[Letter to a provincial cousin, and his reply].
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Biomedical subjects
Publications and source records attributed to M Barat.
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Three new cases of myelopathy without compression associated with paraproteinemia are reported. The neurologic picture was that of spinal muscular atrophy in one case and chronic spinal combined sclerosis in two. The nature of the paraproteinemia is discussed: all three patients seem to have had benign monoclonal dysglobulinemia. One patient died from cardiovascular disease five years after onset, and another from complications related to decubitus seven years after onset. Postmortem examination was not possible in either case. Spinal muscular atrophy has been reported in certain carcinomas (lung, stomach, breast) and, less frequently, in macroglobulinemia. As benign dysglobulinemia is common after sixty, coincidental association cannot be outruled. However, data from the literature and the response to cancer chemotherapy in two patients suggest an original pathologic association. Recent demonstration of demyelinating neuropathies associated with benign paraproteinemia provide further evidence in support of such an association.
From a personal case and a review of the literature, it is recalled that bromocriptine may induce pleuropulmonary fibrosis. The various presentations of this condition are described. The index patient is a 56-year-old man, with Parkinson disease and a negative history for respiratory disease, who was taking bromocriptine in a high dose (60 mg/d). Under this treatment, he exhibited weight loss and an inflammatory syndrome and developed interstitial pneumopathy with secondary pleuropulmonary fibrosis, which resolved in part once therapy was discontinued. Bromocriptine, which is an ergot alcaloid with dopaminergic properties, has been used since 1965 in therapy. Its indications, which at the outset were restricted to endocrinology, were extended in 1972 to Parkinson disease, with a significant increase in dosages from 1979. Its responsibility in pleuropulmonary fibroses was suspected in 1981 by Rinne on data from 5 patients. As of now, 8 cases have been reported. All are Parkinson patients who, after a variable time interval (15 days to 3 years), developed a uniform picture of pleuropulmonary disease with rapidly increasing dyspnea upon exertion and deterioration of general health. These features mirror inflammation then fibrosis of the pleura and lung tissue, which results in a variable degree of chronic restrictive respiratory failure. The course is equally uniform, with partial resolution under corticosteroid therapy and more or less significant residual fibrosis at discontinuation of treatment. Immunoallergic rather than toxic or vasomotor mechanisms seem involved.(ABSTRACT TRUNCATED AT 250 WORDS)
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From the bulk of the Xenopus laevis mitochondrial proteins insoluble in 1% Triton X-100 + 1M NaCl, we have isolated, by DNA-cellulose chromatography, a protein fraction enriched in DNA-binding proteins. This fraction contains proteins showing a specific affinity for supercoiled DNA molecules containing the mitochondrial DNA displacement-loop region, as measured by filter binding and competition assays.
Nuclear inclusions in striated muscle from patients with oculopharyngeal dystrophy have been detected recently. We carried out ultrastructural examinations of biopsy specimens on 5 patients with oculopharyngeal dystrophy and we also reexamined a former case. In these 6 cases we found filamentous inclusions in a few nuclei. These inclusions seem to be characteristic of this disease as they have never been seen elsewhere.
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Language disorders developed in a patient following a subcortical infarct involving the left putamen and caudate nucleus. Language was abundant and fluent, with many perseverations, semantic and ideation incoherences, and all activities concerned with verbal or non-verbal expression were affected. Lesions were present in the head and body of the caudate nucleus, the whole of the putamen, the anterior nucleus of the thalamus and the superior part of the internal capsule. The disorder was unique in relation to disturbances of the aphasic type observed in extensive thalamic lesions, or in transcortical sensorial aphasia. Physiopathological interpretation is based on the functional role of the putamen-caudate system in the regulation of many types of behaviour: animal experiments and results of applying stimuli in humans suggests a release of the inhibition exerted by the caudate nucleus on the frontal cortex. Lack of comparable clinicopathological data implies that other similar cases must be reported before the specificity of the disorder can be confirmed.
Tomometric tests were carried out in 54 patients with a clinical picture of stabilized or regressive aphasia, some time after the initial episode. The correlation between clinical and topographic findings were satisfactory in 90 p. 100 of the cases. The frequency of lesions extending into several lobes (70 cases) and of multiple lesions is stressed. There is a significant relationship between the severity of the clinical findings during tomometric testing and the volume or multiplicity of the lesions. The progression of the disease after 6 months, as studied in 31 patients, was also related to the volume of the lesion, but was not influenced by the presence of diffuse atrophy, except where the disease was deteriorating. The value of tomometry in the study of aphasia involves 3 factors: precise clinical and lesional correlation, and the effect of the volume of the lesion on its initial severity and progression. In the future, tomometry should enable a correlative symptomatic study of aphasic disorders to be made.
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