[Ultrastructural study of the injured striated muscle].
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Biomedical subjects
Publications and source records attributed to M Reznik.
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From 9000 autopsies, three giant fusiform aneurysms of the basilar artery were found in middle-aged men with arterial hypertension, atherosclerosis, symptoms of encephalic circulatory disturbances, transient then permanent cranial nerves palsy. Neuroradiological investigations suggested a basilar dolichomegartery in one case. Anatomically, these unruptured giant aneurysms (respectively: 2, 2.5 and 4.5 cm largest transversal diameter) compressed the brainstem; a recent softening destroyed the midpons in one case, and both cerebral peduncles in another. A review of the literature underlined the paucity of similar cases, and the difficulty of differential diagnosis of giant basilar aneurysms detected only by neuroradiological investigations without anatomical verifications.
Progressive multifocal leukoencephalopathy (PML) occurred in two patients after kidney transplantation. Two years after such a transplantation associated with immunosuppressive chemotherapy, a 54-year-old male developed polyneuropathy, diffuse alterations of the central nervous system and he died with the suspicion of hypertensive encephalopathy due to progressive renal failure. A 45-year-old female had kidney transplantation first complicated by Listeria monocytogenes meningoencephalitis. She was cured from this disease and had a satisfactory social rehabilitation for two years. Afterwards, she suffered from various neurological ailments, including epilepsy, that were attributed to combined renal failure and developing hydrocephalus. One year after the onset of these neurological symptoms, the grafted kidney was removed and chemotherapy was discontinued but she died a few weeks later. Both patients had typical PML. By electron microscopy, performed on formalin fixed brain tissue, intranuclear round particles (40-50 nm) could be recognized in the first case only. These two cases are confronted with the six published observations of PML following organ transplantation. The frequency of PML has been estimated at 1 for 5000 kidney transplantation, 1 for 2000 chronic lymphoid leukemia and 1 for 10,000 Hodgkin's disease.
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Presentation of a caucasian female (70-year-old) who suffered from urinary incontinence and orthostatic hypotension (Shy-Drager syndrome) and died six years after the onset of the dysautonomic clinical disorders. Neuropathological examination demonstrated putamino-nigral degeneration, olivo-ponto-cerebellar atrophy, neuronal depletion in the brain-stem and the spinal cord, mainly in the intermedio-lateral horns. The cerebral cortex did not exhibit senile alteration as often seen in ageing brain. A review of the literature, including 43 autopsied cases, confirmed that the Shy-Drager syndrome results from a diffuse neuronal loss mainly in the putamen, the pigmented nuclei, part of the brain-stem and the spinal cord (multiple system atrophy, with minimal cerebral cortex involvement). The disease occurs sporadically in adults, more frequently in man (sex ratio : 2.5/1). (Acta neurol. belg., 1980, 80, 271-286).
Among the last 7000 consecutive autopsies from our department, nine cases of primary reticulum cell sarcoma (non hodgkinian malignant lymphoma) of the central nervous system were discovered. Another case was found by cerebral biopsy. There were six males and four females (mean age 43.5 years). Mean duration of illness to time of death was 4.6 months. Gross examination revealed a wide range of neuropathological lesions (multifocal or diffusely infiltrating the parenchyma) and predominant in the cerebral hemispheres. Review of this materal underlines the difficulty of clinical diagnosis and the need to search for a more specific tratement.
A 31-year-old overweight man, suffering from high-blood pressure, was hospitalized for transient fits and hemiparesis. MRI disclosed a large irregular mass affecting the vault, meninges and invading the parietal lobe. At neurosurgery, the lesion was necrotic, hemorrhagic and poorly demarcated from the surrounding brain. Histopathology revealed a benign Langerhans cell histiocytosis. No other systemic nor organic lesions could be discovered. After additional local radiotherapy, the patient recovered completely and regained normal activities 6 months later.
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The authors have observed the evolution of a late catatonic syndrome in a 50 years old woman, without psychological background. They have observed a progressive mental (intellectual) deterioration which have had for a long time a partial and paradoxical aspect, while on the thymic and affective stade an atypical melancolic picture evolved toward a schizophrenic syndrome with catatonic traits which finally came to a stade of marasmus and death after three and a half years of illness. A psychological examination performed at mid course confirmed the mental deterioration without intellectual disorganisation (Wechler) while the Rorschach indicated schizophrenia. The neuro-radiological explorations, repeated several times, have demonstrated the existence of a diffuse cerebral atrophy on the white substance and yet more on the cortex, and it was possible to follow the aggravation of this atrophy. Repeated biological tests were less informative: albumine in the CSF was 0.15 to 0.63 g % and an isolated increase of alpha1-globulines in the CSF was observed at the electrophoresis 4 months before the exitus. Histological examination of cortical biopsies and of the white substance indicates a degenerative encephalopathy with spongiosis and cortical atrophy. Because of the limited value of the histopathological examination, one cannot suggest a systematic interpretation of the catatonic symptoms.
The authors report the clinical history of a young athlete who, without any notable family antecedents, developed cardiomyopathy followed by proximal anyotrophy at the age of 24 years. The electromyogram revealed frequent myotonic salves and a typically myogenic tracing. The autopsy showed substantial generalized cardiac enlargement and marked atrophy of the muscle fibres in the girdles, together with grouping of the nuclei into hyperchromatic clusters. The reported case thus shows some characteristics of Steinet's disease and others of non-dystrophic myotonia (Becker, 1964) but cannot be classified as either of these diseases.