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Biomedical subjects

P Bouche

Publications and source records attributed to P Bouche.

At least 127 records · Page 7Linked to original sources

[Subacute myopathy revealing hyperthyroidism].

Progressively increasing muscle weakness of the proximal regions of the lower limbs over a period of two months in a 57-year-old woman was found to be due to an undiagnosed hyperthyroidism. Total relief from muscle weakness was obtained when euthyroidism was restored. In spite of its rarity, hyperthyroidal myopathy should be suspected in all cases of acquired myopathic disorders in adults inasmuch treatment of the hyperthyroidism leads to recovery.

Female↗

[Effort-induced malignant hyperthermia. Electromyographic anomalies with a myogenic component. 10 cases].

Exertion malignant hyperthermia, usually regarded as a form of heat stroke, is mainly observed in young apparently healthy men enlisted in the army and subjected to intensive physical exertion in a warm and damp environment. It is frequently lethal. Ten cases with favourable outcome are reported. In 8 patients, EMG tracings recorded several months after the acute episode showed myogenic abnormalities. Computer-aided analysis of motor units showed a 42% reduction in mean duration of the motor unit collected from the brachial biceps and a 44% reduction of signal energy as compared with controls. Two possible reasons for these abnormalities are discussed: they may result from rhabdomyolysis or from a pre-existing muscular pathology.

Adult↗

[Study of the F wave in the rat].

The F wave is produced by the antidromic stimulation of the spinal motoneurons. It can easily be obtained on the distal muscles of rat hind limbs. In rat and man the F wave amplitude and occurrence are different. It is easy to obtain and the responses are constant. It is, therefore, a more reliable method for measuring motor nerve conduction velocity than conventional methods. This technique enables the study of experimental peripheral neuropathies. By varying the frequency and duration of stimulation it is possible to study the 'central excitatory state.'

Animals↗

[Polyradiculoneuritis associated with plasmocytoma. Recovery following radiotherapy (author's transl)].

A new case of extensive subacute polyradiculoneuritis associated with a costal plasmocytoma secreting IgG lambda is reported. Radiotherapy of the rib, resulted in remarkable neurological improvement and haematological cure, which have now persisted for more than 5 years, though treatment was discontinued 3 years ago. By its distinctive haematological features, this case illustrates the lack of clear-cut distinction between "solitary" plasmocytoma and multiple myeloma. It also emphasizes the need for systematic testing for plasma cell proliferation when confronted with a subacute or chronic neuropathy of obscure origin.

Adult↗

Perhexiline maleate and peripheral neuropathy.

Peripheral neuropathy has been noted as a complication of therapy with perhexiline maleate, a drug widely used in France (and in clinical trials in the United States) for the prophylactic treatment of angina pectoris. In 24 patients with this complication, the marked slowing of motor nerve conduction velocity and the electromyographic changes imply mainly a demyelinating disorder. Improvement was noted with cessation of therapy. In a few cases the presence of active denervation signified a poor prognosis, with only slight improvement. The underlying mechanism causing the neuropathy is not yet fully known, although some evidence indicates that it may be a lipid storage process.

Adult↗

Linkage analyses between dominant X-linked Charcot-Marie-Tooth disease, and 15 Xq11-Xq21 microsatellites in a new large family: three new markers are closely linked to the gene.

X-linked dominant inheritance was suspected in a large family with Charcot-Marie-Tooth disease since no male to male transmission was observed, and since the sensory and motor neuropathy was more severe in males than in females. To test linkage to the dominant X-linked Charcot-Marie-Tooth disease (DCMTX) locus in Xq13, genotypes of 19 affected and 19 unaffected individuals from this family were determined for 4 microsatellite markers. Close linkage to mfd66 (DXS453) was found by bipoint analysis (Zmax = 4.8 at theta = 0.00). Multipoint analysis mapped the gene between the androgen receptor and DXYS1. In addition, linkage analysis performed with 11 microsatellite markers, derived from a high density map spanning 16 cM on Xq11-Xq21 revealed 3 new tightly linked loci: afm287zg1 (DXS1216), afm261zh5 and afm207zg5 (DXS995). Multipoint analysis localized the DCMTX gene to a 7.5 cM interval between afm123xd4 (DXS988) and afm116xg1 (DXS986). Combined analysis with these new microsatellites provides a powerful tool for carrier detection because of their high informativity and the small genetic distance (< 10 cM) between the markers flanking the gene.

Blotting, Western↗