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Biomedical subjects

J Pouget

Publications and source records attributed to J Pouget.

At least 127 records · Page 7Linked to original sources
PubMed1992

[Rademaker and Garcin syndrome associated with pallidal calcifications].

We report gait abnormalities with exaggerated support against gravity in a 76 year-old woman. This exaggeration, and its demonstration by ankle dorsiflexion in supine position, was first described by Rademaker and Garcin (1933) as probably resulting from cerebellar and brainstem lesions. In the present case, quantitative analysis of locomotion showed a strong reduction in both stride duration and stride length. There was an increased variability between two consecutive locomotor cycles and a large increase in both stance duration and relative double-support duration. Electromyography clearly showed leg hypertension following ankle dorsiflexion, contrasting with normal leg mobility in the supine patient. This suggested that reflexive antigravity support abnormalities could explain this pattern of locomotor disabilities, differentiating them from other kinds of "marche à petits pas". The present case was associated with bilateral pallidal calcifications, suggesting an involvement of the basal ganglia in support reaction against gravity and in control of adequate postural muscle tone necessary for locomotion.

Aged↗
PubMed1992

Nociceptive threshold and physical activity.

Previous studies using subjective tools to measure pain have shown that muscle exercise can have analgesic effects in man. The nociceptive leg flexion reflex (or RIII reflex) is a useful objective tool for assessing human pain. In this study, the pain threshold was assessed using the nociceptive flexion reflex in six high-level athletes 1) at rest in comparison with 8 control subjects and 2) after exercise requiring the production of a 200-Watt force over a period of 20 minutes. The nociceptive flexion reflex threshold at rest was found to be spontaneously higher in the athletes than in the controls. Physical activity resulted in a significant increase (+53%) in the threshold of the nociceptive reflex in the athletes. The role of stress-induced analgesia, the reduction in perceived intensity of stimuli during movement, and the release of opioids are discussed.

Adult↗
PubMed1992

[Typical and atypical forms of neuralgic amyotrophy of the shoulder: 86 cases].

Eighty-six cases of neuralgic shoulder amyotrophy are reported. Among these, 67 cases were concordant with the usual semeiological description. The other cases were atypical in their anatomical distribution (extensive or restricted to peripheral nerve rami), in their course (chronic or recurrent or alternating from one side to the other) and in their cause, notably familial forms. The continuum existing between these variants is most probably due to an immuno-allergic mechanism.

Adolescent↗
PubMed1992

[Non hypertrophic amyloid myopathy with muscular inflammation in plasma cell dyscrasia].

The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent. Muscle biopsy showed endo- and perimysial amyloid deposits but also inflammatory infiltrates. Inflammatory cells typing was studied by immunocytochemical methods and revealed a predominant T-helper cell infiltration. Free kappa light chains were present in serum and urine. Serum immunoglobulin levels were reduced. Bone marrow examination revealed mild plasmocytosis without abnormal cells. Immunofluorescence and immunoperoxidase techniques for identification of the type of amyloid fibrils showed positivity with antisera to kappa light chains. A 4-year follow-up revealed a progressive worsening of muscle weakness despite immunosuppressive treatment. No malignant plasmocytosis occurred. The unusual inflammatory muscle infiltration observed in this case may suggest an associated polymyositis.

Adult↗
PubMed1991

[Anomaly in the neurotransmitter amino acids in amyotrophic lateral sclerosis: a therapeutic application].

Although the cause of amyotrophic lateral sclerosis remains unknown, the excitatory amino acids may be involved in its pathogenesis. Glutamate level analysis shows a differential distribution of the amino acids. One of the therapeutic methods consists of reinforcing the inhibitory amino acid activity using L-threonine which has been shown to improve some symptoms and signs of ALS.

Adult↗
PubMed1991

[Mitochondrial and ocular myopathies (62 cases)].

The authors report the clinical signs and histological findings in 62 patients with ocular myopathies divided into two groups: (a) ocular myopathies with mitochondrial abnormalities, themselves divided into pure (19 cases) or progressive (15 cases) ophthalmoplegia, and multisystemic myopathies (14 cases); (2) oculopharyngeal myopathies (15 cases) in elderly subjects, with constant intranuclear tubulofilamentous inclusions. Among myopathies with mitochondrial abnormalities, ocular forms with a favourable prognosis coexisted with earlier and more severe multisystemic lesions.

Adolescent↗
PubMed1991

[Slowly progressive myopathy with accumulation of tubular aggregates].

Slowly progressive myopathy with tubular aggregates is rare and dominantly or recessively inherited. Three sporadic cases are reported in the present study occurring in 2 men and 1 young woman. All patients had proximal limb weakness without severe atrophy. They also complained of exercise-induced stiffening and cramps of their leg muscles. In 1 case severe cardiomyopathy caused unfavorable clinical course and death. Serum creatine kinase activity was normal and electromyogram showed only slight myopathic changes. Tubular aggregates were found to be the sale morphological abnormality. They were present in type II fibres in 1 case and in type I and type II fibres in the others. An immunocytological study with a polyclonal antibody against Ca2+ SR-ATPase showed positivity of the tubular aggregates with this antibody. A quantitative analysis (SAMBA 2000 alcatel TITN) was carried out on frozen sections stained for calcium. It showed a lower calcium content in tubular aggregates than in other part of the fibre. Slowly progressive myopathy with tubular aggregates may be distinguished from other diseases where tubular aggregates are the sale structural change, such as myopathies with myasthenic features and some neuromuscular diseases with exercise intolerance without progressive course. Usually, tubular aggregates are not a specific finding; they have been described in various disorders in association with other structural changes.

Adolescent↗
PubMed1990

Pain relief achieved by transcutaneous electrical nerve stimulation and/or vibratory stimulation in a case of painful legs and moving toes.

A patient is described with painful legs and moving toes. The pain had been occurring for more than 15 years, and a variety of therapies had been attempted with only partial, if any, success. Only morphine had succeeded in relieving the pain, but it had to be discontinued to avoid tolerance and dependence. We devised a treatment consisting of transcutaneous electrical nerve stimulation (TENS), vibratory stimulation (VS), and a combination of the two methods (TENS + VS). TENS brought about partial pain relief, but was less effective than VS; dual stimulation (TENS + VS) led to complete alleviation of the pain. Four months later, the patient was applying dual stimulation himself at home and was thus able to maintain complete relief with 3 or 4 weekly sessions. We suggest that dual stimulation results in a large-scale recruitment of large-diameter afferent fibres and may thus set up a powerful inhibitory control of nociception in our patient.

Adult↗
PubMed1990

[Mutilating ulcerative acropathy in diastematomyelia].

We report a case of diastematomyelia revealed by familial ulceration of the extremities (a unilateral, painless ulceration associated with lumbar hypertrichosis). In such cases conventional radiography may show a lumbar bony spur and widening of the interpediculate space. The diagnosis is made by myelography; CT or MRI which show duplication of the spinal cord and sometimes of the dura mater. The pathogenesis of this type of dysraphism is discussed. In our patient, surgery resulted in healing of a perforating ulcer of the foot.

Adult↗
PubMed1989

Myopathy associated with desmin type intermediate filaments. An immunoelectron microscopic study.

The muscle biopsy performed in a 58-year-old woman with a myopathy involving pelvic girdle and lower limbs displayed unusual intermediate filament aggregates by light and electron microscopy. No cardiac involvement was detected. The filamentous aggregates selective for type 1 fibers were found in subsarcolemmal and intermyofibrillar areas closely related to Z bands. Immunohistochemical study by light and electron microscopy using polyclonal and monoclonal antibodies and avidin-biotin peroxidase method revealed that aggregates consisted of desmin filaments. Desmin positive material was unstained with vimentine antibodies.

Cytoskeleton↗
PubMed1989

[Clinical forms of inclusion body myositis: 12 cases].

Inclusion body myositis is now a well-known disease but its incidence is underestimated. We report 12 cases with clinical heterogeneity. Three groups of patients could be described. The first one corresponded to asymmetrical muscle involvement and distribution with a slow clinical course (4 cases). The second was characterized by a polymyositis-like syndrome (3 cas), but steroid therapy was ineffective. The last group mimicked a chronic spinal muscular atrophy (4 cases). One patient showed a scapuloperoneal syndrome. Both myopathic and neurogenic EMG patterns were present in 6 patients; a neurogenic pattern was found in 4 cases and a myopathic pattern in 2 cases. In all patients, muscle biopsies showed rimmed vacuoles with eosinophilic inclusions. In 9 cases ultrastructural studies displayed abnormal filaments of 15-18 nm in diameter in the vacuoles. Intranuclear filaments were rarely observed. The significance of the filaments is unknown and their specificity is doubtful because they are present in other myopathies with rimmed vacuoles (some distal myopathies and oculopharyngeal muscular dystrophies). Finally a rich inflammatory exudate was present in 8 patients only.

Adult↗
PubMed1989

[Kearns-Sayre syndrome: mitochondrial encephalomyopathy caused by deficiency of the respiratory chain].

We report the cases of a 46 year old woman and of a 18 year-old boy who met the criteria for Kearns-Sayre syndrome. Additional atypic features were present in one case: family history, psychosis and acute respiratory failure. In both cases histoenzymatic analysis of the muscle biopsy and biochemical studies of mitochondria isolated from the muscle sample demonstrated mitochondrial myopathy associated with combined partial deficiency of complexes I and IV of the electron transfer chain. Although there is no correlation between clinical and biological data in the mitochondrial myopathies our cases confirm that such defects are involved in Kearns-Sayre syndrome. Improvement with coenzyme Q10 therapy in these patients is reported.

Adolescent↗