We evaluated trends in incidence rates, risk factors, prestroke therapy, and survival in CE/AF stroke from a prospective population-based registry, from 1985 to 2006. RESULTS: A total of 3,064 ischemic strokes, including 572 (18.7%) CE/AF strokes, were recorded. Over the 22 years, a decrease in the incidence of overall CE/AF stroke was noted (incidence rate ratio 0.9858, 95% confidence interval [CI] 0.9731-0.9986; p = 0.03). We observed a higher prevalence of previous AF, previous myocardial infarction, and patients aged >70 years in CE/AF stroke (p < 0.0001) whereas hypercholesterolemia was more prevalent in other ischemic strokes (p = 0.003). A significant increase in the use of anticoagulants and antiplatelet agents was noted, and was particularly pronounced for CE/AF stroke with previous AF. For CE/AF stroke, survival rates were 72% at 1 month (95% CI 0.68-0.76), 52% at 1 year (95% CI 0.48-0.56), and 43% at 2 years (95% CI 0.39-0.48), and remained lower than those of other ischemic stroke. CONCLUSIONS: The decrease in the incidence of cardioembolic/atrial fibrillation stroke in our study was probably due to a slight increase in the utilization of antithrombotic therapy in patients with atrial fibrillation, but the use of such therapies will have to increase further because of the expected aging of the population in coming years.

Ischaemic stroke subtypes and associated risk factors: a French population based study.

BACKGROUND: There is little reliable population based information about the distribution of risk factors among the various ischaemic stroke subtypes, even though determining risk factor profiles is of major importance to develop targeted preventive strategies. METHODS: The distribution of first ever ischaemic stroke subtypes was established in a prospective population based study conducted in Dijon, France (152,606 inhabitants). Cases were collected between January 2005 and December 2006, and were classified using TOAST classification. Vascular risk factors were recorded to determine a risk factor profile for each subtype. RESULTS: 332 patients with first ever ischaemic stroke (150 men and 182 women) were recorded. Adjusted incidence to world population was 54/100,000/year. The distribution of ischaemic stroke subtypes was as follows: 119 (35.8%) cases of large artery atherosclerosis, 89 (26.8%) small artery occlusions, 81 (24.4%) cardioembolisms and 43 (13%) other and undetermined causes. The most frequent vascular risk factor was hypertension, irrespective of the ischaemic stroke subtype, with a total prevalence of 62%. Using multivariate regression, a positive association between cardioembolism and age (OR 1.051; 95% CI 1.026 to 1.076; p<0.001) was demonstrated and between small artery occlusion and either high blood pressure (OR 1.86; 95% CI 1.06 to 3.27; p = 0.03) or hypercholesterolaemia (OR 2.23; 95% CI 1.33 to 3.76; p = 0.02). CONCLUSION: This comprehensive prospective population based study has demonstrated that vascular risk factors exhibit a particular distribution according to the ischaemic stroke subtypes. These findings, as well as the great frequency of hypertension among stroke patients, have implications for prevention strategies, the design of clinical trials and the organisation of health care services.

[Contribution of the Dijon Stroke Registry after 20 years of data collection]. / Les apports du registre dijonnais des accidents vasculaires cérébraux en 20 ans d'activité

INTRODUCTION: The Dijon Stroke Registry is the only population-based registry in France which has collected neurological data without interruption for more than 20 years. This registry has produced reliable epidemiological data from a large non-selected population. PATIENTS AND METHODS: During the 20-year study period, 3142 ischemic strokes, 341 primary cerebral hemorrhages and 74 subarachnoid hemorrhages were recorded. Age at first stroke rose by five years in men and eight years in women. RESULTS: Comparing the descriptive epidemiology data between 1985 to 1989 and the 2000-2004 periods, the following results were noted: age- and sex-standardized incidences of first-ever stroke were stable except for lacunar stroke where the incidence increased significantly (p=0.05), and for cardioembolic stroke where incidence decreased significantly (p=0.01); 28-day case-fatality rates decreased significantly mainly for lacunar stroke (p=0.05) and for primary cerebral hemorrhage (p=0.03). The proportion of subjects with hypercholesterolemia and diabetes increased significantly (p<0.01). Analysis of cohort data issuing from the registry yielded the following findings: hyperglycemia during the acute stage of cerebral infarct was linked with poor prognosis; lower levels of E. apolipoprotein linked to HDL-cholesterol were associated with atherothrombotic infarcts; activation of coagulation factors was linked with atherothrombotic infarct; decreased N-acetyl-aspartate, a marker of the number of neurons, and increased serum lactate, a marker of anaerobic metabolism measured by proton magnetic resonance spectroscopy were noted in the cohort of cerebral infarct victims; release of platelet V-glycoprotein was noted in cerebral infarct; the incidence of cerebral infarct rose during autumn and during periods with high levels of atmospheric ozone and was higher in male smokers aged more than 40 years with hypertension. The population-based registry contributed to the assessment of medicoeconomic expenditures and professional practices. CONCLUSION: In Dijon, age- and sex-standardized stroke incidence has remained stable over the past 20 years. Increasing age at first-ever stroke, decreasing case-fatality rate, increasing use of antiplatelet treatments and a reduction in certain prestroke risk factors were noted.

The adolescent and adult form of cobalamin C disease: clinical and molecular spectrum.

BACKGROUND: Cobalamin C disease is the most common inborn error of cobalamin metabolism with an autosomal recessive mode of inheritance and mutations within the MMACHC gene. Clinical features, including systemic, haematological and neurological abnormalities, usually occur in the first year of life. Adolescent and adult onset presentations are rare. METHODS: We report on the clinical, molecular and imaging features in three patients aged 40, 42 and 42 years at the last follow-up. We examine these cases together with eight previously described cases to determine the clinical and molecular features of the disease in adults. RESULTS: Mean age at onset of clinical symptoms was 26 years; clinical features included predominant neurological disturbances and thromboembolic complications. White matter abnormalities on brain MRI were sometimes observed. Most patients (eight of nine patients investigated) were compound heterozygotes for the 271dupA mutation and a missense mutation. Intramuscular or intravenous hydroxycobalamin therapy stopped the progression of the disease and resulted in a better clinical outcome and favourable biological status in 7/9 treated cases, while the two untreated patients died quickly. CONCLUSIONS: As cobalamin C disease and related disorders of homocysteine metabolism are treatable conditions, homocysteinaemia should be included in the investigations of patients with progressive neurological deterioration, unexplained psychiatric disturbances or recurrent thromboembolic events.

[Contribution of ecological evaluation of executive disorders in multiple sclerosis]. / Apport de l'évaluation écologique des troubles exécutifs dans la sclérose en plaques.

INTRODUCTION: Multiple sclerosis (MS) is a major cause of neurological disability among young adults. The cognitive disorders are the second cause of alteration of quality of life after physical handicap and are often responsible for loss of social-occupational adaptability. The prevalence of cognitive disorders is 40 to 65%. The alteration of executive functions predominates whereas instrumental functions are generally preserved. The assessment of these disorders is often underestimated by the usual battery of neuropsychological tests. However, the link between psychometric results and executive difficulties of daily life is uncertain. OBJECTIVES: To evaluate the sensitivity of an ecological test compared to standard psychometric tests in assessment of executive disorders in MS. METHODS: Twenty subjects with clinically definite MS were matched for age, sex and pre-morbid intellectual level with control subjects. A battery of neuropsychological and ecological tests was applied to all subjects. The performances on these tests formed a global score of executive function (SFE). The "paper and pencil" multiple errands test was used as the ecological test to examine planning and goal-oriented behavior. We also assessed fatigue and depression with the Fatigue Severity Scale and the Beck Depression Inventory. RESULTS: There was no significant differences between MS patients and controls in neuropsychological executive tests, except for verbal fluencies (p=0.01). The performances were significantly decreased in the MS group for the multiple errands test (p=0.01). 75% of MS subjects have a pathological score for this test. There was a significant link between the performances with this test and SFE (p=0.009). CONCLUSIONS: Executive disorders are underestimated in MS. However, we suggest that an ecological approach is more reliable than standard neuropsychological tests to estimate the cognitive difficulties in daily life in MS subjects. The results of our study favor further research to ascertain the usefulness of ecological assessment in MS.

[Immunofixation compared to isoelectric focusing in the detection of oligoclonal bands in cerebrospinal fluid of multiple sclerosis patients]. / Comparaison de l'immunofixation après électrophorèse sur gel d'agarose avec la focalisation isoélectrique dans la détection des bandes oligoclonales d'IgG du liquide céphalo-rachidien de patients atteints de sclérose en plaques.

INTRODUCTION: Intrathecal immunoglobulins (Ig) synthesis, reflected by oligoclonal bands (OCBs) in cerebrospinal fluid (CSF) is observed in up to 90 percent of patients with clinically definite Multiple Sclerosis (MS). The gold standard laboratory test to establish the presence of OCBs in CSF of MS patients is isoelectric focusing (IEF). However, a quicker and less expensive method has been developed: immunofixation (IF). METHODS: The aim of this study was to compare these two methods carried out 74 CSF/serum pairs of MS, 103 CSF/serum pairs of subject controls and to determine their sensitivity and specificity. RESULTS: The agreement between results from IEF and IF was excellent (Kappa = 0.84). IEF sensitivity (78 percent) was not significantly different from that of IF (74 percent) (p = 0.3). Similarly, the specificity of IEF (93 percent) was not significantly different from that of IF (95 percent) (p = 0.2). CONCLUSION: IF is a semi automated method which is easier to perform than IEF and which appears to be as efficient as IEF.

[Alien hand syndrome due to a corpus callosum infarct: a case report]. / Le syndrome de la main étrangère par infarctus du corps calleux. A propos d'un cas.

A 64-year-old man with alien hand syndrome presented with abnormal feelings in the left upper limb, associated with an intermanual conflict. There were no clinical signs of frontal or parietal apraxia. This syndrome was induced by an infarct localized in the right posterior area of the splenium, subsequent to a cardiogenic embolus. This observation enlightens the rising syndrome of callosal type alien syndrome due to a posterior callosal infarct.

[Seasonal variation in strokes incidence and the influence of the meteorological conditions]. / Variation saisonnière des accidents vasculaires cérébraux et influence des conditions météorologiques.

The aim of this paper was to study the seasonal and Monthly distribution of different subtypes of strokes, and also the influence of the most usual meteorological factors on their incidence. The study, based on the population-based data of the Dijon register of stroke (France), involved 3287 patients with a cerebrovascular event during the Years 1985-1998. The seasonal distribution pointed out a summer decrease for all stroke subtypes. The cerebrovascular risk was the highest in autumn for small artery atheroma infarcts and for cerebral infarcts and, among these later, for cardioembolic infarcts and large artery atheroma infarcts, as well as for subarachnoid hemorrhages and for the total number of strokes. It was followed by spring (total number of strokes, small artery atheroma infarcts, cerebral infarcts) and/or winter (subarachnoid hemorrhages, cerebral infarcts, cardioembolic infarcts and large artery atheroma infarcts). For intracerebral hemorrhages, the risk peaked in spring and secondly in autumn. But the difference from one season to another was significant only for total number of strokes, cerebral infarcts and, among the later, for cardioembolic infarcts and large artery atheroma infarcts. As regards the Monthly distribution, the difference between Months was significant only for the total number of strokes and cerebral infarcts, with a minimum from July to September and a maximum in October for the later. Correlations with meteorological data were found for the total number of strokes, cerebral infarcts, cardioembolic infarcts, large artery atheroma infarcts and small artery atheroma infarcts. They showed an influence of temperature and relative humidity of the day of stroke or of the one to five days before. Correlations with wind speed, duration of sunshine or snow, even if less frequent, could also be found. Such results sometimes differ from those of some earlier studies, for which the climate and the risk factors were not the same, showing that regional epidemiological studies are necessary in order to determine the relations between seasons, meteorological factors and strokes.

[Gustatory disorders during multiple sclerosis relapse]. / Les troubles du goût au cours de poussées de sclérose en plaques.

Gustatory dysfunction is a known but uncommon element in the course of multiple sclerosis. Gustatory dysfunction has been described during the chronic progressive phase and during the relapse phase. We report five patients with clinically definite multiple sclerosis who developed transient gustatory disorders during the relapse phase of their disease. Agueusia occurred as one of the first symptoms in three patients, revealing the disease. Symptoms generally improved with remission or corticosteroid administration. These disorders are due to demyelinating lesions of the gustatory pathways in the thalamus or brainstem. MRI studies were not performed during the relapse phase and no clinical neuroimaging correlations could be established. Agueusia may be accompanied by olfactory dysfunction which is due to plaque demyelinization of the olfactory pathways, particularly in the temporal and inferior frontal lobes. Taste anomalies can also be observed in other diseases, including systemic diseases such as sarcoidosis or Sjogrën's syndrome. Drug-induced gustatory disorders are also reported.

[Epidemiology of hemorrhagic strokes]. / Epidémiologie des accidents hémorragiques cérébraux.

Hemorrhagic stroke (HS) may be secondary to spontaneous intracerebral hemorrhage or to subarachnoid hemorrhage. There are no clinical symptoms that differentiate hemorrhagic stroke form ischemic stroke (IS) and only imaging can make the difference. But epidemiology, pathologic process and management are different in HS and IS. We will discuss the different risk factors of spontaneous intracerebral hemorrhage and subarachnoid hemorrhage.

[Evaluation of follow-up and evolution of multiple sclerosis]. / Evaluation du suivi et de l'évolution de la sclérose en plaques.

The aim of this paper is to review the quantitative methods used for assessing neurological status in multiple sclerosis patients. The Expanded Disability Status Scale (EDSS) is the most wide used. Its psychometric properties, validity, and inter- and intra-rater reliability are modest and responsiveness is weak. Similar results are obtained with the other scales used. The recently developed Multiple Sclerosis Functional Composite (MSFC) scale for clinical trials satisfies this requirement, but is not suitable for individual evaluation. There is no scale in French for assessment of cognitive disturbances and only one, the SEP-90, for quality of life. Brain and spinal cord abnormalities with conventional magnetic resonance imaging parameters (T1, T2-weighted and gadolinium enhanced images) have a weak relationship with disability. New magnetic resonance techniques (magnetic resonance spectroscopy and magnetized transfer imaging) would be more sensitive and should be further investigated.

Immunolocalization and characterization of the low molecular weight antigen (4-5 kDa) of Toxoplasma gondii that elicits an early IgM response upon primary infection.

A striking feature of toxoplasmic seroconversion is the prominent and early IgM response to a low molecular weight antigen of 4-5 kDa. Two different monoclonal antibodies directed against the 4-5 kDa antigen have been generated and used to characterize this molecule. Using these monoclonal antibodies, we could demonstrate the surface localization of the low M(r) antigen by immunofluorescence and immuno-electron microscopy assays. By immunoblotting, we observed that one of the monoclonal antibodies was unable to recognize the 4-5 kDa antigen in tachyzoites propagated in cell culture, indicating an epitope variability between Toxoplasma gondii tachyzoites grown in vivo and in vitro. We discuss the implications of this latter finding in the design of diagnostic reagents.

Surface antigens of Toxoplasma gondii.

Monoclonal antibodies reacting with pellicular antigens of Toxoplasma gondii tachyzoites have been selected among hybridomas produced against this organism by immunofluorescence assay. These antigens have been further characterized by immunofluorescence on living zoites, Western immunoblotting and immunoprecipitation of lactoperoxidase surface radio-iodinated tachyzoite lysates. The simultaneous characterization of 5 different surface antigens (P43, P35, P30, P23, P22) some of which have already been studied individually allowed a better definition of these antigens and the characterization of a yet undescribed surface molecule (P23).