French validation of the Quality of life in Essential Tremor Questionnaire (QUEST) and the Essential Tremor Embarrassment Assessment (ETEA).

Two scales have been developed and validated in English to evaluate the impact of tremor on daily life, namely Quality of life in Essential Tremor Questionnaire (QUEST) and Essential Tremor Embarrassment Assessment (ETEA). The psychometric properties of the French version of these two scales were assessed for 117 patients with head tremor. Both scales showed excellent acceptability, very good internal consistency (Cronbach's alpha coefficient>0.8) and reproducibility (Lin concordance coefficient>0.8), satisfactory external validity and satisfactory sensitivity to change. In conclusion, the French versions of QUEST and ETEA are comprehensive, valid and reliable instruments for assessing patients with head tremor.

[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.

Postural disorders in Parkinson's disease.

Posture is often affected in Parkinson's disease. Postural abnormalities belong to the motor axial involvement. Generally, postural dysfunction induces clinical impairment at the latest stages of the disease, except in late-onset idiopathic Parkinson's disease and in atypical parkinsonian syndromes. Posture may be affected in its orientation component (stooped posture, camptocormia, Pisa syndrome) or in its balance component (loss of postural reflexes). Overall, postural impairment is poorly improved by levodopa, which implies that it is unlikely due to the nigrostriatal dopaminergic denervation. Several methods of investigation have been proposed but are generally not available in clinical practice. Medical treatment and deep brain stimulation (DBS) of the subthalamic nucleus or globus pallidus pars interna are less efficient on axial than on distal motor signs. The pedonculopontine nucleus seems promising as a new target for DBS in combination with the subthalamic nucleus. Physical therapy is, in most cases, the best way to improve postural dysfunction.

[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.

[Leptomeningeal dissemination after ethmoidal sinus adenocarcinoma surgery: a rare complication]. / Dissémination leptoméningée après chirurgie d'un adénocarcinome de l'ethmoïde.

INTRODUCTION: Although rare, adenocarcinoma is the most frequent neoplasm of the ethmoid sinus and must be regarded as an occupational disease secondary to chronic wood dust exposure. Few cases with neurological metastasis have been reported. CASE REPORT: We report the cases of two patients who developed a multiple cranial nerve disorder for the first case and a cauda equina syndrome for the second, after ethmoid adenocarcinoma surgery. CONCLUSION: Diagnosis of carcinomatous meningitis is difficult and is based on clinical data, CSF analysis and gadolinium-enhanced T1-weighted brain and spinal cord MRI. The implication of surgery is discussed. Prognosis of such a disorder is poor.

[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.

Subthalamic nucleus stimulation in Parkinson's disease : anatomical and electrophysiological localization of active contacts.

OBJECTIVES: 1 - To assess the anatomical localization of the active contacts of deep brain stimulation targeted to the subthalamic nucleus (STN) in Parkinson's disease patients. 2 - To analyze the stereotactic spatial distribution of the active contacts in relation to the dorsal and the ventral electrophysiologically-defined borders of the STN and the stereotactic theoretical target. METHODS: Twenty-eight patients underwent bilateral high-frequency stimulation of the STN (HFS-STN). An indirect anatomical method based on ventriculography coupled to electrophysiological techniques were used to localize the STN. Clinical improvement was evaluated by Unified Parkinson's Disease Rating Scale motor score (UPDRS III). The normalized stereotactic coordinates of the active contact centres, dorsal and ventral electrophysiologically-defined borders of the STN were obtained from intraoperative X-rays images. These coordinates were represented in a three-dimensional stereotactic space and in the digitalized atlas of the human basal ganglia. RESULTS: HFS-STN resulted in significant improvement of motor function (62.8%) in off-medication state and levodopa-equivalent dose reduction of 68.7% (p < 0.05). Most of the active contacts (78.6%) were situated close to (+/- 1.6 mm) the dorsal border of the STN (STN-DB), while 16% were dorsal and 5.4% were ventral to it. Similar distribution was observed in the atlas. The euclidean distance between the STN-DB distribution center and the active contacts distribution center was 0.31 mm, while the distance between the active contacts distribution center and the stereotactic theoretical target was 2.15 mm. Most of the space defined by the active contacts distribution (53%) was inside that defined by the STN-DB distribution. CONCLUSION: In our series, most of the active electrodes were situated near the STN-DB. This suggests that HFS-STN could influence not only STN but also the dorsal adjacent structures (zona incerta and/or Fields of Forel).

[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.