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J Pouget

Publications and source records attributed to J Pouget.

At least 163 records · Page 9Linked to original sources
PubMed1986

[Aplasia of the thenar eminence: a partial and distal form of radial ray dysplasia. 6 cases].

Six cases of thenar muscle aplasia are reported. Aplasia of the abductor pollicis brevis and opponens pollicis muscles was noted in all cases. The flexor pollicis brevis muscle was inconstantly involved and the abductor pollicis muscle was normal. The aplasia was unilateral in five cases and bilateral in one. Bone dysplasia of the scaphoid, first metacarpal and thumb was associated with muscle aplasia. Arterial dysplasia was also observed in four cases. In all cases electrophysiological examination showed that the thenar branch of the median nerve was not involved. Electromyography showed no sign of denervation of the thenar muscles. Motor and sensory conduction velocities of the hand nerves were normal. Thenar aplasia is considered a partial and distal form of the radial ray defect caused by a focal embryonic necrosis before the differentiation stage.

Abnormalities, Multiple↗
PubMed1986

Mechanism and significance of insulin resistance in myotonic dystrophy.

In order to elucidate the mechanism of the glucose intolerance frequently associated with myotonic dystrophy (MD), glucose metabolism of 10 patients and 10 controls was investigated using the following tests: successive intravenous stimulation of insulin secretion by glucose and tolbutamide, detection of serum islet cell antibodies, measure of the specific insulin binding on erythrocytes and evaluation in vivo of insulin sensitivity by the euglycaemic glucose clamp method. Glucose tolerance was decreased in MD patients (K value: 1.51 +/- 0.15 vs 2.4 +/- 0.2 X 10(-2)) in spite of a basal (26 +/- 4 vs 11 +/- 2 microU/ml) and post-stimulative hyperinsulinism (area of the plasma insulin curve after glucose IG: 642 +/- 120 vs 315 +/- 28 microU/ml/min and area after tolbutamide IT: 740 +/- 166 vs 335 +/- 35 microU/ml/min). No islet cell antibody was detected in the serum of MD patients. These results suggest that decrease of glucose tolerance is secondary to peripheral insulin resistance. Specific binding of insulin on erythrocytes was slightly but not significantly reduced in MD patients (specific binding: 8.21 +/- 0.9 vs 9.64 +/- 0.9%, receptor number: 23 +/- 2 receptors/cell, concentration of unlabelled insulin displacing 50% of the bound radioactivity: 7.2 +/- 0.56 vs 6.6 +/- 0.6 ng/ml). There was a loss of normal down regulation of insulin receptors in MD. The results of the euglycaemic glucose clamp confirmed the insulin resistance especially for the highest insulin infusion rates. These data show that the insulin resistance of MD is due to both receptor- and post receptor-defect but that the main abnormality is an unresponsiveness located after the insulin signal on the receptor.

Adult↗
PubMed1986

[Peripheral neuropathy during treatment with cimetidine].

A case of peripheral neuropathy following cimetidine treatment is reported. Four days after beginning cimetidine (200 mg four times a day), the patient developed muscle pain and a symmetric motor neuropathy in all 4 limbs, predominant distally and in the lower limbs. Cimetidine was discontinued. Within seven days motor function began to return and within five months recovery was complete. Electrophysiological studies showed an axonal neuropathy. Morphometric studies revealed loss of large myelinated fibers in some fascicles while other fascicles were normal. Teasing studies showed predominant axonal lesions. Microvasculitis was present in the epineurium. Such findings suggest a role for small-vessel immune-complex vasculitis in the pathogenesis of this cimetidine-induced peripheral neuropathy.

Adult↗
PubMed1986

[135 cases of polymyositis].

The classification of 135 patients with either dermatomyositis (DM) or polymyositis (PM) showed 34 cases of DM and 56 cases of PM in adults, 6 cases of DM associated with cancer, 9 cases of DM in children, 16 cases of localized PM and 14 cases of an overlapping syndrome. Results of biological tests, erythrocyte sedimentation rate and serum enzyme determinations were inconstantly abnormal. The electromyograms were generally of a myogenic type with spontaneous activity in about half the cases. Muscle biopsy usually showed inflammation necrosis and regeneration, sometimes only of moderate severity only. Results were normal in several cases. In 21 patients only the pathognomonic perifascicular atrophy was reported. Proposed classifications are unsatisfactory. Polymyositis may be considered as a syndrome. Among the primary forms the distinction between acute dermatomyositis and subacute or chronic polymyositis is poorly defined and passage from one disorder to another is frequent. Pseudo-myasthenic forms are not entities and pseudo-myopathic types are actually muscular dystrophies. Associations with polymyositis are common and may consist only in the addition of one sign of no clinical significance (PM "plus"). The polymyositis lesion may be part of a syndrome such as Gougerot-Sjögren's or of another connective tissue disease. A system of diagnostic criteria uses numerical ratings of each criterion as a function of its semiologic importance.

Adrenal Cortex Hormones↗
PubMed1986

[Sensory trigeminal neuropathy in systemic diseases: 4 cases with study of the trigeminofacial reflex].

Isolated trigeminal nerve affections can occur during the course of various connective tissue diseases, particularly scleroderma and mixed connective tissue lesions. Four cases are reported: a patient with systemic scleroderma, one with atrophic polychondritis, one with Gougerot-Sjögren's disease and one with atypical and frustes connective tissue lesions. The mechanisms of onset and lesional location in these neuropathies are poorly understood. A blink reflex study by electrical stimulation of the supraorbital nerve was carried out in these 4 patients to determine the site of lesions. Response was normal in 1 case suggesting a lesion of a distinct branch of the supraorbital nerve. In 2 cases the anomalies of the early response were strongly suggestive of a peripheral, truncal or radicular lesion. In the last patient the early response was normal and latencies in tardive responses of the stimulated side were in favor of a central lesion of the spinal root or spinal nucleus of the trigeminal nerve. Clinical characteristics of some reported cases of neuropathy of trigeminal nerve also appear to point to a central lesion.

Adult↗
PubMed1986

[Clinical recovery from Isaacs' syndrome].

A patient with Isaacs' syndrome (previously reported in 1966) has been clinically normal since 1972. The possibility of an outcome of this type has been reported by Isaacs and Heffron (1974) and in subsequent reports. This does not provide an explanation for the syndrome of continuous muscle fiber activity but constitutes an additional clinical feature.

Adult↗
PubMed1986

[Emery-Dreifuss disease or syndrome of amyotrophy with early contractures and secondary disorders of cardiac conduction with variable heredity].

Emery-Dreifuss myopathy is characterized by the association of early muscle contractures, atrophy predominant on the scapulohumeroperoneal muscles, secondary cardiac conduction anomalies and an X-linked heredity. The case presented here had features corresponding to these criteria except in two respects: the patient was a female, and transmission was of the dominant autosomal type. Three similar families have been reported. This genetic heterogeneity together with doubt as to the exact nature of muscle anomalies, suggests that the syndrome should be termed amyotrophy syndrome with early contractures and secondary cardiac conduction abnormalities with a variable heredity.

Adult↗
PubMed1986

[AMP deaminase deficiency (myoadenylate deaminase). Disease or syndrome?].

Two recent publications have shown the advantage of understanding the deficit in AMP desaminase in rheumatology. On this subject, the authors report 4 cases of deficit in AMP desaminase. The first one includes a semiology made of pain, and stiffening, the second case is discovered in the course of a primary muscular disease. The third case is present during the first stage of a spinal cord compression. The fourth case is a muscular deficit accompanied with a histological picture of inflammation, considered initially as a chronic polymyositis, but explained secondarily as a pseudo-polymyositis form of facio-scapulo-humeral dystrophy. In this respect, the cases from the literature are divided into three groups: asymptomatic, infraclinical forms, forms occurring in the course of specific diseases (muscular diseases, spinal cord diseases, inflammations of the connective tissues, metabolic diseases), apparently isolated forms. In the latter, emerges a semiology made of pain, cramps, stiffening of the lower extremities occurring on exertion. However, the specificity of the symptoms remains to be discussed.

AMP Deaminase↗
PubMed1985

[Localized myositis with eosinophilia].

A 49-year old man developed myositis of the left masticatory muscles followed by myositis of the right supinator longus; these two lesions were histologically confirmed. Blood and bone marrow eosinophilia was present. Cure was obtained with systemic corticosteroids. Only one similar case was found in the literature. These cases differ from the various localized or focal forms of myositis as well as from eosinophilic polymyositis. Despite some similarities with masseteric myopathies in animals, no hypothesis can be formulated concerning the cause of the disease.

Eosinophilia↗
PubMed1985

Pulmonary function and electromyographic study of respiratory muscles in myotonic dystrophy.

Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory resistive loads. Present results show that (1) a moderate restriction of lung volumes with hypoxemia plus normocapnia is often observed; (2) patients sustain dynamic ventilatory efforts more easily than static work; and (3) abnormalities in respiratory muscle EMG exist with spontaneous expiratory and inspiratory intercostal activities during quiet breathing and changes in muscular response to resistive loads. Inspiratory loading evokes contraction of expiratory muscles, with a marked decrease in inspiratory activities. Expiratory resistive loads prolong the diaphragmatic contraction throughout the expiratory time, and in some patients, relaxation of the diaphragm does not occur during the loaded run. These EMG data suggest that the reciprocal inhibition among respiratory neurons is enhanced in myotonic dystrophy and that myotonia also occurs in the diaphragm when loads oppose its relaxation.

Adult↗
PubMed1985

Chronic neurogenic quadriceps amyotrophies.

Two cases of quadriceps amyotrophy, probably of chronic neurogenic origin are reported. Only the knee jerks were diminished, the calves hypertrophic, and the serum creatine kinase level very high in one case, and there were neurogenic electromyographic abnormalities in the quadriceps. In the first case, biopsy of the quadriceps muscle revealed a neurogenic origin with hyalinized hypertrophic fibres. CT scan showed abnormalities not only in the quadriceps but also in the sartorius, gracilis and gastrocnemius muscles. A second biopsy specimen from the gastrocnemius muscle showed histological findings similar to those of the quadriceps. In the second case, the EMG and biopsy findings suggested a myogenic origin, but 6 years later they were compatible with neurogenic atrophy. Differentiation from Becker dystrophy is very difficult in the first case and the second case is more a focal spinal amyotrophy. Further, in spite of their localization, the extension of the affected muscles changes the diagnosis. The same applies to chronic quadriceps amyotrophy in general, which cannot be regarded as an entity, but which suggests muscular dystrophy, spinal atrophy, polymyositis or a metabolic disorder. These cases can be compared with the four cases reported in the literature, which were regarded as a "forme fruste" of chronic spinal amyotrophy.

Adult↗
PubMed1985

[Results of muscular x-ray computed tomography in 145 cases of neuromuscular disease].

CT Scan examination in 145 cases of neuromuscular diseases yielded the following results: Diagnosis between myogenic and neurogenic process is inconstant and cannot be considered as absolute. In myogenic diseases the X ray density of muscle is early decreased with a preservation of muscle outline. In neurogenic diseases muscle volume is early decreased. Coexistence of atrophic and hypertrophic muscles indicates primarily muscle disease. Some patterns of involvement appear to be frequent. In Duchenne's dystrophy a contrast exists between atrophic "empty" or hypertrophic muscles during the ambulatory period and "ghostly" muscles during the terminal period. In facio-scapulo-humeral muscular dystrophy, tibialis anterior and hamstring muscles have often a decreased density and psoas muscles are normal or hypertrophic. In myotonic dystrophy a hypodense perifemoral crescent is frequently observed. Diagnosis between limb-girdle myopathy ("empty" muscles with preserved limits, hypertrophic muscles, hypodense gastrocnemius medialis muscles) and chronic spinal amyotrophy (irregular and atrophic muscles without selective involvement and hypertrophic muscles) is tentatively proposed but is not considered to be clear-cut. Muscle involvement has an asymmetric distribution in amyotrophic lateral sclerosis and is rather symmetric in peripheral neuropathies.

Adolescent↗