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P Couratier

Publications and source records attributed to P Couratier.

At least 19 recordsLinked to original sources
PubMed2006

[Functional rating scales for amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of the peripheral and central motor neurons. The principal consequence is a loss of motor functions. Evaluation of motor deficit implies an assessment of the resulting deficiency or incapacity and final disability. Many evaluation are proposed for patient follow-up in order to analyze the state of motor function and their consequences on activities of everyday life. Few recommendations can be formulated. Scales must be validated and relatively simple to use and generate ordinate results allowing statistical analysis. The choice of which scale to use depends on the clinical objective. Global scales (ALS Functional rating Scale, ALS Severity Scale, Appel scale, Norris scale and Honda scale) can be used to evaluate progression of the disability. Some of these scales are strongly correlated with patient survival. Other scales (ALS Health State Scale, global clinical impression) are used to classify patients by homogeneous stage of gravity. Still other scales, such as the Sadoul and Borg scales and the Epworth score are designed for more specific evaluation of a given function. The clinician should be aware of these different scales and their relative utility. Knowledge of these scales, their validity, their sensitivity to modification, and their specificity and interpretation pitfalls is a prerequisite to good evaluation in daily practice and clinical research.

Activities of Daily Living↗
PubMed2006

[Analysis of reasons for the late diagnosis of amyotrophic lateral sclerosis].

INTRODUCTION: The mean diagnostic delay of amyotrophic lateral sclerosis (ALS) is greater than one year. Its causes are multiple, related to the affection, the patient, or medical practices. METHODS: An investigation was carried out in 77 consecutive patients, to describe their medical course since the date of the first symptoms until the diagnosis. Interrogation of the private practitioners potentially implied in the diagnostic procedure enabled an evaluation of the degree and origin of their knowledge of the disease. RESULTS: In the majority of patients, the first consultation was conducted by a general practitioner. Referral to a neurologist occurred on average 7 months after this consultation. A first-intention electrophysiological examination was prescribed in one-third of patients. Practitioners were unknowledgeable about certain clinical signs. Their knowledge on the disease came primarily from medical school training and for two-thirds, their satisfaction concerning their degree of training was low. CONCLUSION: This observational study shows that medical practices contribute to delayed diagnosis of ALS, particularly delayed referral to a neurologist. To enable earlier diagnosis, general practitioners should be informed of the usefulness of early referral for multidisciplinary care of patients.

Adult↗
PubMed2006

[Announcement of amyotrophic lateral sclerosis diagnosis].

Breaking the news of amyotrophic lateral sclerosis (ALS) is considered as a daunting task in most cases and is not a standardizable procedure. However, proven techniques exist to reduce the trauma to the patient. Announcing ALS falls upen the neurologist who must respect the ethical principle of the patient's independence. After the diagnosis is firmly established, the patient should be informed that he or she has a progressive disease of the motor nerves, for which no curative therapy is available. The name of the disease must be stated and explained. If the family history is negative, it is reassuring for the patient and family to know that their children are unlikely to be at risk. Positive aspects (no pain, no disturbances in sensation, cognition, memory and continence) should be stressed as well as the availability of efficient palliative measures for practically all symptoms. Current research efforts, and when available, the possibility of taking part in clinical studies of new drugs should be pointed out as a means of hope. The answer to the question of prognosis should include the information that there are no sudden worsenings to be expected, that the course of ALS may vary between months and decades, that making a firm statement on prognosis all but impossible for any single patient and that respiratory function may worsen during the disease course. It is therefore mandatory to inform patients and families about the existence of ALS patients'associations. The way the patient is told the diagnosis is of great importance and is considered as a multiple-step procedure. Discussion should take place in a private and quiet room and respect some fundamental objectives such as finding out what the patient already knows or suspects and how much more the patient wants to know, observing and responding to the patient's reactions, reinforcing the information and planning the future. It is proven that communicating the diagnosis of ALS in an empathetic fashion is an important and sensitive step to disease management.

Amyotrophic Lateral Sclerosis↗
PubMed2006

[Nutritional assessment in amyotrophic lateral sclerosis patients].

During ALS, malnutrition may occur. The causes are multiple. A reduction in caloric and protein intakes, due to swallowing disorders, play a major role. Moreover, (2/3) of patients develop an hypermetabolism of unknown cause, leading to an increase in resting energy expenditure. Malnutrition, whose prevalence is 10-55 percent, is an independant significant prognostic factor for survival. Nutritional assessment is recommanded every 3-6 months at least, and more often if needed. After a simple clinical examination, nutritional assessment includes the measurement of weight (W) and height (H), in order to calculate the Body Mass Index (BMI=W/H(2)). BMI<18.5 (age: 18-65ys) or<20kg/m2 (age>65ys) indicates malnutrition. A weight loss more than 5 to 10 percent of usual weight in the last six months is another malnutrition criterion. The measurement of skinfolds and the calculation of mid arm muscle circumference give informations on respectively fat mass and fat-free mass, but their interest is mainly during the follow-up, or for triceps skinfold when included in a validated bioimpedance formula. Bioimpedance analysis (BIA) is a simple bedside technique, recently validated for measurement of fat-free mass in ALS patients. The BIA phase angle could be an easy obtained parameter of severity. Dietary interview is important when patients still use oral route, but is difficult if they have elocution or writing problems. Indirect calorimetry and dual X-ray absorptiometry give reliable informations on respectively energy expenditure and body compartments, but remain scarcely used. Additional methods, like swallowing tests, psychological, digestive or respiratory assessments are often useful for optimal nutritional prescriptions. We recommand as minimal nutritional assessment a systematic clinical examination, anthropometric measurements and bioimpedance evaluation.

Amyotrophic Lateral Sclerosis↗
PubMed2004

[Review of nutritional and respiratory support in ALS].

During the evolution of amyotrophic lateral sclerosis (ALS), quality of life and survival of patients are related to respiratory and nutritional status. After diagnosis, a multidisciplinary care has to be promptly organized and coordinated by the referring neurologist. The nutritional and respiratory support imply that neurologists know their specific means of evaluation with their sensitivity and sensibility and be able to recognize clinical risk situations. The informations of patients on assisted-ventilation and nutritional support by using gastrostomy may be done early, precisely and trustfully. Well informed patient's choices must be respected. Nutritional and respiratory supports may be based on recommendations established by the American Academy of Neurology. This review will present and discuss their main aspects in patients with ALS.

Amyotrophic Lateral Sclerosis↗
PubMed2004

[Involvement of the peripheral nervous system in multiple sclerosis].

Multiple sclerosis is a demyelinating disease limited to the central nervous system, but the literature has provided recurring evidence which raises the question of associated peripheral nervous system abnormalities. The prevalence of peripheral neuropathy during multiple sclerosis remains controversial without prospective study. Nevertheless, some data have reported well documented case reports describing the co-occurrence of multiple sclerosis and radiculopathy or mononeuropathy or polyneuropathy in the same patients. By contrast, more frequent subtle nerve abnormalities may be found by using electrophysiological and neuropathological examinations. Some hypotheses have been proposed by Waxman to decipher the electrophysiological and neuropathological findings. The mechanisms for demyelinating disease and peripheral nerve pathophysiology may imply the antigenic properties or the presence of diffusing factors between peripheral nervous system and central nervous system myelin and the molecular plasticity of myelinated fibers.

Antigens↗
PubMed2003

[Isolated neurosarcoidosis without systemic signs].

Sarcoidosis is a multisystemic disease which involves the nervous system in 5 to 15 p.cent. Neurosarcoidosis without signs of systemic disease is rare and may be difficult to diagnose. We report a case of a 61 year-old patient with a pseudotumoral neurosarcoidosis and along evolution of 25 years without systemic signs. In such cases, histological analysis is rewarding.

Adrenal Cortex Hormones↗
PubMed2003

[Glycogenesis due to adult phosphorylase kinase deficiency].

A 42-year-old man presented exercise-induced muscle pain without myogloburia since the age of 12 years. Histochemistry and electronmicroscopy of a muscle biopsy revealed subsarcolemmal and inter-myofibrillar accumulation of glycogen. Exercise on a bicycle ergometer produced a normal raise of lactate. Biochemical study showed a partial defect in phosphorylase activity.

Adult↗
PubMed2002

SMN1 gene study in three families in which ALS and spinal muscular atrophy co-exist.

Spinal muscular atrophy (SMA) is caused by SMN1 gene deletions or mutations, and ALS is the most frequent motor neuron condition in adults. The authors describe three families in which ALS and SMA coexist. The authors found that no SOD1 mutation was found within these families; all three ALS cases had at least two SMN1 copies; and an abnormal SMN1 gene locus did not explain the co-occurrence of these two motor neuron disorders in these families.

Amyotrophic Lateral Sclerosis↗
PubMed2002

[Validation of the combined SF36/MSQOL test of evaluation of quality of life in migraine patients in France].

The impact of migraine on patients quality of life is great, even between attacks. The aim of this study was to assess the properties of a French language quality of life questionnaire, which associates the Medical Outcome Study Short Form (MOS SF 36) with the Migraine-Specific Quality of Life (MSQOL). Data were obtained from 110 consultant patients of neurology units located in three University hospitals in the center of France. The results indicated a good acceptability of the association of the two questionnaires with a 84p.cent response rate. The Principal Component Analysis showed no change in both SF-36 and MSQOL structures. It also suggested that the two questionnaires were complementary. The high values of the Cronbach alpha coefficients for each scale indicated a high level of internal consistency. The quality of life scores were correlated with external parameters such as frequency, intensity and length of migraine attacks, and social activity reduction due to these attacks. In conclusion, the association of the SF-36 with the MSQOL has the interest of associating generic and specific questionnaire, in a complementary way. This association has good psychometric properties, and can be used to evaluate migraineurs quality of life in French speaking countries.

Adult↗
PubMed2001

Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a severe disease characterized by neurogenic amyotrophy and degeneration of upper and lower motor neurons. Although ALS patients usually experience reductions in fat-free mass (FFM), hypermetabolism via an undetermined source has also been reported. OBJECTIVE: The objective was to clarify the metabolic level of ALS patients. DESIGN: We measured the resting energy expenditure (REE) of 62 patients (32 men and 30 women) with ALS and investigated the factors correlated with metabolic level. Nutritional evaluation included bioelectrical impedance analysis, indirect calorimetry, and calculation of the body mass index. Neurologic assessment included an evaluation of peripheral and central neurologic deficit. Forced vital capacity was measured and smoking status was noted. A complete blood cell count was made and thyroid hormone and C-reactive protein concentrations were measured. RESULTS: Patients were hypermetabolic, by an average of approximately 10% more than in a reference healthy population. FFM, age, and the neutrophil count were significantly associated with REE. The only variable that contributed to the prediction of REE, REE/Z100 kHz (bioimpedance at 100 kHz), REE adjusted for FFM, or the ratio of measured REE to calculated REE was the neutrophil count, which explained only a small percentage of variance in the multiple regression analysis. Hypermetabolism was not associated with a reduction in respiratory function, tobacco use, hyperthyroidism, spasticity and fasciculation intensities, or infection. CONCLUSIONS: Our study corroborates the surprising finding that ALS patients are hypermetabolic. FFM, age, sex, manual muscular testing, the modified Norris limb score, weight, and an increase in circulating neutrophil counts correlated with the hypermetabolic state. Other factors may play a role in pathophysiologic processes that involve mitochondrial energy production or even sympathoadrenergic activation.

Adult↗
PubMed2001

Motor neuron disease after electric injury.

The occurrence of motor neuron disease after electrical injury in six patients is reported and compared with patients from the literature. The patients were five men with spinal onset and one woman with bulbar motor neuron disease after electric shock. Two patients were struck by lightning and four by industrial electric shock. For all six of them, the disease started at the site of the electrical trauma. The mean delay for onset of motor neuron disease was 44 months. In four of the spinal patients the disease progressed slowly with mild handicap after several years. For the fifth patient, improvement was noted progressively. The patient with bulbar disease died 26 months after onset. A link between electric shock and motor neuron disease is likely, given the homogenous profile of the patients both in the five spinal cases presented here and in the literature. Bulbar onset has not been reported to date. However, in this patient the long delay between the electrical injury and motor neuron disease, together with the rapid evolution may suggest a chance association.

Adult↗
PubMed2000

In vitro induction of neuronal apoptosis by anti-Fas antibody-containing sera from amyotrophic lateral sclerosis patients.

Sera from 26% of patients with sporadic amyotrophic lateral sclerosis (ALS) induced in vitro apoptosis of a human neuroblastoma cell line, as detected by two methods, and most contained anti-Fas autoantibodies. In contrast, Alzheimer sera (studied as controls) very rarely induced apoptosis and did not contain detectable anti-Fas antibodies. Soluble Fas-ligand levels in ALS sera were not different from those in normal sera, except for slightly higher levels in a single case. In mixed cultures of rat embryonic brain and spinal cord cells, ALS sera (and agonistic anti-Fas monoclonal antibodies and soluble Fas-ligand) induced the apoptosis of a subpopulation of neurons. These neurons were motoneurons on the basis of staining with the monoclonal antibody SMI 32 and Fas expression was restricted to these SMI 32-positive neurons. These data are compatible with the hypothesis of the participation of an autoimmune mechanism possibly related to anti-Fas autoantibodies in certain ALS patients.

Amyotrophic Lateral Sclerosis↗
PubMed2000

Prevalence and clinical significance of anti-phospholipid antibodies in multiple sclerosis: a study of 89 patients.

The prevalence of serum anti-phospholipid antibodies (aPL) was evaluated in multiple sclerosis (MS) patients to search for a possible association with a distinct form of the disease. Anti-cardiolipin antibodies (Ab) (aCL) and anti-beta 2 glycoprotein I Ab (abeta2GPI) were measured together with antinuclear Ab (ANA), anti-double-stranded DNA Ab (anti-ds DNA) and anti-myelin Ab in 89 patients. Twenty-nine (32.6%) patients had serum aPL, 19xaCL (15 of the IgG and four of the IgM isotype), 14 abeta2GPI (two IgG and 12 IgM) (four of these patients had both Ab). Prevalence of aCL correlated with that of ANA, which were positive in 52 patients (P=0. 005) and with anti-myelin Ab detected in two patients (P=0.046) but not with that of anti-ds DNA (mostly of the IgM class) detected in 28% of case by ELISA. No correlation could be found between aPL and age, sex, duration of the disease from diagnosis, category of MS, clinical course, clinical symptoms, serum IgM levels nor atypical lesions by magnetic resonance imaging. Hence, aCL and abeta2GPI are neither rare in MS nor associated with a specific clinical form of the disease and they cannot be a diagnosis exclusion criteria.

Adult↗
PubMed2000

Estimation of the amyotrophic lateral sclerosis incidence by capture-recapture method in the Limousin region of France.

Amyotrophic lateral sclerosis (ALS) is a rapidly fatal degenerative neurological disease categorized among motor neuron diseases. In the literature, the incidence of ALS varies between 0.7 and 2.4 per 100, 000 inhabitants. A study using the capture-recapture method (multiple records system analysis) was undertaken in Limousin (France) ascertaining all patients having onset of definite or probable ALS during the period 1994-1995. Three information sources able to identify these new ALS cases were selected: the first source was a computerized database of the Neurology Department of the University Hospital of Limoges; the second source consisted of the neurologists of the Limousin region and neighboring provinces (county-sized regions); the third source grouped the hospitals of the Limousin region and neighboring provinces (county-sized regions). During this period, 46 new cases of ALS were seen, corresponding to an observed mean annual incidence of 3.2 (+/-0.6) per 100,000 inhabitants. After standardization for age, the annual incidence was 2.5 per 100,000 inhabitants. The number of new cases estimated by the capture-recapture method was 70, corresponding to an estimated mean annual incidence of 4.9 (+/-1.0) for 100,000 inhabitants. Hence, statistical modeling utilizing partially overlapping information sources permitted a more exhaustive compendium of the new cases of ALS and may be a truer reflection of actual disease incidence than has been previously reported.

Amyotrophic Lateral Sclerosis↗