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Biomedical subjects

P Couratier

Publications and source records attributed to P Couratier.

At least 37 records · Page 2Linked to original sources
PubMed2000

Nutritional assessment and survival in ALS patients.

Malnutrition, present in 16-50% of ALS patients, is an independent prognostic factor for worsened survival. It is caused primarily by swallowing dysfunction, resulting from involvement of the lower sets of cranial nerves, but hypermetabolism is also implicated. Malnutrition itself can produce neuromuscular weakness and adversely affect patients' quality of life, thereby creating a vicious circle. The nutritional status of ALS patients can be assessed with dietary review and measurements of weight (W) and height (H). A body mass index (BMI = W/H2) below 18.5-20 kg/m2 indicates a state of malnutrition. Dietary counselling is important, but rapidly becomes insufficient, particularly in bulbar-onset ALS, where enteral nutritional support is then necessary. Percutaneous endoscopic gastrostomy tube placement is well tolerated, and provides more efficient enteral nutrition than nasogastric tube feeding. Enteral nutrition support can improve the respiratory status of ALS patients. The effect on survival remains to be confirmed.

Aged↗
PubMed2000

[Case study of 199 patients with multiple sclerosis: the use of EDMUS program].

European Database Multiple Sclerosis (EDMUS) is a standardized tool which allows the collection of clinical, biological, radiological and therapeutic data on multiple sclerosis (MS). A descriptive analysis of 199 patients was done using EDMUS (version 2.2). Most data agreed with previous published results, except a low 0.4 sex ratio. At the beginning of MS, 42 p. 100 of MS patients developed pyramidal and sensory involvements and 14 p. 100 retrobulbar optic neuritis. Optic neuritis was the most frequent initial symptom of relapsing-remitting MS (25.9 p. 100) but not in the chronic progressive form (5.5 p, 100; p < 0.01). Surprisingly, relapsing-remitting form occurred more frequently whereas the secondary progressive form was diagnosed less. The concordances between the three disability scales, EGS (EDMUS Grading Scale), EDSS (Expanded Disability Status Score) and AI (Hauser Ambulation Index) were correct. In clinical practice, EDMUS is user-friendly and rapid. The lack of radiological data and the necessary quantification of only one disability scale, EGS may be too restrictive. The analysis of most of clinical data is possible by using integrated selection within the software, but sometimes needs more complex procedures.

Databases as Topic↗
PubMed2000

[Interest of a computerized ALS database in the diagnosis and follow-up of patients with ALS].

The aim of the study is to present a computerized database of the Neurology department of Limoges University Hospital and the main results obtained from data of 340 patients suffering from amyotrophic lateral sclerosis (ALS), diagnosed between 1984 and 1997. It is a user friendly and can be accessed by all neurologists at any level of computer knowledge. This database is modular (6 modules) and flexible according to need. The software used, Access 7, is an open relation database, which allows export of data to statistical or other compatible software. One of the reasons, which led to the elaboration of this database was to develop a means of collecting data in an analyzable manner for therapeutic trials. During these trials, a great number of data can be collected during each clinic visit for the evaluation of the degree of impairment, disability, and handicap. We present part of the data from the patients followed, some of whom were treated with riluzole, the current reference molecule for ALS.

Aged↗
PubMed1999

Nutritional status is a prognostic factor for survival in ALS patients.

OBJECTIVE: To evaluate the occurrence of malnutrition in patients with ALS, to assess the relation of malnutrition to the neurologic deficit, and to determine the impact of nutritional status on patient survival. BACKGROUND: Although ALS may be associated with significant malnutrition, the relative impact on patient survival has not yet been well established. METHODS: In a prospective 7-month study of 55 ALS patients in a referral neurology practice, nutritional status was assessed by calculating body mass index. Neurologic evaluation includes four functional scores and identifies the form of disease onset. Slow vital capacity (VC) was also measured. RESULTS: Occurrence of malnutrition in patients studied was 16.4%. Survival (using the Kaplan-Meier method) was worse for malnourished patients (p < 0.0001), with a 7.7-fold increased risk of death. Using multivariate analysis, only reduced VC (p < 0.0001) and malnutrition (p < 0.01) were found to have significant independent prognostic value. The degree of malnutrition is independent of neurologic scores and of forms of ALS onset. CONCLUSION: Nutritional surveillance of ALS patients is very important, both in bulbar-onset and spinal-onset patients.

Aged↗
PubMed1999

[The involvement of myelin proteins in hereditary neuropathies].

Hereditary sensoro-motor neuropathies such as Charcot-Marie-Tooth disease (CMT) form a heterogeneous group including some genetic conditions whose clinical manifestations differ in severity within a group or even within a sub-group. Diagnosis is based on the clinical, electrophysiological and pathological findings along with a genetic analysis. The current classification of CMT encompasses the clinical signs, mode of transmission, genomic localization and identification of the proteins actually involved. Several authors have identified the mutations on genes coding for proteins of peripheral myelin in CMT patients. Recent advances in molecular genetics have thrown more light on the differences between phenotypes within a sub-group, and have established genotype-phenotype relationships. It has been shown recently that the severity of the clinical signs depends on the nature and site of various mutations affecting the genes coding for certain myelin proteins. These mutations give rise to "dominant negative effects" or "mutations with loss of function of the allele". These observations may be extended to other proteins as many of them belong to the super family of immunoglobulins and have similar structures. In this study, we present a review of the literature focussing on the principal myelin proteins and the genomic modifications observed in patients with CMT.

Alleles↗
PubMed1999

[Cerebral miliary granulomatosis with Histoplasma capsulatum in an HIV seronegative patient].

A 51 year old patient who worked in Africa for eight years, presented twelve years later a progressive ataxia associated with headaches. Neuroimaging studies done after a partial complex seizure demonstrated multiple supra and sub-tentorial cortical ring enhancing lesions. Histoplasma capsulatum histoplasmosis was found on histological examination of brain biopsy and confirmed by isolation of the fungus. Medical treatment with intravenous amphotericin B followed by oral itraconazole (400 mg per day) improved both clinical and radiological status. This observation of cerebral histoplasmosis is rather unusual for a seronegative HIV patient in a non endemic area.

Amphotericin B↗
PubMed1998

Serum autoantibodies to neurofilament proteins in sporadic amyotrophic lateral sclerosis.

Anti-neurofilament (NF) autoantibodies were searched for by enzyme-linked immunosorbent assays (ELISA) in the serum from 85 sporadic amyotrophic lateral sclerosis (ALS) patients, 98 healthy controls and 79 patients with unrelated immunological diseases (Guillain-Barré syndrome, myasthenia gravis and multiple sclerosis). ELISA cutoff value was determined as mean control levels +2 SD and it corresponded to a specificity of 94%. Such high level antibodies were detected in 24.7% of ALS patients contrasting with 12.6% of neurological controls (P<0.05) and only 6.1% of healthy subjects (P<5.10[-4]). In ALS, anti-NF antibodies were significantly associated with a slow evolution, as measured by the mean time spent in the initial functional states. They did not relate with age, sex and clinical form. The predominant isotype of the anti-NF antibodies was IgM lambda by ELISA. In contrast to negative sera, indirect immunohistochemical studies demonstrated that most sera positive for anti-NF antibodies reacted with axons with predominant isotypes restricted to IgM lambda. By using Western blotting, small amounts of serum monoclonal IgM were found with a high frequency in anti-NF antibody-positive patients. These results suggest the possible involvement of anti-NF antibodies in an autoimmune process in a subgroup of ALS patients.

Aged↗
PubMed1998

[Lead intoxication and amyotrophic lateral sclerosis].

A seventy-six-year-old patient developed a bulbar form of amyotrophic lateral sclerosis, after chronic lead intoxication from drinking water. Treatment with 2,3 dimercaptosuccinic acid was administered for six months and had no effect on clinical course. We discuss the imputability of lead as a possible etiological factor.

Aged↗
PubMed1997

Mutations in the X-linked form of Charcot-Marie-Tooth disease in the French population.

The present study reports eight additional mutations in the connexin32 gene associated with the X-linked form of Charcot-Marie-Tooth disease. One of these mutations was found twice in two apparently unrelated families. This form of the disease is demyelinating and dominant. However, patient selection for mutational screening should not be limited to these criteria since presentation can either be familial or sporadic, and some patients may be incorrectly classified as suffering from an 'axonal' form. These new mutations complete our previously published work on 12 other mutations and enable meaningful observation in a representative sample of the French population. Mutations are found in all regions of the gene. The most frequently observed mutations were those affecting arginines and mainly involved CpG sequences. Compared with other sources, some of the mutations were present at a higher frequency in the French population.

Adolescent↗
PubMed1996

Phorbol ester enhances phosphorylated tau protein immunoreactivity in neuronal cultures.

One of the hallmarks of Alzheimer's disease (AD) is neurofibrillary degeneration which results from the aggregation of phosphorylated tau proteins into paired helical filament (PHF) structures. AD2 is a new monoclonal antibody raised against PHF tau which detects neurofibrillary tangles in AD brain. In primary neuronal cultures, phorbol ester treatment induced a time- and dose-dependent increase in AD2 immunoreactivity quantified by laser confocal microscopy and immunoblottings. Alkaline phosphatase treatment reversed these immunocytochemical changes. These results suggest that the modifications of neuronal metabolism induced by phorbol ester including protein kinase C activation produce an increase in phosphorylated tau immunoreactivity.

Animals↗
PubMed1996

Ultrastructural PMP22 expression in inherited demyelinating neuropathies.

Charcot-Marie-Tooth type 1A (CMT-1A) disease results from a duplication of the PMP22 gene on chromosome 17p11.2. A deletion of the same region causes hereditary neuropathy with liability to pressure palsies (HNPP). We examined the expression of PMP22 in sural nerve biopsies from 2 unrelated patients with CMT-1A, 2 unrelated patients with HNPP, and control patients. The ultrastructural immunocytochemical quantitative analysis of cases of CMT-1A and HNPP showed, respectively, an elevated and reduced expression of PMP22 level compared with controls.

Charcot-Marie-Tooth Disease↗
PubMed1996

Modifications of neuronal phosphorylated tau immunoreactivity induced by NMDA toxicity.

Glutamate toxicity has been involved in the pathophysiology of a large variety of neurodegenerative disorders. Tau Protein is a micro-tubule-associated protein that promotes microtubule polymerization and stabilization. Phosphorylated tau protein accumulates in paired helical neurofilaments, the major constituent of neurofibrillary tangles observed in the brain of patients suffering from Alzheimer disease (AD). In this study, using confocal laser microscopy and immunoblot analysis, we report that acute (500 mu M for 15 min) or chronic (20 mu M for 16 h) N-methyl-D-aspartate (NMDA) neuronal toxicities modify the immunoreactivity of phosphorylated tau. Neuronal degeneration produced by N-methyl-D-aspartate is associated with an augmented immunolabeling of phosphorylated tau proteins at serine 202 (AT8 antibody) as observed in paired helical neurofilaments. This finding could help to determine the cellular mechanisms at the origin of neuronal degeneration associated with modifications of phosphorylated tau immunoreactivity produced by receptor-mediated extracellular signals.

Alzheimer Disease↗
PubMed1996

Link between peripheral neuropathy and monoclonal dysglobulinemia: a study of 66 cases.

The association of peripheral neuropathy (PN) and monoclonal dysglobulinemia has often been reported, although a direct link between the two is not readily established. Linkage is generally based on consideration of the clinical, electrophysiological and immunological findings along with details of the course. We report here a study of 66 of our own cases which benefitted from immunological and immunopathological analyses of serum. In 62 of these cases, histological and immunopathological examinations of nerve biopsy specimens were realized. Such a discussion about a link between the dysglobulinemia and the peripheral neuropathy is of clinical interest as it provides information for decisions about the continuation of potentially neurotoxic chemotherapy. In this prospective study conducted on a case by case basis, the origin of the neuropathy, whether due to the chemotherapy, the dysimmune process, the presence of abnormal immunoglobulins in the nerve, amyloid deposits, infiltration of nerve parenchyme by abnormal cells, or a combination of one or more of these mechanisms, could be determined in most cases. We divided our population of 66 patients into 4 etiological groups: group 1: direct link (56.1%), group 2: indirect link (12.7%), group 3: no link (10.6%), group 4: doubtful link (21.2%). In group 1 we found a statistically significant association between the peripheral neuropathy and an IgM kappa MGUS.

Humans↗
PubMed1996

NMDA antagonist blockade of AT8 tau immunoreactive changes in neuronal cultures.

Antagonists at four distinct regulatory sites on the N-methyl-D-aspartate (NMDA) receptor were tested for their ability to attenuate NMDA-mediated chronic excitotoxicity and the consequences on AT8 tau immunoreactivity in neuronal cultures. Excitotoxicity was monitored in cultures by diacetate fluorescein staining. Immunoreactivity of tau phosphorylated at serine 202 was quantified by laser confocal microscopy. The NMDA-receptor antagonists MK801, AP7 and 7-chlorokynurenate significantly blocked NMDA-induced cell death and significantly reduced AT8 tau immunoreactivity. NMDA antagonism by the polyamine site antagonist, ifenprodil, did not completely reverse the increase in AT8 tau immunolabeling induced by NMDA and did not completely protect NMDA-sensitive neurons, suggesting an heterogeneity in the NMDA receptor population.

2-Amino-5-phosphonovalerate↗
PubMed1996

Survival prediction in sporadic amyotrophic lateral sclerosis. Age and clinical form at onset are independent risk factors.

Amyotrophic lateral sclerosis is a progressive neurological disease of unknown etiology and fatal outcome. Patient management can be aided by careful assessment of prognostic factors. A prospective study of 158 patients was carried out to examine the prognostic significance of age and clinical form at onset. The overall 5-year survival rate was 14.7%. The higher the age was at first symptoms, the worse the prognosis. The bulbar and common forms had a worse prognosis than the pseudo-polyneuritic forms. After adjustment for age, the clinical form at onset remained a prognostic factor. In a multivariate analysis using the Cox model, these two factors remained independent despite the later onset of the bulbar forms. In view of the discrepancies between the different published studies, the evaluation of the survival of an individual patient is of doubtful value.

Age of Onset↗