PubMed Health⌕ Search

Biomedical subjects

J Pradas

Publications and source records attributed to J Pradas.

At least 19 recordsLinked to original sources
PubMed2006

[The relationship between lung function and health-related quality of life in patients with generalized myasthenia gravis].

OBJECTIVE: To study lung function abnormalities and health-related quality of life (HRQL) in a group of patients diagnosed with generalized myasthenia gravis, and to analyze the relationship between these 2 sets of variables. PATIENTS AND METHODS: In a prospective study of 20 patients diagnosed with generalized myasthenia gravis, the following parameters were measured: spirometry, static lung volumes, breathing pattern, maximal respiratory pressures, and maximum voluntary ventilation. HRQL was assessed using the 36-item short form general health questionnaire (SF-36). RESULTS: The mean (SD) age of the patients was 64 (11) years. Patients were classified into 2 groups depending on whether they had IIa (12 patients) or IIb (8 patients) type disease. A small decrease in total lung capacity (86%) and slight reductions in maximal inspiratory pressure (88%) and maximum voluntary ventilation (63% in group IIb) were observed. The HRQL domains most affected were those related to physical activity and self-perceived health status in all groups, although women were more affected. The scores relating to vitality and physical activity were found to be significantly associated with forced vital capacity and lung volumes. Tidal volume was associated with maximal inspiratory and expiratory pressures, inspiratory capacity, and maximum voluntary ventilation. The respiratory rate to tidal volume ratio was inversely associated with the first three of these variables. CONCLUSIONS: A very slight restrictive pattern and a reduction in inspiratory muscle strength were observed. The HRQL domains most affected were those related to physical activity and the patients' self-perceived health status. The weakness of the respiratory muscles contributes to the abnormalities observed in lung function and to the deterioration of health-related quality of life.

Aged↗
PubMed2003

Stimulated single-fiber EMG of the frontalis and orbicularis oculi muscles in ocular myasthenia gravis.

We performed single-fiber electromyography by axonal stimulation (stimulated SFEMG) of the frontalis and orbicularis oculi muscles of 20 patients with ocular myasthenia gravis (OM) and 46 controls. In controls, mean consecutive differences (MCD) ranged from 5 to 55 micros (average, 14.7 +/- 2.8 micros) in the frontalis and from 4 to 56 micros (average, 12.56 +/- 2.19 micros) in orbicularis oculi. The mean MCD of individual muscle potentials (MPs) was 14.6 +/- 6.8 micros in frontalis and 12.68 +/- 6.10 micros in orbicularis oculi. In the OM patients, the mean MCD was 43.85 +/- 25.18 micros in the frontalis and 69.85 +/- 29.55 micros in orbicularis oculi (P < 0.0001), and the number of MPs with altered MCD was 7.15 +/- 4.66 (range, 1-18) and 12.65 +/- 4.90 (range, 6-21), respectively (P < 0.0001). We conclude that stimulated SFEMG of the orbicularis oculi muscle is more sensitive for the diagnosis of OM than of the frontalis muscle.

Adult↗
PubMed2000

Stimulated single fiber EMG of the frontalis muscle in the diagnosis of ocular myasthenia.

We performed single fiber electromyography by axonal stimulation (SFEMG-AS) of the frontalis muscle of 16 patients with ocular myasthenia gravis (OM) and 33 controls. In the controls, values of mean consecutive differences (MCD) ranged from 5 to 55 micros (average, 14.7 +/- 2.8 micros) and mean MCD of individual MPs was 14. 6 +/- 6.8 micros. All the OM patients showed abnormal SFEMG-AS jitter before prostigmine was administered (mean MCD: 49.19 +/- 21. 82 micros, percentage of blocks: 20.97 +/- 18.53). Twenty or 30 min after prostigmine had been administered, we saw a significant improvement in jitter: mean MCD was 36.38 +/- 22.49 micros (P = 0. 005), and percentage of blocks was 10.16 +/- 18.87 (P = 0.008). The method was well tolerated. We conclude that SFEMG-AS of the frontalis muscle is a sensitive technique for the diagnosis of OM and is easy to carry out.

Adult↗
PubMed1999

[The reform of primary health care: the economic, care and satisfaction results].

OBJECTIVE: To compare the overall effect on the general public before and after the primary care reform, its economic outcome and professional satisfaction, following the model of the European Foundation for Quality Management. DESIGN: A descriptive analysis of results at reformed primary care centres compared with results at non-reformed centres in the same city. SETTING: The study was conducted at Sant Boi de Llobregat, a town of 77,591 inhabitants in Baix Llobregat county (Barcelona). 32.7% of the population was covered by two reformed centres. The rest was covered by one single non-reformed primary care centre. MEASUREMENTS AND RESULTS: Clinical audits and data on pharmaceutical prescription quality were used to find attendance. For economic results, the formula of attribution of cost/inhabitant and cost/inhabitant seen, including the costs of labour, structure, referral, further tests and pharmacy, were used. The satisfaction of the outside customer (user) was measured by a population survey. Internal customer satisfaction was measured by a survey of the professionals. Results were compared with those for 1997. The study showed that the reformed primary care sector's results, measured in terms of professional satisfaction, user-outside customer, attendance, economic results and social impact, were better than the non-reformed sector's. Inside and outside customers' satisfaction was higher in the reformed network. The cost per inhabitant in the reformed network was 31,874 pesetas, against 25,177 in the non-reformed network. The cost per inhabitant seen was 34,482 and 44,603, respectively. CONCLUSIONS: The reform creates efficient resource management and greater satisfaction of the general public and professionals, when an indicator sensitive to the real use of services is used.

Costs and Cost Analysis↗
PubMed1999

[Electrophysiological evaluation of level compression in elbow ulnar neuropathy].

OBJECTIVES: In order to establish the focal compression in the elbow, a study of 318 patients with clinical manifestations of ulnar neuropathy was carried out. We divided the patients in three groups: I) those who presented mechanical anomalies with background of fracture, elbow valgus...; II) those who did not present mechanical anomalies, but they have been in bed long time or they have been operated...; III) those who did not have evident cause for the compression. MATERIAL AND METHOD: Antidromic sensory conduction and segmentary motor conduction of the ulnar nerve were studied, and segmental motor conduction along the elbow with interval of 2 centimetres (technique of Kanakamedala). RESULTS: 83.6% of the 318 ulnar neuropathy had the focal compression in the ulnar sulcus. 8.2% had the focal compression distal to the ulnar sulcus. 0.94% had double focal compression. In 7.2% was not possible to determinate the focal compression. In group I, the focal compression was in the ulnar sulcus in 93.8% of the cases. In group II, the focal compression was in the ulnar sulcus in 94.3% of the cases. In group III, the focal compression was in the ulnar sulcus in 55.8% of the cases, and distal to the ulnar sulcus in 29.1% of the cases. CONCLUSION: It is possible to localize the focal compression of the ulnar neuropathy in the elbow, with high level of probability, with the nerve conductions. This allows the surgeon to be orientated about therapeutical attitude.

Adult↗
PubMed1998

[Electrophysiological study of 921 cases of carpal tunnel syndrome: its application for prognosis and treatment].

A neurophysiological study of 921 hands with clinical manifestations of carpal tunnel syndrome (CTS), 88 of normal individuals and 588 of patients with disorders not related with median neuropathy was carried out to establish the diagnostic sensibility 130 non-operated on patients with slight CTS were controlled one year later. The same was achieved with 105 surgically treated patients in order to establish the electrophysiological changes related to therapeutic methods. Antidromic sensory conduction and segmentary motor conduction of the median nerve were studied along with the difference between median and ulnar sensory latencies. Only 84 of the 921 hands (5.2%) showed normal electrophysiological findings and 469 (50.9%) minimal changes. 343 hands (37.2%) showed signs suggestive of axonal degeneration in sensory fibers and 147 (16%) in motor ones. Of the 130 slight CTS not surgically treated, clinical manifestations persisted for one year in 118, 17 of which have normal electrophysiological parameters. Twenty six (20%) of these 130 hands got worse. Of the 105 CTS surgically treated hands, 58 continued with symptoms one year later in spite of the electrophysiological improvement in 88.6% of them. Among 588 patients hands without CTS symptoms, only 0.8% had electrophysiological signs suggesting CTS. A high yield of the electrophysiological diagnosis of the CTS is shown. Symptoms frequently persist in patients without definite nerve compression.

Axons↗
PubMed1996

[Amyotrophic lateral sclerosis].

Several aspects of amyotrophic lateral sclerosis are reviewed, with special emphasis on arguments based on etiological and pathogenic theories currently in vogue: free radicals, glutamate-induced toxic excitation, trophic factors, autoimmunity and axon transport. Each of these theories has inspired therapeutic trials, some of which are already completed and others are still in progress. More than one neurodegenerative mechanism may be involved in amyotrophic lateral sclerosis, meaning that it might be necessary to combine several drugs in future trials.

Adult↗
PubMed1996

[Prognostic factors in amyotrophic lateral sclerosis].

Survival in patients with amyotrophic lateral sclerosis is highly variable. In a prospective study of 71 patients, we analyzed the influence of several clinical factors on survival: age of onset, sex, initial involvement (bulbar, upper extremities or lower extremities) and familial history. Mean time of evolution was 2.6 years, with 25% survival 5 years after onset. Patients under 45 years old had better survival than those over 45 (5.8 and 2.2 years, respectively, p < 0.002). The prognosis for women was worse (2.07 and 3.6 years for women and men, respectively, p < 0.001), probably because age of onset was later in women (61 versus 53 years, respectively, p < 0.006). Neither first symptom or familial history of the disease affected prognosis. We conclude that age at onset is a decisive prognostic factor that is inversely related to survival. In the design of clinical trials in which survival is a variable, the treatment and control groups should be matched for age.

Adult↗
PubMed1995

Respiratory function deterioration is not time-linked with upper-limb onset in amyotrophic lateral sclerosis.

INTRODUCTION: In amyotrophic lateral sclerosis (ALS), symptoms apparently spread following regional rules, and depending on the site of onset. We examined if respiratory function deterioration appears earlier or is more severe in patients with upper-limb onset. MATERIAL & METHODS: We compared the results of various pulmonary function tests (PFT) obtained at diagnosis depending on the site of onset in 49 ALS patients. In a longitudinal study, we compared the deterioration of forced vital capacity (FVC) in relation to the site of onset, and analyzed the time elapsed to reach values below 80% of predicted according to site of onset, and we compared the survival depending on the site of onset. RESULTS: No significant differences in PFT were found in the upper-limb onset group in any of the analysis performed. No differences in survival were detected in any disease onset group. CONCLUSION: Pulmonary function deterioration does not appear to be time-linked to arm onset in ALS.

Adult↗
PubMed1994

High-dose dextromethorphan in amyotrophic lateral sclerosis: phase I safety and pharmacokinetic studies.

Much interest has focused on the role of glutamate-mediated excitotoxicity in the etiopathogenesis of amyotrophic lateral sclerosis (ALS). We therefore conducted a phase I study of high-dose dextromethorphan (DM) in ALS. DM is a selective, noncompetitive antagonist of the N-methyl-D-aspartate subtype of the glutamate receptor. Thirteen patients were given DM in an escalating dose fashion, to a target of 10 mg/kg/day or the maximum tolerable dose, and then maintained on this dose for up to 6 months. Total daily doses ranged from 4.8 to 10 mg/kg (median, 7 mg/kg). Side effects were dose limiting in most patients. The most common side effects were light-headedness, slurred speech, and fatigue. Detailed pharmacokinetic and neuropsychology studies were performed. This study demonstrates the feasibility of long-term administration of high-dose DM in ALS, as well as in other conditions associated with glutamate excitotoxicity.

Adult↗
PubMed1993

The natural history of amyotrophic lateral sclerosis and the use of natural history controls in therapeutic trials.

We analyzed the natural history of amyotrophic lateral sclerosis in 277 patients. Our goal was to develop a better understanding of the clinical disease and thus improve the design of therapeutic trials. The Tufts Quantitative Neuromuscular Exam (TQNE) was used as the primary assessment instrument. Our analysis suggested that although more observations are desirable, six monthly TQNEs were adequate to establish the rate of disease progression. We observed a spectrum of deterioration rates without definable subgroups. The striking linearity of deterioration was confirmed. We found a high correlation between deterioration rates in arm and leg strength for individual patients, but a wide range between different patients. Sex and the age at clinical onset did not affect the deterioration rate. As compared with patients without a positive family history, those with other affected family members had a slower loss in arm but not leg strength. We propose that natural history controls can be used effectively in the design of ALS therapeutic trials.

Adult↗
PubMed1992

Clinical status of motoneuron disease does not correlate with serum neurotoxicity on cultured neurons.

To test the hypothesis that a serum factor may be toxic to motoneurons in Motoneuron disease (MND), we evaluated neuronal survival in dissociated cultures from chick ciliary ganglia neurons in which MND or control sera were applied. No neurotoxic activity was found when comparing neuronal survival at 1 and 3 days in MND and control group. To examine the influence of the clinical MND status on neuronal survival, we used the following parameters: age at onset, sex, months from onset of disease to sera sampling, MND form, rhythm of progression, and scoring with Appel scale. No effect was noted for any of these factors. Sera from the same patient obtained at different clinical stages did not allow any conclusion of a fixed behavior for the same patient. Our findings argue against the existence of a cytotoxic factor in sera from MND patients.

Adult↗
PubMed1990

[Prenatal diagnosis of myotonic dystrophy: the first experience in Spain].

We report the first prenatal diagnosis of myotonic dystrophy (MD) in Spain by DNA techniques. The previous familiar study allowed us to determine the DM haplotype in this family with the following probe/enzyme combinations: p4.1/Msp I, LDR 152/Bgl II, Apo CII/Ban I, Taq I, Bam HI, pSCII/Bgl I. In the tenth week of amenorrhea, a transabdominal biopsy was done to obtain chorionic villi. One part of the sample was processed for the cytogenetic analysis that revealed a 46 XY karyotype. The other part was used to perform the molecular analysis with two probes, p4.1 and LDR 152, determining that the fetus was a DM gene carrier with a 96% probability.

Family Health↗
PubMed1990

[Predictive factors of the response to treatment of myasthenia gravis with prednisone].

The influence of age at onset, sex, duration of disease and severity degree on the response to therapy have been evaluated in 30 patients with myasthenia gravis treated with prednisone. During the evaluation period (3 months as a maximum) the patients were only treated with prednisone. The evaluation of the response was based on the use of quantified tests of clinical fatigability. Among all the evaluated factors, only the age at onset seemed to have a predictive value of the response to prednisone therapy. The patients older than 40 years and particularly those older than 60 years showed a better response than younger patients.

Adult↗
PubMed1990

[Genetic analysis of Spanish families with myotonic dystrophy].

Myotonic dystrophy (MD) is the most common form of muscular dystrophy in adult life, with a clinical prevalence of 5.5/100,000, being the gene prevalence of 13.5. The disease is autosomal dominant with variable expressivity, existing a congenital form with a severe prognosis. Recent genetic studies revealed that the DM gene is localized in the chromosome's 19 proximal long arm and with recombinant DNA techniques, several polymorphic markers have been isolated near the gene, the closet being Apo-CII, LDR 152 and p 4.1. We have studied clinically, electrophysiologically and by genetic analysis 6 families with 32 individuals. We have diagnosed the disease in 16 individuals and after the molecular analysis, the DM gene was totally excluded in 6 clinically healthy individuals. In our preliminary study, the use of Msp I/p 4.1, Bgl II/LDR 152 y Ban I, Bgl I, Taq I/Apo-CII c-DNA and genomic was found the most informative combination. These molecular analysis seem to be very useful as a complement to the diagnosis of myotonic dystrophy.

Adolescent↗