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Biomedical subjects

G Serratrice

Publications and source records attributed to G Serratrice.

At least 37 records · Page 2Linked to original sources
PubMed1997

Gait initiation and impairments of ground reaction forces as illustrated in old age by 'La marche à petits pas'.

Ground reaction forces during gait initiation and kinematics of the first step were recorded in 11 elderly patients with idiopathic 'marche à petits pas' and 18 age-matched normal adults. Smaller values of vertical forces, and impaired amplitudes and directions of anteroposterior forces might explain start difficulties of the patients. Higher vertical displacement of the foot, and laborious establishment of the rhythm in the patients could also be related to the perturbations observed in the reaction forces. These perturbations are likely to reflect impairments of muscular synergies of the lower limbs and the lack of limb co-ordination.

Aged↗
PubMed1997

Long term follow up of multifocal motor neuropathy with conduction block under treatment.

Eighteen patients (15 men, three women; age range 30 to 71 years, mean 45.8 years) with multifocal motor neuropathy treated with high dose intravenous immunoglobulin (IVIg) were evaluated for nine to 48 months (mean follow up 25.3 months). The median time between onset of multifocal motor neuropathy and treatment was 5.8 years. The dose of IVIg was 0.4 g/day for three to five days. The interval between each treatment was determined for each patient by the evaluation of the effect of the first course. Muscle strength was evaluated by a computerised analyser. Clinical improvement was seen in 12 patients treated with IVIg (67%). Isometric strength increased from 32% to 97% (mean 54.5%) of the initial value. Functional scales corroborated these findings. No clear predictive factors of response to IVIg was found except the presence of high titres of IgM anti-GM1 antibodies. Often, patients needed repeated courses of IVIg to maintain the improvement. In two patients, IVIg infusions were stopped without signs of relapse after one year. Four patients were initially treated with prednisone (1 mg/kg/day), without any clear improvement. Five patients with no response to IVIg or who were IVIg dependent were treated with cyclophosphamide, but only one showed improvement. These results show the long term benefits and safety of IVIg in multifocal motor neuropathy but also the transient effect of this expensive treatment in most patients.

Adult↗
PubMed1997

[Late onset myasthenia: 34 cases in patients over 65 years of age].

This study, a retrospective view of 34 patients with myasthenia gravis, compared the course of the disease for patients with onset before 65 and after 65 years. 70% of those under 65 were female while 55.8% of those over 65 were male. Bulbar symptoms were more frequent in older patients. Only 3 patients had another immune disease (rheumatoid arthritis, diabetes mellitus, thyroid pathology), and two a thymoma. All patients were treated with anticholinesterases. Prednisone was used in 44% of cases and rarely Azathioprine. In our cases and in the review of the literature there is no significant difference between age groups except for the sex ratio and the outcome in the older group in case of thymoma or respiratory failure.

Aged↗
PubMed1997

[Variable and recurrent ptosis and ophthalmoplegia in chronic polyradiculoneuritis].

Fluctuating ptosis and diplopia are typical features of myasthenia gravis. This report describes four patients who developed these symptoms and in whom the investigations have demonstrated typical features of chronic inflammatory polyradiculoneuropathy. Ptosis and ophtalmoplegia were in most the cases unilateral and were sometimes painful. The evolution was fluctuant with episodic exacerbations. These unusual clinical features have responded like other features of CIDP to corticosteroids or to intravenous immune globulin but relapses are always possible. We did not find in the serum of three patients anti-GQ1b IgG antibodies as previously found in Miller-Fisher syndrome or in Guillain-Barré syndrome associated with ophtalmoplegia. Considering these results, on can argue that other immune mechanisms are involved.

Adrenal Cortex Hormones↗
PubMed1997

[Neurological aspects of malignant hyperthermia. Clinics, physiopathology, genetics].

Malignant hyperthermia, a disease formerly considered to occur almost exclusively in an anesthesia setting, has been observed in wider contexts due to progress in screening tests and genetics. Different neurological and neuromuscular conditions, with and even without hyperthermia, have been considered to belong to the disease, including overt or infraclinical signs of myopathies, and most significantly central core disease. Exercise-induced malignant hyperthermia is another similar syndrome as are syndromes with isolated exercise intolerance in young subjects, certain rhabdomyolysis syndromes and certain cases of sudden infants death syndrome. The pathophysiological mechanism would involve excessive calcium release from the sarcoplasmic reticulum caused by a protein structure anomaly in the ryanodin receptor, a non-energy dependent channel. Genetic studies have localized the ryanodin receptor gene anomaly on chromosome 19 in man in 30 to 40 p. 100 of the families. Other mutations with other chromosomic localizations are however known. Management of suspected malignant hyperthermia includes muscle biopsy and well-defined contraction tests.

Disease Susceptibility↗
PubMed1996

[Inclusion body myositis].

Inclusion body myositis has been recently recognized as a clinical entity although its exact definition remains uncertain. Initially considered to be an inflammatory dermatomyositis, inclusion body myositis can actually take on three specific forms: disseminated muscle atrophy and weakness, pseudopolymyositis, or pseudo-degenerative disease. Inclusion body myositis is different from non-inflammatory neuromuscular diseases with vacuoles. Abnormal deposits are seen within the muscle fiber may contain amyloid substance, beta-amyloid precursor, ubiquitin, antichymotrypsin, protein tau, apolipoprotein E and even prions. The signification of these deposits is unknown. Deletions in mitochondrial DNA have been demonstrated but do not appear to play a causal role. More and more hereditary forms are being recognized and certain may be related to an abnormality in chromosome 9.

Amyloid beta-Peptides↗
PubMed1996

[Acute myopathy in an asthmatic patient treated with corticoids and muscle relaxants in the intensive care unit].

Acute myopathy occurred in a 49-year-old woman hospitalized in the intensive care unit for status asthmaticus. She was given high-dose intravenous steroid therapy and intubated. Pancuronium bromide was used for prolonged curarization. Flaccid quadriplegia developed with preservation of the deep tendon reflexes. Muscle biopsy showed a myogenic process with disorganized myofibrils and selective loss of thick myosin filaments. This mainly myogenic process would result from the toxic effect of corticosteroids favored by prolonged curarization although the effect of other factors still remains unknown.

Acute Disease↗
PubMed1996

Plasma concentrations and pharmacokinetics of idebenone and its metabolites following single and repeated doses in young patients with mitochondrial encephalomyopathy.

OBJECTIVE: The pharmacokinetics and tolerance of idebenone after single or repeated doses have been studied in young patients with mitochondrial encephalomyopathy. RESULTS: No significant adverse effects were noted. In 3 out of 7 patients idebenone induced overall stimulation and improvement in arousal. Plasma concentrations of idebenone and its main metabolites were determined and the pharmacokinetic parameters of idebenone after single and repeated doses were estimated. During the single dose study, the mean plasma concentrations of idebenone and its main metabolites and mean pharmacokinetic parameters were comparable to published results (Cmax = 452.2 ng.ml-1, tmax = 2.3 h, AUC = 26 micrograms. ml-1.h, t1/2 beta = 16.5 h). During the repeated doses study, no significant difference was found between mean residual plasma concentrations of idebenone on Day 2 (47 ng.ml-1) and Day 5 (70.6 ng.ml-1), and mean t1/2 beta of idebenone after the single and after repeated dose studies, i.e., there was no evidence of accumulation. Although idebenone did not appear to accumulate during this study, the coadministration of anticonvulsants, often prescribed during mitochondrial encephalomyopathy, can affect its pharmacokinetics.

Adolescent↗
PubMed1996

A controlled one-year trial of dextromethorphan in amyotrophic lateral sclerosis.

In a one-year parallel group double-blind placebo-controlled study of dextromethorphan (1.5 mg/kg) in amyotrophic lateral sclerosis, no significant differences were observed in the rate of progression (Norris scale) in comparing 24 patients randomly assigned to the dextromethorphan group and 25 patients randomly assigned to the placebo group. Of the 24 patients in the dextromethorphan group, 17 had limb onset and 7 had bulbar onset disease; average duration of disease was 12.5 +/- 6 months and sex ratio (M:F) was 1.4:1. Of the 25 patients in the placebo group, 18 had limb onset and 7 had bulbar onset disease; average duration of disease was 9.9 +/- 6 months and sex ratio (M:F) was 1.55:1. Dextromethorphan is a weak noncompetitive N-methyl-D-aspartate (NMDA) antagonist and higher doses or other potent NMDA receptor antagonists should be tested.

Adult↗
PubMed1996

Bent spine syndrome.

Eight elderly patients developed progressive paravertebral muscle weakness with bent spine on standing but normal supine posture. Computed tomography showed pronounced hypodensity of the paraspinal muscles. Serum creatine kinase was moderately increased, paraspinal EMG non-specific, and biopsy myopathic. The syndrome was familial in two patients and may be caused by a late onset paraspinal muscle dystrophy. In the absence of specific pathological changes, such cases could be classified as having "bent spine syndrome".

Age Factors↗
PubMed1996

[Hiramaya disease: disease or syndrome?].

Hirayama's disease, a benign juvenile amyotrophy of "oblique" topography of an upper limb, has been acknowledged in the international literature. 4 cases are reported in this paper. However there present some differences: presence of antigangliosides antibodies in 1 case, cryoglobulinemia in another case developing bilaterally, conduction blocks in a third case. Finally, in the patient illustrating the typical form, the neuroradiological abnormalities considered as characteristic of cervical spine compression by Hirayama are lacking.

Adolescent↗
PubMed1996

[Automatic motion analysis of gait in patients with Parkinson disease: effects of levodopa and visual stimulations].

Gait analysis of 13 patients affected by Parkinson's disease (PD) and 7 healthy elderly volunteers was performed with a fully automatic motion analyser. The recording included stride parameters for walking velocity, stride length, stride duration and swing time. Maximal amplitudes of rotation of the hip, knee and ankle were also recorded. The analysis was performed for the PD's patients before and after L-Dopa intake. All the patients walked with and without transversal stripes on the floor. The contrasting white lines were 45 cm apart. After medication, the stride length, the velocity and the swing phase duration were significantly increased. The movements around the hip, knee and ankle joints that were initially reduced, poorly increased after L-Dopa intake. When stripes were placed on the floor, no significant changes occurred for the overall group of patients. Seven parkinsonian patients did improve with visual guidance, increasing their stride length and their speed. Some rotations of hip and knee were also influenced by stripes. This sub-group of patients was characterized by older age, a slower walking velocity, a smaller stride length and a shorter swing time. We correlated the sensitivity to visual cues to the defect of the visual contrast sensitivity that has been established in PD by several recent works. Locomotion on the transverse lines might have produced a motion perception at a rate determined by the speed of the patient. For appropriate intervals between stripes, one can hypothesise that the resulting visual stimuli belonged to a range of spatio-temporal frequencies that are decreased in patients with PD.

Aged↗
PubMed1996

[Guillain-Barré syndrome: development of concepts].

The features initially described by Guillain et Barré have been updated through the proposal of precise criteria even if a number of questions remain. Hundred of works have contributed to the evolution of these concepts. This paper is a summary of several opinions: 1. From nerve inflammation (lesions have been progressively analysed) to dysimmunity (with a combination of humoral and cellular dysimmunity), 2. The most frequent initial factors seems Campylobacter Jejuni with particular clinical correlations, 3. The acute axonal form assessed by some, contested by others, 4. Several varieties among which the Miller Fisher syndrome associated with anti-GQ1b antibodies almost constantly, 5. The treatment presently plasma exchanges and immunoglobulines infusion. In spite of the great number of papers devoted to Guillain-Barré syndrome a number of questions remain, and none among the varieties recently described, is unanimously acknowledged. However the plasma exchanges and mainly the intravenous immunoglobulines have a positive effect in the severe forms.

Autoimmune Diseases↗
PubMed1996

[Treatment of multifocal motor neuropathies].

Multifocal motor neuropathy is characterized by a progressive asymetrical weakness, predominantly affecting the upper limbs with persistent conduction blocks on motor but not sensory nerves. Treatment woth prednisone and plasma exchanges have failed to demonstrate any positive effects. Some improvements have been reported with cyclophosphamide. Mainly immunoglobulin therapy has been evaluated with a beneficial response in almost 70% of the cases. These benefits obtained over periods of less than six months have recently been confirmed by a long-term evaluation of 18 patients treated by repeated infusions.

Adrenal Cortex Hormones↗
PubMed1996

[Proximal spinal muscular atrophy in the adult].

Based on personal experience and recent advances in the molecular genetics of spinal muscular atrophies (SMA), the author reviews the main features of adult SMA predominantly affecting the proximal musculature. Hereditary SMA forms linked to characteristic chromosome abnormalities are distinguished from those that have not been linked to any known gene. Among the first type, recessive autosomal inherited forms linked to chromosome 5 are mentioned and the description is extended to progressive bulbar-spinal amyotrophy and Kennedy syndrome, linked to the X chromosome, whose gene also codifies androgenic receptors, constituting a syndrome with clear neuro-endocrine involvement. In dealing with SMA forms that have not been linked to a gene, the author details, always from personal experience, those with scapuloperoneal involvement, of which Stark-Kaeser syndrome is the prototype. Finally, the author discusses non hereditary types, covering current knowledge of chronic anterior poliomyelitis, the concept of monomelic spinal muscular atrophy and post-poliomyelitis muscle syndrome. The author's extensive personal bibliography completes the chapter.

Adult↗