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Biomedical subjects

M Vanasse

Publications and source records attributed to M Vanasse.

At least 55 records · Page 3Linked to original sources
PubMed1984

Intermittent treatment of febrile convulsions with nitrazepam.

Intermittent oral or rectal administration of diazepam for the prophylactic treatment of febrile convulsions has given results comparable to the continuous use of phenobarbital while limiting side effects and risks of toxicity. Since we believe that nitrazepam is a better anticonvulsant than diazepam, we performed a study to evaluate the effectiveness of this medication in the prophylactic treatment of febrile convulsions. Nitrazepam was given only when the children had fever and almost exclusively in children with a high risk of recurrence (less than 12 months of age at first convulsion; atypical convulsion; one or several previous convulsions). Thirty one children with a high risk of recurrence received nitrazepam. The rate of recurrence in this group was 19.3% after a follow-up of 16 months, compared to 45.8% in 24 children who also had a high risk of recurrence but in whom the parents refused the medication or gave it inadequately (p less than 0.05). Fifty one children with a low risk of recurrence also were evaluated and followed for at least 12 months (mean 15.4 months). Six were treated with nitrazepam, mostly because of parental anxiety, and none had a recurrence; of the 45 untreated children in this group, 6 (13.6%) had another convulsion. These results show the efficiency of nitrazepam in the prophylactic treatment of febrile convulsions.

Akathisia, Drug-Induced↗
PubMed1984

Recurrent aseptic meningitis secondary to intracranial epidermoids.

Two patients with recurrent aseptic meningitis are reported. Intracranial epidermoid tumors were eventually found in both patients. CSF changes in our first case with an epidermoid tumor were similar to those reported in Mollaret's meningitis, with an initial polymorphonuclear pleocytosis early in each attack, followed by a lymphocytic predominance several days later. Large cells which proved to be macrophages were also seen in the CSF of this patient. Both patients had meningitis attacks for several years before the underlying epidermoid tumor was found. Mollaret's meningitis remains a diagnosis of exclusion. These patients should remain tumor suspects for many years and deserve careful and repeated investigation.

Brain Neoplasms↗
PubMed1984

Impaired muscle differentiation in explant cultures of Duchenne muscular dystrophy.

Muscle cultures were established from 11 normal controls and 13 Duchenne muscular dystrophy biopsies by an explant technique. Under light microscopy the most distinctive features of Duchenne muscular dystrophy cultures were an earlier migration and a delay or even an absence of fusion of mononucleated cells in comparison with the controls. Furthermore, most fusing dystrophic cells were morphologically different from conventional myoblasts, and, at a later stage, myotube formation was significantly reduced. In contrast to controls, dystrophic cultures displayed a total absence of spontaneous contractions and poor myofiber longevity. Atypical myogenic cells seen in Duchenne muscular dystrophy cultures differed ultrastructurally from normal myoblasts in having a smaller population of free ribosomes and polyribosomes, prominent rough endoplasmic reticulum, and abundant intermediate filaments. The dystrophic myofibers that had reached relative maturity always exhibited a poorly developed contractile apparatus in contrast to structurally well-defined normal myofibers. It is inferred that the impaired myogenesis observed in Duchenne muscular dystrophy cultures results primarily from defective fusion involving atypical myogenic cells.

Adolescent↗
PubMed1982

Oral lecithin and linoleic acid in Friedreich's ataxia: I. Design of the study, material and methods.

A clinical and biochemical evaluation of twenty-two patients with Friedreich's Ataxia and ten normal controls was undertaken in 1980 to assess the effect of lecithin and linoleic acid supplements on the course of the disease. The trial consisted of two consecutive six months periods on either supplements in a double-blind crossover fashion. Clinical appraisal was performed with regards to the following parameters: joints mobility, muscle strength, equilibrium, coordination, motor accuracy, speech and numerous day to day activities. Blood samples were obtained at the beginning and in the course of the trial for enzymatic determinations. This paper describes the methodology of the study.

Administration, Oral↗
PubMed1982

Oral lecithin and linoleic acid in Friedreich's ataxia: II. Clinical results.

Twenty-two patients with Friedreich's Ataxia and ten normal controls were followed for one year and assessed as to their clinical performance after two successive six-month periods of lecithin or safflower oil. Results demonstrated no significant difference in performance scores according to group assignation, neither in patients nor in controls. According to stages, two patients in stage I and to a lesser degree, one patient in stage IV showed better scores for muscle strength and some motor accuracy and coordination tests with lecithin. Controls as groups maintained positive scores in all tests. Patients as groups showed negative mean values in nine out of eleven tests. Again as groups, patients receiving safflower oil demonstrated a mean 8% less deterioration than patients receiving lecithin. This study demonstrates that objective clinical tests and the participation of normal controls are a must in a therapeutic trial implicating patients with a progressive disorder such as Friedreich's Ataxia. The possible role of linoleic acid as the active factor from which clinical improvement proceeded in some specific patients and with early functional stages of the disease, has to be considered and reevaluated in the near future.

Administration, Oral↗
PubMed1982

Oral lecithin and linoleic acid in Friedreich's ataxia: III. Biochemical results.

Lecithin and safflower oil brought about the same changes in serum LAD activity and kinetics in patients with Friedreich's Ataxia as in controls when results of this double-blind crossover study were analyzed according to group assignation. According to functional stages, pretrial LAD activity decreased with advancing severity while Km for lipoamide increased. Lecithin and safflower oil supplements corrected the elevated Km for lipoamide but produced a further reduction in LAD activity. These changes may have been due to the increased intake of linoleic acid, a precursor of lipoic acid, which is present in high percentage in both lecithin and safflower oil. Results of the biochemical study thus agreed with the clinical data gathered during the course of the one-year trial in suggesting that linoleic acid may well have been the active factor through which biochemical and clinical improvement was previously observed in patients with Friedreich's Ataxia supplemented with lecithin.

Administration, Oral↗
PubMed1980

Correlation between serum lipoamide dehydrogenase activity and phosphatidylcholine therapy in Friedreich's ataxia.

Serum lipoamide dehydrogenase activity and kinetics were studied in nine patients with Friedreich's ataxia before and three months after therapy with oral lecithin. Results disclosed a significant reduction in LAD inhibition by NADH in all patients after therapy. Three patients normalized their increased Km for lipoamide and one patient showed the opposite results after therapy. Two patients ceased lecithin after one month. All seven patients who remained in the trial group and one additional patient, showed subjective and objective signs of improvement in physical resistance. This study has offered some biochemical basis for the apparent clinical improvement in patients with Friedreich's ataxia who undergo lecithin therapy.

Adolescent↗
PubMed1980

The beta-amino acid transport system in Friedreich's ataxia.

Taurine and beta-alanine uptake in cultured skin fibroblasts proceeds through at least two distinct amino acid transport systems. The predominant beta amino acid uptake system which we refer to as the "Beta" system, incorporates taurine in a proportion of 95%. Beta-alanine in a proportion of 80% and does not incorporate beta-amino-isobutyric acid. A second transport system for beta-alanine seems to be operative cultured skin fibroblasts and this system shares the characteristics of system "L" for branched-chain and ring-side neutral amino acids. Results of ion depletion experiments, metabolic inhibition by drugs and blocking agents and previous kinetic studies of taurine and beta-alanine uptake in cultured skin fibroblasts failed to disclose any major difference in beta-amino acid transport between control individuals and patients with Friedreich's ataxia.

Alanine↗
PubMed1979

Decrease in the rate of protein synthesis by polysomes from cultured fibroblasts of patients and carriers with Duchenne muscular dystrophy.

Polysomes extracted from cultured fibroblast cells isolated from patients with Duchenne muscular dystrophy (DMD), carriers of the disease, and normal controls were used for in vitro measurement of protein synthesis in a wheat germ extract system. It was observed that polysomes from patients and carriers (seven of each aged 17 years or older) exhibited a 3-fold and a 1.5-fold decrease in the rate of protein synthesis, respectively, as compared with controls. These results are discussed with a view to developing a sensitive and easily available assay for the detection of DMD carriers.

Adolescent↗
PubMed1979

[Brain tumors in children in a French Canadian environment].

Between 1964 and 1976, we have seen 197 cases of cerebral tumors in children. We have studied the frequency of each histological group of tumors, their localization, the results of the clinical investigation, their clinical manifestations and the prognosis for each group. This study has confirmed that in children brain tumors are more frequently localized in the posterior fossa (56.4%). As in other series, tumors derived from the glia represent nearly 80% of the total. Clinically, increased intro-cranial pressure is the most frequent manifestation of posterior fossa lesion (75% of cases) while cerebellar involvement was seen in 60% of these children. Brain lesions and supratentorial tumors are more insidious in their presentation and their clinical manifestations more diversified. The EEG appeared useful in the investigation of brain tumors and showed irregularities in 78% of our patients. However, a more specific diagnosis requirelography. As in previous series, the mortality rate remained high in the children we have followed, particularly for some groups of tumors (glioblastoma, medulloblastoma, brain stem lesions). It is to be hoped that with the introduction of the CT Scan, the improvement of neurosurgical techniques and the use of immuno and chemotherapy, the prognosis may be improved.

Adolescent↗
PubMed1976

Shuddering attacks in children: an early clinical manifestation of essential tremor.

Six infants and children presenting with shuddering attacks had evidence and a family history of essential tremor. Although this association had not been recognized, the shuddering spells caused considerable concern and led to a wide range of diagnoses. The attacks start in infancy or early childhood, are brief, often associated with some posturing, and may be very frequent. They are benign and tend to become less frequent or to remit during the latter part of the first decade. The recognition of this syndrome should avoid unnecessary investigation and concern. The pathophysiology of shuddering attacks seems to represent an expression of the mechanism of essential tremor in the immature brain. The ultimate nature of these attacks will undoubtedly be clarified when a neurochemical basis for essential tremor is found.

Adult↗