Epidemiological time-trend of amyotrophic lateral sclerosis (ALS) over two decades: The French population-based register of ALS in Limousin (FRALim register).

INTRODUCTION: Population-based registers are key to understanding disease patterns. Taking advantage of the long-standing operation of the French register of amyotrophic lateral sclerosis (ALS) in Limousin (FRALim register), we sought to determine the time trends in incidence, clinical features and survival of ALS patients from 2000 to 2020. METHODS: FRALim register included incident cases through multiple sources of ascertainment. A capture-recapture method was used to assess the exhaustiveness of case ascertainment. Crude and standardized incidences were calculated per 100,000 person-years of follow-up (PYFU). Time-period was divided (period 2000 to 2010 and period 2011 to 2020) to compare incidence rates and clinical features. Survival was analyzed using Kaplan-Meier method. Cox proportional hazards model was performed to calculate hazards for the time periods. RESULTS: Overall, 501 incident cases were identified during 21 years. The overall crude incidence was 3.26 (95% CI 2.97 to 3.55) per 100 000 PYFU. The exhaustiveness of the register was estimated at 98.8% (95% CI 97.4-99.6%) by capture-recapture analysis. Several fluctuations were observed without a consistent trend over the last two decades. The crude and standardized incidences were higher in males than females. The peak of incidence was observed in the 75-79 years age band. Almost one-third of the cases exhibited a bulbar onset. There were significant differences in clinical features between time periods. Four hundred and ninety-one cases were included in the survival analysis. The median survival time from diagnosis was 16.0 months (95% CI 14.3 to 17.7 months). Patients in the last decade experienced a lower risk of dying but the difference did not reach statistical significance (adjusted HR: 0.89 (95% CI 0.73 to 1.08, P=0.229). CONCLUSION: We provided reliable epidemiological data over two decades. We showed that incidence has been relatively stable, while clinical variability was observed. A slight improvement in survival time was found in the last decade but it was not statistically significant. Further quality register data are needed to improve our understanding of ALS epidemiological trends.

[Social representations of cardiovascular disease in the Tanvè Health Study (TAHES) cohort in Benin]. / Représentations sociales des maladies cardio-vasculaires dans la cohorte Tanvè Health Study (TAHES) au Bénin.

OBJECTIVE: To describe the social representations associated with cardiovascular diseases (CVD) in the village of Tanvè in Benin. METHODS: Focus groups discussions and semi-structured interviews were conducted among participants of Tanvè Health Study (TAHES) cohort. Audio recordings were made during the interviews, as well as some note-taking. Data were organized by themes and analyzed to identify key representations that underlie attitudes and health care practices. RESULTS: A total of 19 subjects (including 11 women) participated in 2 focus groups and 17 (including 5 women) in individual interviews. Etymologies of CVD's names evoke either clinical signs (example: ''tesisi glô mɛ'' [can't stand up]), guessed etiology (example: ''kuvitɔ zɔn'' [ghosts' disease]) or a local adaptation of a biomedical name (example: ''a'tension'' [from hypertension]), to cite the example of stroke. According to beliefs about etiology, diseases were classed as ''simple'' or "organic" diseases ("azɔn yaya") and ''supernatural'' or ''induced'' diseases linked to "azé" [immaterial witchcraft] or "bô" [material bewitchment]. Chronic or recurrent diseases usually end up in the ''supranatural'' category and induce the consultation of "bokɔnɔ᷉" (diviner) or other religious for diagnosis. Health care seeking refer to modern medicine, "amanɔn" (leaf-based care), "bokɔnɔ᷉" or traditional religious (various rites) and Christians (prayers). CONCLUSION: Social representations of CVD in this community are based on ancestral beliefs, but are also influenced by modern knowledge. Taking them into account could improve quality of health to fulfill communities' care needs.

Prevalence of stroke survivors in Parakou in northern Benin: A door-to-door community survey.

INTRODUCTION: Stroke is a major public health problem with a high burden in Sub-Saharan Africa. We aimed to determine the prevalence of stroke in Titirou in Parakou. METHODS: It was a cross-sectional study using a door-to-door survey in Titirou (Parakou) from 15 march to 15 October 2016 and included 4671 adults. We did a two stages survey. In the first stage the World Health Organization (WHO) tool for the diagnosis of stroke in community was used. In the second phase all suspected cases underwent neurological exam and sometimes brain CT-scan. The WHO definition was used for the diagnosis of stroke. We recorded the socio-demographic data and the vascular risk factors. The prevalence was standardized on age according to the WHO type population. Multiple logistic regression was done to identify associated factors and estimate the adjusted prevalence ratio (aPR) and their 95% confidence interval (CI). RESULTS: The mean age of the subjects was 27.7±12.9 years with a sex ratio of 0.97. After screening 161 were suspected and 54 confirmed cases, the overall prevalence of stroke in Titirou was 1.156 per 100,000 inhabitants [95% CI: 0.850 to 1.426]. The age-standardized prevalence of stroke was 3223 cases per 100,000 inhabitants. The associated factors were age (aPR 1.7 [1.5-1.9] for 10 years), history of hypertension (aPR: 64.8 [46.1-108.9]), diabetes mellitus (aPR: 4.5 [1.6-12.3]), low consumption of fruits and vegetables (aPR: 2.3 [1.2-4.4]), history of heart disease (aPR: 6.0 [2.6-13.7]), family history of stroke (PR: 4.6 [2.1-10.0]). Among the 54 subjects who had a stroke 10 were able to perform the brain CT-Scan: 40% were hemorrhagic and 60% ischemic stroke. CONCLUSION: Our study showed a high prevalence of stroke in Titirou and suggested urgent action for prevention.

Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis.

BACKGROUND AND PURPOSE: The aim of this study was to investigate patients with amyotrophic lateral sclerosis in order to determine their nutritional, neurological and respiratory parameters, and survival according to metabolic level. METHODS: Nutritional assessment included resting energy expenditure (REE) measured by indirect calorimetry [hypermetabolism if REE variation (ΔREE) > 10%] and fat mass (FM) using impedancemetry. Neurological assessment included the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised score. Survival analysis used the Kaplan-Meier method and multivariate Cox model. RESULTS: A total of 315 patients were analysed. Median age at diagnosis was 65.9 years and 55.2% of patients were hypermetabolic. With regard to the metabolic level (ΔREE: < 10%, 10-20% and >20%), patients with ΔREE > 20% initially had a lower FM(29.7% vs. 32.1% in those with ΔREE ≤10%; P = 0.0054). During follow-up, the median slope of Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised tended to worsen more in patients with ΔREE > 20% (-1.4 vs. -1.0 points/month in those with ΔREE ≤10%; P = 0.07). Overall median survival since diagnosis was 18.4 months. ΔREE > 20% tended to increase the risk of dying compared with ΔREE ≤10% (hazard ratio, 1.33; P = 0.055). In multivariate analysis, an increased REE:FM ratio was independently associated with death (hazard ratio, 1.005; P = 0.001). CONCLUSIONS: Hypermetabolism is present in more than half of patients with amyotrophic lateral sclerosis. It modifies the body composition at diagnosis, and patients with hypermetabolism >20% have a worse prognosis than those without hypermetabolism.

Epidemiology of amyotrophic lateral sclerosis: A review of literature.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of motor neurons, resulting in worsening weakness of voluntary muscles until death occurs from respiratory failure. The incidence of ALS in European populations is two to three people per year per 100,000 of the general population. In Europe, crude prevalences range from 1.1/100,000 population in Yugoslavia to 8.2/100,000 in the Faroe Islands. Major advances have been made in our understanding of the genetic causes of ALS, whereas the contribution of environmental factors has been more difficult to assess and large-scale studies have not yet revealed a replicable, definitive environmental risk factor. The only established risk factors to date are older age, male gender and a family history of ALS. Median survival time from onset to death is usually 3 years from the first appearance of symptoms. Older age and bulbar onset are consistently reported to have poorer outcomes. However, there are conflicting data regarding gender, diagnostic delay and El Escorial criteria. The rate of symptom progression has been revealed to be an independent prognostic factor. Psychosocial factors and impaired cognitive function are negatively related to ALS outcome, while nutritional status and respiratory function are also related to ALS prognosis. The effect of enteral nutrition on survival is still unclear, although noninvasive positive pressure ventilation (NIPPV) has been found to improve survival. These findings have relevant implications for the design of future trials.

Diet, Alcohol Consumption and Cognitive Disorders in Central Africa: A Study from the EPIDEMCA Program.

Western research into dementia has focused on finding effective means of prevention, particularly through nutrition. To date, however, little is known about the relationship between diet and cognitive disorders in Africa, where the number of people with dementia is expected to increase most over the coming decades. The objective of the study was to investigate the relationship between diet and alcohol intake and cognitive disorders among elderly people in Central Africa. Between 2011 and 2012, a cross-sectional multicentre population-based study was carried out in rural and urban areas of the Central African Republic (CAR) and the Republic of Congo (ROC). Participants aged ≥65 years were interviewed using the Community Screening Interview for Dementia (CSI-D). Elderly people who performed poorly (COGSCORE≤24.5/30) were clinically assessed by neurologists and underwent further psychometric testing. DSM-IV and Petersen criteria were required for a diagnosis of dementia or mild cognitive impairment (MCI), respectively. A food frequency questionnaire assessed the intakes of dairy products, fruit, vegetables, starches, legumes, oleaginous foods, meat or fish, eggs and sweet foods over the previous three days. We also collected data on alcohol intake. Sociodemographic, vascular, and psychological factors were documented. Multivariate multinomial logistic regression models were used to estimate the associations. In fully adjusted models, a lower consumption of oleaginous foods was associated with MCI (OR=3.7 [1.4-9.9]) and dementia (OR=2.8 [1.0-7.7]) in a rural area of CAR. Alcohol consumption was associated with reduced probability of dementia in CAR (OR=0.3 [0.1-0.8]). In ROC, food groups and alcohol intake were not associated with MCI or dementia. In conclusion, our study provides new data about the association between diet and cognitive disorders in Africa. Further studies should investigate the relationship between diet and cognitive disorders at the level of specific foods rather than food groups.

[Methodology of neuroepidemiological studies in tropical countries: a challenge?]. / Méthodologie des études neuroépidémiologiques dans les pays tropicaux : un challenge ?

The purpose of this paper is to highlight the difficulties of applying neuroepidemiological methods in low income countries or developing countries, which are mostly tropical countries, taking advantage of the experience of the Institute of Neuroepidemiology and Tropical Neurology, which was created in Limoges in 1982. These difficulties could be related to several aspects: methodological, logistical, political or economical, linked to ethical issues, even difficulties to publish the studies. However, concept and neuroepidemiological methods should stay the same worldwide, even if their translation into practice could sometimes raise some problems in developing countries. Study protocol should be more detailed. Some specific epidemiological methods could be useful. Collection of data should be standardized. True cooperation at every level is needed for these researches to be valid.

[Management of stroke in sub-Saharan Africa: current issues]. / Prise en charge des accidents vasculaires cérébraux en Afrique subsaharienne.

In sub-Saharan Africa, stroke is likely to present an increasingly important public health problem with a larger relative share of overall morbidity and mortality. Overall, sub-Saharan Health Care is characterized by a lack of human resources, lack of facilities for special investigations, and especially an absence of specific programs addressing the prevention of cardiovascular conditions. Current data on the epidemiology of stroke in sub-Saharan Africa, although sparse and fragmentary, indicate a comparatively high incidence of cerebral hemorrhage associated with high blood pressure, while ischemic stroke in black Africans still appears to be related primarily to small artery disease, HIV infection, and sickle cell disease. With urbanization, the role of large-vessel atherosclerosis is increasing. It is thus essential to coordinate government funding, health care professionals and development agencies to address this rising health problem. Access to health care needs to be better structured, and screening programs should be developed in order to identify and treat vascular risk factors. Improved training of health care professionals is also required in the areas of prevention, diagnosis and management of stroke. Implementation of best-practice recommendations for the management of stroke adapted to the specificities and resources of African countries would help rationalize the scarce resources currently available.

Social and cultural representation of Epilepsy in elderly aged 65 and more, during a community survey in two French Departments (Hautevienne and Creuse).

BACKGROUND: Epilepsy is often associated with discrediting representations even in the developed countries. The prevalence of disease is 3-5 in thousand. PURPOSE: Our purpose was to assess social and cultural representation of epilepsy in the elderly in two French departments (Haute-Vienne and Creuse). METHODS: An analysis of the answers given by 380 (190 in Haute-Vienne and 190 in Creuse) persons aged 65 and more, selected with the method of quota, during a face to face interview using a questionnaire which explores the representations of epilepsy. The interview was done in 2003 during the local step of an international multi centric community survey entitled "Mental disorders: Image and Realities in community sample". RESULTS: Beliefs in supernatural causes of epilepsy, witchcraft, curse and punishment are more important in elderly aged 65 and more prevalent compared to the younger subjects; it's the opposite for social causes (conflicts, bereavements, stress). The alimentary interdictions are also persistent. Medical assistance keep a good place in the knowledge of the therapy of epilepsy. It showed that those aged 65 and more in Haute-Vienne had social and cultural representation of epilepsy, generally more pessimistic than those of the persons aged less than 65. CONCLUSION: The study confirmed a decrease in the stigma about epilepsy. This data suggest a better consideration of the social and cultural representations in the fight against epilepsy even in western countries.

Prevalence of migraine in a rural community in south Benin.

Migraine is a very common neurological disorder worldwide. Its prevalence is lower in developing countries. There were no data concerning the general population in Benin. We aimed to determine the prevalence of migraine in a rural community of Benin. A cross-sectional study was conducted in a rural area of Abomey from February to April 2003 and included 1113 persons selected by a two-stage survey. A case was defined according to International Headache Society criteria (1988). Of the 1113 persons, 37 had migraine. The lifetime prevalence of migraine was 3.3% (male 2.2%, female 4.0%). The peak prevalence was found in persons in the second decade of life. Higher levels of education were associated with migraine. Migraine without aura was the more frequent form (67.5%). The most frequent triggers of migraine were annoyance (73.0%), exposure to the sun (73.0%), heat (65.0%) and anxiety (51.4%). The low prevalence rate of migraine in Benin confirmed the results of the few available African studies. The disease is severe and occurs in a young population and could lead to a high socio-economical burden.

[Outbreak of konzo disease in health region No. 2 of the Central African Republic]. / Résurgence de la maladie de konzo dans la région sanitaire n(o) 2 en République centrafricaine.

INTRODUCTION: Konzo is a neuromyelopathy characterized by permanent spastic paraparesis, linked to a subacute poisoning by cyanide found in cassava. The purpose of the study is to describe the epidemiological aspects of konzo in health region No. 2 in the Central African Republic. METHOD: A descriptive cross-sectional study was conducted among patients collected during a one-month period (July 16 to August 16, 2007) of active surveillance for acute flaccid paralysis. RESULTS: Eighty-one cases of konzo were identified during the study period, representing a prevalence of 10 per 100,000. Mean age of patients was 10.7+/-7.7 years. Children and women were most affected. The main warning signs were fatigability (97.6%), tremor (88.9%), walking difficulty (100.0%), dysarthria (67.9%) and a loss of visual acuity (65.4%). The predominant neurological signs were lower limb paresis (90.0%) and hyperesthesia (66.7%). CONCLUSION: Konzo is a serious public health problem in this region of the Central African Republic. A prevention program should be set-up.

Diagnosis of peripheral arterial disease in general practice: can the ankle-brachial index be measured either by pulse palpation or an automatic blood pressure device?

BACKGROUND: Despite its validity as a screening test for peripheral arterial disease (PAD), and its prognostic value, the ankle-brachial index (ABI) is infrequently used in primary care, probably because a Doppler device is required, along with the requisite skill for its use. We hypothesized that ABI could be accurately measured either by pulse palpation (pABI) or automatic blood pressure devices (autoABI) instead of Doppler method (dABI). DESIGN AND METHODS: In 54 subjects, we compared the results and the intra-observer reproducibility of pABI to dABI, as well as the inter-observer reproducibility of both pABI and autoABI to dABI. Arm and ankle systolic pressures were measured by the three methods by two observers. The first observer repeated pABI and dABI measurements. The results were compared by the Student paired t-test. Reproducibility was assessed by the intra-class correlation coefficient of agreement (R) and the Bland and Altman method. RESULTS: The mean dABI obtained by the first observers was 1.03 +/- 0.26 vs. a pABI of 0.85 +/- 0.44 (p < 0.0001) and an autoABI of 1.09 +/- 0.31 (p < 0.05). The intra-observer R-coefficient was at 0.89 for dABI vs. 0.60 for pABI (p < 0.05). The inter-observer R-coefficients were 0.79 for dABI vs. 0.40 for pABI (p < 0.05) and 0.44 for autoABI (p < 0.05). CONCLUSION: Neither pulse palpation nor automatic oscillometric devices can be recommended as reliable methods for ABI measurement.

[Electroencephalograms (EEG) in 250 patients with epilepsy in a cysticercosis endemic area in Burundi]. / Electroencéphalogrammes réalisés chez 250 patients épileptiques dans une zone d'endémie cysticerquienne au Burundi.

OBJECTIVES: This work aimed at describing EEG abnormalities in epileptic patients living in areas endemic for cysticercosis, underlining the electroclinical correlations and discussing the interest of EEG examination in this context. METHODS: During a case-control study, 250 EEGs from patients with epilepsy were recorded with a portable system. Types of seizures were assessed clinically and from information obtained through a standardised questionnaire, and along with EEG were related to the results of cysticercosis serological tests. RESULTS: Among the 249 EEGs, 48% were normal, 5.2% had epileptic abnormalities, 6.8% showed an association between epileptic abnormalities and slow alterations. Slow theta and delta abnormalities were found in 21.8% of cases, and isolated deterioration of basic rhythms was observed in 17.3% of cases. Most seizures were generalized, and 61% of the patients had positive serology. One EEG was uninterpretable and another showed isolated spikes. Electroclinical agreement was considered to be satisfactory in 33 patients, and was better with the epileptic than with slow abnormalities. The existence of epileptiform EEG abnormalities confirmed clinically diagnosed epilepsy, but did not allow etiological diagnosis. Electroserological agreement was good in 24 patients. A significant association (Chi2, p = 0.03) existed between slow focal abnormalities and positive cysticercosis serology. Conversely, no significant association was detected between epileptic patterns and serology results. CONCLUSION: While the EEG alone clearly does not allow aetiological diagnosis, its joint use with clinical and biological results was a key element of the etiological and therapeutic discussion. When it shows focal abnormalities in a patient with epilepsy living in a high prevalence cysticercosis area, it confirms the clinical suspicion of neurocysticercosis. Morphological imagery alone can provide etiological information on the seizures by showing the nature and localization of the parenchymal lesions.

[Anxiety and depression among the epileptics in general population in Benin (Western Africa)]. / Anxiété et dépression chez les épileptiques en population générale au Bénin (Afrique de l'Ouest).

In order to assess prevalence of depression and anxiety among epileptic patients and to compare it to a control population, a matched case-control survey was performed in 196 persons above 18 Year old (98 epileptics and 98 controls matched according to sex, age 10 and social environment) in Republic of Benin (West Africa), using Goldberg's Depression and Anxiety scale. Two main investigators helped by 5 sociology students were trained on a questionnaire by a psychiatrist skilled with public health matters. People taking part in the survey are epileptic patients who already used health services. Inclusions took place within 17 communes of four departments (Mono, Zou, Ouémé, Atlantique) located in Southern part of Benin. The questionnaire used an Identity sheet and the Goldberg Depression Scale. Results are shown as mean standard deviations, for quantitative values, and percents for qualitative ones. Comparisons of proportions in qualitative variables are carried out using c2 test or Fisher's exact test. Comparisons of means rates between subject's groups are carried out with a Student t test or variance analysis. The correlations between two quantitative variables were assessed by linear correlation coefficient. Significance threshold chosen for the whole set of statistics analysis is 0.05. The majority of interviewed epileptic patients is young (average 32.6 11.5 Years old). A male predominance exists (sex ratio 1.28). 93% of interviewed persons live within their family, are married or cohabit (controls: 98.2%; cases: 87.9%); 57.4% are married (controls: 70%; cases: 44%). The most represented professional categories are craftsmen and shopkeepers (29.2%) as well as farmers (19.5%). Most of recruited patients live in an urban setting (55.4%) and 63.6% of interviewed persons had been living in the area of survey for over 10 Years. The most represented religion within the sample is Christian religion (67.7%), Animists (23.3%) and Muslims (5.8%). 97% of epileptic patients reported they had one fit during the two Years before the survey; roughly one half (48%) had 2 to 5 fits and 41.5% of them had more than 10 fits; only 14% say they have had an EEG. Presence of an Anti Epileptic Drug (77.5%) reduces anxiety and depression. Considering a severity threshold of 5 for anxiety and 2 for depression (8), proportions of epileptic patients displaying a severe anxiety (79.8%) or a severe depression (89.6%) are significantly higher (p<0.0001) than in control subjects (12.3% and 46.9%). Comparison of average scores confirms the difference (p<0.0001) between cases (5.8 2.0 and 2.3 1.9) and controls (4.7 2.4 and 2.0 2.1) regarding anxiety and depression. Neither the sex, nor age, nor life environment (urban/rural), nor frequency of fits hold significant influence over an-xiety and depression. However, results in this survey include higher average rates of anxiety and depression for women (6.3 1.8) than men (5.5 1.8), though such statement is only nearly significant (p=0.06). Results of the survey confirm the other works on this topic about characteristics of depression for epileptic patients, though results here are higher than usual. Anxiety and depression are common troubles found in epileptic patient, both often occurring at the same time. Two distinct theories about this fact are opposed, first explain the connection of anxiety and depression with epilepsy because of the social and cultural burden upon an epileptic patient in those countries, the second theory is about depression and epilepsy sharing some neuroaminergical dysfunctions; these facts were not considered in this survey. When taking into account the thresholds of seriousness on Goldberg's scale, proportion of controls displaying a probably deeper depression is high (46.9%) compared to usual hospital prevalence rates (4 to 25%) found in Western Africa where survey in general population are scarce. It could either be a bias in the selection or the confirmation that family and relatives of an epileptic patient are enduring a great psychic pain too; finally, this high rate o, this high rate of severe depression within controls may suggest a need to adapt thresholds of Goldberg's scale to match African populations. This hypothesis is interesting regarding the results of some Authors who validated this scale in population of aged people; they highlighted the fewer precision of two items (lost of interest, focus difficulties) and also a slight discriminatory weight for other few items. However the psychometric characteristics of Goldberg's scale are accurate (for depression subscale, a sensitivity of 88% and a specificity of 68%; for the whole scale, a sensitivity of 84% and a specificity of 68%). It would be pertinent to carry out a study to validate Goldberg's scale within some African populations. This study also highlights the importance of psychological factors in epilepsy and suggests a specific global care of the disease.

[French version of structured interviews for the Glasgow Outcome Scale: guidelines and first studies of validation]. / Version française de l'entretien structuré pour l'échelle de devenir de Glasgow (GOS): recommandations et premières études de validation.

OBJECTIVES: The Glasgow Outcome Scale (GOS) is the most widely used outcome measure after traumatic brain injury. The GOS's reliability is improved by a structured interview. The two aims of this paper were to present a French version of the structured interview for the five-point Glasgow Outcome Scale and the extended eight-point GOS (GOSE) and to study their validity. METHODS: The French version was developed using back-translation. Concurrent validity was studied by comparison with GOS/GOSE without structured interview. Inter-rater reliability was studied by comparison between assignments made by untrained head injury observers and trained head injury observers. Strength of agreement between ratings was assessed using the Kappa statistic. RESULTS: The French version and the guidelines for their use are given in the Appendix. Ratings were made for 25 brain injured patients and 25 relatives. Concurrent validity was good and inter-rater reliability was excellent. CONCLUSION: Using the structured interview for the GOS will give a more reliable assessment of the outcome of brain injured patients by French-speaking rehabilitation teams and a more precise assessment with the extended GOS.

[Neurological manifestations of fish, mollusk and shellfish poisoning]. / Manifestations neurologiques des intoxications par les poissons, mollusques et crustacés.

Sea-food poisoning is observed in several areas of the world. Intoxication results from ingestion of fresh fish, mollusks, or shellfish contaminated by toxins produced by microorganisms (dinoflagellates). Neurological manifestations are sometimes associated with signs and may be life-threatening. We describe here the principle toxins, their geographic distribution, clinical manifestations, therapeutic management, and possible prevention measures.

Interferon beta-1a as an investigational treatment for CIDP.

A prospective, multicenter, open-label study was conducted to determine the safety and efficacy of intramuscular (IM) interferon beta-1a (IFNbeta-1a) (Avonex) for treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Eligible patients received IM IFNbeta-1a 30 microg once weekly for 6 months. Safety and tolerability were evaluated by reporting of adverse events, measurement of vital signs, and results of blood chemistry, hematology, and urinalysis. The primary efficacy end points were changed from baseline to month 6 on a quantitative Neurologic Disability Score (NDS), a clinical grading (CG) scale, and grip strength (GS) measures. Electrophysiologic measurements were performed at baseline and month 6. A total of 20 treatment-resistant patients with CIDP were enrolled in the study. The tolerability of IFNbeta-1a in patients with CIDP was similar to that seen with its use in MS. There were no serious adverse events, and no patients discontinued treatment due to adverse events. Seven patients (35%) showed clinical improvement, 10 (50%) had stable disease, and 3 (15%) continued to deteriorate. Significant improvements from baseline were observed in NDS in both the intent-to-treat and per protocol analyses (p=0.0005). For CG, significant improvement from baseline was observed in the per protocol analysis (p<0.05) but not in the intent-to-treat analysis. There was no significant effect of treatment on GS. Clinical improvement was not dependent on age, gender, clinical form of CIDP, or duration of symptoms. Electrophysiologic data showed improvements in mean median, ulnar, and tibial motor nerve potential areas. There was no correlation between clinical improvement and electrophysiologic data. The promising results of this study, especially given the refractory nature of the patient population, suggest that a larger placebo-controlled study should be performed to further evaluate the efficacy of IM IFNbeta-1a for the treatment of CIDP.

[Quality of medical records in the "Medical Information System Program": application of the lot quality assurance sampling method]. / Qualité des données P.M.S.I. au CHU de Limoges: application de la méthode L.Q.A.S.

BACKGROUND: The aim of this study was to validate a new method of quality control of medical records of the "Medicalisation of Information System Program" (P.M.S.I.) in Limoges university hospital. METHODS: Lot quality assurance sampling (L.Q.A.S.), developed to meet industrial quality control needs, has been applied. This method allows to give an opinion on the quality of a lot from a sample randomised in this lot. Ten hospital units have been randomised. During three months, these medical units have recoded ten medical units abstracts (R.U.M.) which were randomised each month within their stays. If five or more R.U.M. had been modified on Principal Diagnosis (D.P.), Associated Significant Diagnosis (D.A.S.) or Diagnosis Related Group (D.R.G.), the lot was rejected. The hospital unit had then less 70% R.U.M. of good quality (alpha=5%). The global errors rates were compared to the results of usually performed quality control (1998). RESULTS: Nine hospital units were included. Hospital units which already had a low quality rate during the 1998 quality control had also an insufficient quality by L.Q.A.S. method. Among these, 33.7 [CI(95%): 21.6-45.8] R.U.M. have been modified. Medical units had a poorer data quality than surgical ones. No statistically significant difference was shown between 1998 errors rates and L.Q.A.S. rates, except for D.P. (p=0.002). CONCLUSION: L.Q.A.S. is easy to apply, could be repeated more frequently than usual controls, and allows to detect hospital units which require a faster action. This process could be applied to P.M.S.I., in which the search of quality is necessary because of the new cost model which includes secondary D.R.G.

Variability of ankle-arm index in general population according to its mode of calculation.

BACKGROUND: Ankle-arm index (AAI) is commonly used in epidemiological studies on peripheral arterial disease but its mode of calculation varies throughout the literature. We aimed to study the variance of the different measurements required to calculate the AAI according to different formulas, in order to find out the best way of AAI calculation, based on its lesser variability. METHODS: A sample of 222 subjects from the general population was prospectively studied. Ten persons were excluded because of the presence of intermittent claudication or due to a lack of a Doppler posterior tibial artery (PT) signal. The systolic pressures of both arms, the dorsalis pedis arteries (DP) and the PT were finally measured in 212 healthy persons (mean age 49.9, range 18-101). RESULTS: Methods using the lowest ankle artery pressure were excluded, as an absent pedal pulse may not be abnormal. The mode using the lowest variance was the choice of the highest pressure between PT and DP of each leg divided by the mean of the systolic pressures of both arms. CONCLUSIONS: We recommend this mode of AAI calculation in epidemiological studies. As the variances of the measurements used are the lowest, it would be easier thereafter to determine normal ranges and cutoff points.