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Biomedical subjects

R Escourolle

Publications and source records attributed to R Escourolle.

At least 55 records · Page 3Linked to original sources

[Asymptomatic multiple sclerosis - 3 cases (author's transl)].

Multiple Sclerosis (MS) cases found at autopsy in patients who had died from other diseases and in whom no sign or symptom could be related to MS are called "asymptomatic". Three cases are reported. The first patient was a 62 year old man who presented with a slowly progressive disturbance of gait, incontinence and deterioration of intellectual function. A falx meningioma was surgically removed. The patient died 3 years later with an acute respiratory illness. Examination of the brain disclosed evidence of the operation and numerous old plaques disseminated through the cerebral hemispheres (centrum semi-ovale, periventricular regions, internal thalamus and junction between cortex and white matter) and in the brain stem. The second case, a 77 year old woman with diabetes mellitus and hypertension, presented with cortical blindness and disturbances of memory of acute onset. She died one year later. Examination of the brain showed multiple infarcts involving the territories of both posterior cerebral arteries and the left middle cerebral artery. Numerous old plaques were seen in the periventricular regions, in the corpus callosum and in the left middle cerebellar peduncle. The third case, a 60 year old woman with mitral and aortic stenosis, presented with cortical deafness and transient right hemiparesis. She died 5 years later. Brain examination showed infarcts involving both middle cerebral artery territories. There was also many old plaques in the periventricular areas, thalamus, internal capsule, centrum semi-ovale, brain stem and right nucleus dentatus. In the 3 cases, the optic tracts were normal. The spinal cord, examined only in the first case, was also normal. The asymptomatic character of these MS cases can be explained first by the location of the plaques and the lack of spinal cord and optic tract involvement. It could also be due to the small size of the plaques and to axonal preservation. Such features are rare since our 3 observations have been selected from a pathological collection of 125 MS cases and 9,300 general neuropathological records. Six other cases have been previously reported by other authors.

Aged↗

Perhexiline maleate-induced lipidosis in cultured human fibroblasts: cell kinetics, ultrastructural and biochemical studies.

Perhexiline maleate reduced the growth of human skin fibroblasts in cell culture at a concentration range of 0.3-3 micrograms/ml. At the highest concentration, the cells survived only four days. Pleomorphic inclusions characteristic of drug-induced phospholipidoses appeared in cultured cells. Analysis of the major lipid classes was performed on cells exposed to 3 micrograms/ml at four days. Gangliosides, phospholipids and cholesterol levels four to six times above controls were found. No major qualitative abnormalities were detected in phospholipids. On the contrary, an abnormal pattern of gangliosides was seen by densitometry of silica gel thin-layer plates with increases of GD3 and of an unknown ganglioside. Drug induced lipidosis may involve other lipids than phospholipids, particularly gangliosides.

Cell Division↗

[Necrotic myelopathies and neoplastic pathologie. Three clinico-pathological cases (author's transl)].

Three clinico-pathological cases of necrotic myelopathies with a distant malignancy are presented. Two cases had a lymphosarcoma and one case a prostatic carcinoma. They were compared to 13 well studied other cases collected in the literature. These myelopathies were related to solid visceral tumours in 8 cases and to lymphomas in 5 cases. The disease could be individualized on clinical grounds (flaccid paraplegia with bladder and bowell incontinence and sensory loss without clear-cut upper boundary developing over a few weeks with normal CSF and fast impairement of general condition), and, on pathological features. It is characterized by one or several spinal cord necrosis areas, often asymetrical, involving mostly white matter, without any vascular topography. Axons are involved as well as myelin sheats. There is mild inflammation and no specific vascular alteration. There is no metastases in the cord, meninges, vertebral column or nerve root. No vascular occlusion is found. The mechanism of the disease is unknown. The frequent occurence of lymphomas could suggest the presence of immunopathological factors.

Adenocarcinoma↗

[Acute spongiform leucoencephalopathy with selective intramyelinic involvement of U fibers associated with an ovarian carcinoma. Syndrome of disconnection of U fibers (author's transl)].

Case report of clinical, pathological and ultrastructural features in an acute spongiform leucoencephalopathy with selective involvement of U fibers. A 52 years old woman exhibited an acute encephalopathy of 2 months duration, with dementia and multifocal impairment of cortical functions. The cerebral cortex was normal. This acute dementia resulted from a diffuse intercortical disconnection. Spongy degeneration was only found in U fibers. No other changes were noted especially in basal ganglia, optics tracts, and spinal cord. The white matter status spongious was related to an intramyelinic oedema. Such intramyelinic oedema is known only in Van Bogaert and Bertrand and Canavan disease, which is quite different, and in toxic encephalopathies, especially those induced by the hexachlorophene and triethyltin. In the present case no drugs or toxins were found. An ovarian carcinoma was found at post-mortem examination.

Acute Disease↗

[Neuropathological study of adult intracranial hemorrhage. III. Analysis of 107 meningeal hemorrhages due to rupture of arterial aneurysms].

The findings in 107 pathological studies of meningeal hemorrhages due to rupture of arterial saccular aneurysms are reported. There were 62 women (58 p. 100) and 45 men (42 p. 100). Mean age was 56, lower in men (53) than in women (58). 45 aneurysms of the Anterior Communicating Artery, 26 of the Middle Cerebral Artery, 15 of the Internal Carotid Artery, 10 of the Anterior Cerebral Artery, 1 of the Anterior Choroidal Artery and 10 of the Posterior System were studied. These ruptured aneurysms were compared to 31 non ruptured ones. Mean size of the ruptured aneurysms was 10, 62 mm, not statistically different from that of non ruptured aneurysms (9, 05 mm). Multiple aneurysms represent 10 p. 100 of the whole ruptured aneurysms population. They affected mostly the Middle Cerebral Artery. High blood pressure had been present in 56/107 cases of ruptured aneurysms (52 p. 100). The prevalence of high blood pressure in patients with ruptured aneurysms (men: 60 p. 100 between 35 and 49, 63 p. 100 between 50 and 64; women: 31 p. 100 between 35 and 49, 54 p. 100 between 50 and 64) was statistically higher than in the same age and sex ranges of the general French population. The other causes of bleeding were rare: 4 liver cirrhosis (2 of the patients were also hypertensive) and 5 anticoagulant therapies (2 of which were also associated with high blood pressure). Intracerebral hematomas were found in 43 cases (40 p. 100): 39 lobar, more frequent in the frontal (26) than in the temporal (13) lobes, due mainly to Anterior Communicating Artery and Anterior Cerebral Artery aneurysms; very few were in the basal ganglia (3) or brainstem (I). Intraventricular hemorrhage was found in 39 cases (36 p. 100). Forty (37 p. 100) cerebral infarcts had occurred. They were located in the territory of the same artery in 20 cases (19 p. 100), in a different territory in 11 cases (10 p. 100), in both in 9 cases (8. p. 100).

Adult↗

[318 cases of intracerebral hemorrhage. A pathologic study (author's transl)].

The systemic analysis of cerebral hemorrhages (large single and multiple hematomas and small slit-hemorrhages) has been practiced in 318 patients. The emphasis is put on the site of the bleeding and the etiological data. Single hematomas were situated: 1) in 127 cases in the basal ganglia region (26 were medial, 44 intermediate, 48 lateral, 8 quadrilateral and one subthalamic), 2) in 67 cases in cerebral lobes (24: front; 22: temporal; 21 parietal) and 3) in 33 cases in the posterior fossa (19: cerebellum; 14: brain stem). Large hematomas in 71 cases and slit-hemorrhages in 20 others were multiple. In more than one case out of three, lateral hematomas extended into the temporal lobe and in one case out of two, the internal capsule was affected. The incidence of high blood pressure is significantly higher in patients with intermediate haematomas and slit hemorrhages. This factor is often found in lateral or cerebellar hematomas. Anticoagulant therapy is more frequent in lobar hematomas. In 43 cases without any proved etiology, the patients' mean age of death is inferior to that of the whole studied population. These findings are discussed in relation with the literature data.

Aged↗

Moyamoya disease and intracerebral hematoma. Clinical pathological report.

This report concerns a hypertensive woman who suffered a stroke at the age of 51 and recovered from left hemiparesis after 3 to 4 months. During the subsequent 24 years she had four seizures which involved the left arm and face and became generalized, ending with left hemiparesis, from which she recovered after 4 to 5 days. Carotid angiography was performed in 1967, 1973 and 1974 and the characteristic picture of moyamoya disease was demonstrated. She died at the age of 77 with extensive vascular disease. The literature concerning 215 cases of moyamoya disease, in which there were 14 intracerebral hematomas, is reviewed and discussed.

Adult↗

[Diagnosis of cerebral hemorrhages. A report of 247 anatomo-pathological cases (author's transl)].

A series of 247 cases with simple and apparently primitive cerebral hemorrhages selected from the pathological records of Charles Foix laboratory from 1962 to 1977 is reviewed with regard to the accuracy of the premortem diagnosis as a function of the size, the location and the age of the bleeding, the patient's age, the practices of angiography or lumbar puncture and the considered period of death. The clinical diagnosis of cerebral vascular disease was made in 75% of the cases from that serie but the hemorrhagic mechanism was identified only in 50,6% of cases. The diagnosis accuracy was enhanced in large and recent hemorrhages occuring in patients under 70 years old. It seemed better in cerebellar and intermediate locations. It was enhanced in more recent observations (collected since 1974). The practice of cerebral angiography and lumbar puncture rose up (in a somewhat equivalent way) the proportion of accurate diagnoses. The lumbar puncture practice was not associated with a higher rate of cerebral herniae.

Adult↗

[Quantitative and electron microscope study of the nerve in seven cases of sporadic idiopathic sensory neuropathy (author's transl)].

An electron microscope study and quantitation of myelinated and unmyelinated fibers of seven nerve biopsies performed in sporadic cases of idiopathic sensory neuropathy is reported. The number of myelinated fibers is markedly decreased or absent in every case. On the contrary, the unmyelinated fiber numbers are normal or increased. In most cases, the small diameter myelinated and unmyelinated fibers proportions are higher than those of control biopsies. The electron microscope study discloses evidence of degeneration of Wallerian tye and regeneration is also indicated by quantitative studies. Regenerative phenomena seen more obvious in sporadic cases than in previously reported studies of familial cases.

Biopsy↗

[Progressive dialytic encephalopathy. Role of the aluminium and neurological study. One case (author's transl)].

Report a typical case of dialytic dementia in a patient treated with aluminium gels. The course was fatal in fifteen months duration. Before interruption of aluminium gel intake, the aluminium blood level measured by atomic absorption spectrography was at 1300 microgram/l (normal less than 40 microgram/l). Cerebral aluminium was studied by the method of Le Gendre and Alfrey. On the three studied specimens of gray matter including, the parieto rolandic cortex, the thalamus, the cerebellar cortex, the mean aluminium concentration was seven times higher than the witness. The optic and electronic microscopy study showed important accumulation of lipofuscin. No neurofibrillary degeneration was observed. In contrast to the intensity of the clinical signs and the fatal course the cerebral lesions were slight.

Aluminum↗

[Electron microscopic and neurochemical study of Alexander's disease (author's transl)].

The authors report the results of a cerebral and of a neuromuscular biopsies and of the autopsy findings in another infantile case of Alexander's disease in a girl. They review the 17 previously reported cases of this disease and the various etiopathogenic hypotheses mentioned. The presence of numerous, sometimes abnormal enlarged mitochondria and of abundant membranous cytoplasmic bodies in the astrocytic cytoplasm seems to be unreported elsewhere. Peripheral nerve changes are mentioned for the first time.

Astrocytes↗

Analysis of the major lipid classes in human peripheral nerve biopsies. Age group differences and abnormalities of ganglioside level in perhexiline maleate therapy.

We report here the results of a simple and reproducible technique which can be used in semi-routine analysis of peripheral nerve biopsy specimens, so as to have a quantitative analysis of the major lipid classes, i.e. cholesterol, cerebrosides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine + phosphatidylinositol, sphingomyelin and gangliosides. Glycolipid hexoses, cholesterol and total phospholipids have been compared in different age groups. Although all lipid classes increased from the younger to the older age group, the molar ratio of cholesterol to phospholipid differed less than the glycolipid to phospholipid ratio. Both increased significantly, even between age group 10--16 and older patients (36, 54, 61, 68, 72 and 73 years old). Although individual variations in lipid content are noteworthy, it must be emphasized that evolution with age of the lipid composition must be taken into account. Furthermore, this study confirms and extends earlier findings of increased ganglioside levels in some cases of peripheral neuropathies observed during perhexiline maleate therapy where characteristic lipid-like polymorphous inclusions have been demonstrated.

Adolescent↗

[Chronic inflammatory neuromyopathies in adults treated for gluten-sensitive enteropathy. A report on three cases with microvascular nerve and muscle lesions (author's transl)].

Neuromyopathies developed in three patients with gluten-sensitive enteropathy, a long time after they had been cured of their digestive disease by following a gluten-free diet. These cases differed radically from typical deficiency neuropathies by the presence of microvascular inflammatory lesions in nerves and muscles. The semiological findings were similar in all 3 cases, and were distinguished by the association of signs eveking lesions of the largest myelinated nerves fibers to the posterior rami with lesions in the muscles. Corticotherapy improved the condition but did not affect its chronic course. Nerve and muscle biopsies revealed the presence of segmentary microrascularitis, mainly lymphohistiocytic. The probable mechanism of these histological changes is alterations in the circulating immune-complexes, usually found in gluten-sensitive enteropathy, producing various types of associated disorders. Some of these immune-complexes would not be related straight to digestive intolerance to gluten, but would persist during the gluten-free diet period, and could be responsible for the micro-angiitis.

Adult↗