Biomedical subjects
Publications and source records attributed to P Cornu.
Epidermoid tumors are seldom found as a cause of spinal cord compression; still fewer involve the spinal cord itself. We report a case of a thoracic intramedullary epidermoid tumor documented by MRI.
Forty six patients who had normal pressure hydrocephalus (NPH) and who presented 2 or more of the 5 following preoperative signs: dementia with gait disturbances and urinary incontinence, gait disturbance as first sign, known etiology, positive lumbar puncture withdrawal test, presence of periventricular hypodensity on CT and absence of visible cerebral sulci, were treated by lumboperitoneal shunt. Postoperative results were evaluated at 1 month, 6 months and 1 year with 60.6 p. 100 of good results. Complications of LP shunts (26 p. 100) were mild and did not compromise the outcome. We emphasize the necessity of clinical selecting criteria before surgery and the value of LP shunt in the treatment of NPH.
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The topography and magnitude of increase in peripheral type benzodiazepine binding sites (omega 3 sites) was investigated autoradiographically in the brains of patients with ischemic cerebrovascular disease, with multiple sclerosis, and with malignant glioma. 3H-PK 11195, a selective omega 3 site ligand, was employed. A manyfold increase in omega 3 site density was observed in all these disease states; this increase reflects macrophage invasion or glial proliferation or both as demonstrated by neuropathological studies carried out in parallel. There was an excellent spatial correlation between increased omega 3 site densities and extent of the lesion histologically. Specifically, an elevated density of omega 3 sites was observed in the plaques of demyelination in multiple sclerosis patients, in the periphery of infarcted zones in stroke patients, and throughout tumor in patients with grade IV astrocytomas. As our approach is applicable to both tomographic (in vivo) and autoradiographic investigations, imaging of omega 3 sites may be considered for the detection and monitoring of the natural evolution of many disorders of the human central nervous system.
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A metastasis from a breast carcinoma developed in the cervical cord. The localisation and nature of the lesion were easily diagnosed by a contrast MRI study with Gadolinium DTPA.
A qualitative abnormality of antithrombin III (AT III) was found in the plasma of a 41-year old patient. The plasmatic AT III antigen concentration was 130% and the progressive anti-F IIa and anti-F Xa activities were normal (105% and 137%). The plasma heparin cofactor activity was less than 10%, when measured by F IIa or F Xa inhibition. Crossed immunoelectrophoresis of AT III in the presence of heparin revealed in the plasma an abnormal slow-moving peak. When tested by affinity chromatography on heparin Sepharose, this abnormal AT III did not bind to heparin. Among the investigated relatives, 5 subjects had normal AT III levels, whatever the test used, the nine others having reduced levels of antithrombin heparin cofactor activity (45-61%) but normal levels of immunoreactive AT III (97-122%). Consanguinity was found in the family history. We therefore considered our patient as homozygous for an AT III molecular abnormality affecting the binding site for heparin.
The human parvovirus (HPV) is responsible for aplastic crises in patients with chronic haemolytic anaemia. We describe the cases of four children with aplastic crises in various types of thalassaemia (alpha and beta thalassaemias, major and intermediate forms). In all four patients, specific anti-human parvovirus IgM was detected in their serum, thereby indicating recent infection.
A simple and reliable method of quantifying tissue damage is described. This method, based on atomic absorption spectroscopic determinations of Na, K, and Ca concentrations in small brain samples, was applied to the rat middle cerebral artery occlusion model (MCAo). At the infarct site by 24 hours, Na concentration more than doubled, Ca concentration increased by greater than 70%, and K concentration fell nearly 80%; these changes are consistent with a greater than 80% disruption of cells. A remarkable acceleration of ionic shifts occurred between 4 and 6 hours after MCAo. At 4 hours, only 20-30% of the ionic shifts found at 24 hours had occurred; by 6 hours, 80-100% of the ionic shifts found at 24 hours had taken place. Since the measurements reflect ionic movement into and out of the tissue, they are likely to represent irreversible tissue damage. Although blood brain barrier breakdown may have contributed to an increased rate of ionic shifts, large ionic gradients must have been present between the extracellular space and the vascular compartment at 4-6 hours to drive the ionic shifts. Our results suggest an upper time limit of 4 hours for treatments of acute ischemic tissue damage in the rat MCAo model. The methods and analytical approach described may be useful for determining the time window for therapeutic intervention in acute CNS injuries, as well as for evaluating treatment effects.
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The influence of blood transfusion on erythropoiesis (bone marrow erythroblasts, peripheral blood erythroblasts and reticulocytes) has been studied in 20 non splenectomized homozygous beta thalassaemia patients aged 3 to 16 years and in 10 splenectomized patients aged 8 to 24 years affected with the same disease. The number of reticulocytes was the same in the two groups but the number of erythroblasts in the splenectomized group was higher than in the other group. There was no correlation between the erythroblasts and the reticulocytes of the peripheral blood on one hand and the haemoglobin level proceeding from the same sample on the other hand. In the non splenectomized group of patients, an inverse relationship was found between the percentage of bone marrow erythroblasts and the mean annual haemoglobin level (r = -0.71; p less than 0.01). These results demonstrate the effect of blood transfusion on the erythroid cell line in homozygous beta thalassaemia and the delay between the transfusions and the medullary erythroblastic response.
Ten cases of multiple myeloma with spinal cord compression are reported. The compression was located in the thoracic spine in 9 cases and in the cervical spine in 1 case. It led to the discovery of the myeloma in 4 cases. Three patients suffered, during several months, from local pain aggravated by activity and from slight and slowly progressive neurologic symptoms resembling intermittent claudication. At the time of diagnosis, sphincter dysfunction was observed only in patients with low thoracic cord compressions. In 4 cases, lesions were first treated by radiotherapy which did not produce regression of the compression. Tumor excision surgery was carried out seven times, once after failure of radiotherapy. In 6 cases an definite and steady regression of the neurological symptoms was achieved. Survival varied from 10 months to 7.5 years after identification of spinal cord compression. Survival was equal to or more than 3 years in 4 patients and will probably reach 3 years in another. Thus spinal cord compression is not by itself a sign indicating a poor short term prognosis in multiple myeloma. It should be treated by excision surgery, then by chemotherapy as in multiple myeloma at other sites.
Fifty-three patients with severe aplastic anaemia were admitted to this hospital between January 1976 and June 1980, of whom three arrived in terminal condition and died before treatment for their basic disease could be given. Thus 50 patients were treated and evaluated in a prospective study according to one protocol. Eighteen patients with an HLA-identical sibling underwent bone-marrow transplantation with the aim of achieving haematopoietic chimerism. Thirty-two patients without an HLA-identical sibling were given antilymphocyte globulin with or without an infusion of HLA-haplotype-identical marrow. All these 32 patients received low-dose androgens after the procedure. In the first group eight patients (44%) survived. In the two other groups, 22 patients survived (69%), of whom 20 were completely self-sustaining (63%). Engraftment and graft-versus-host disease did not occur in the group who received antilymphocyte globulin and haploidentical marrow, and the haematopoietic reconstitutions in these patients were all autologous. These results confirm the efficacy of antilymphocyte globulin in the treatment of severe aplastic anaemia and show that such treatment is at least as good as bone-marrow transplantation. Its mechanism of action remains unknown, but most patients with aplastic anaemia have a pool of haematopoietic stem cells able to repopulate the marrow after this type of treatment.
Report on 5 patients with thrombotic thrombocytopenic purpura. Four of the five survived. The following forms of treatment have been used: up to 1976, splenectomy and corticosteroids; since 1976, antiplatelet agents and exchange transfusions, and since 1978, antiplatelet agents and fresh plasma or plasma exchange. The author's experience is identical to that in the more recent case reports: early treatment with antiplatelet agents and fresh plasma-in case of failure with plasma exchange - appears to improve the hitherto fatal prognosis.
Experience with a total of 49 plasma exchanges in 14 patients is reported. All exchanges were performed with the Aminco cellseparator in an attempt to remove documented or suspected antibodies completely. Indications were ABO-barrier before bone marrow-transplantation (6), acute rejection following renal transplantation (3), autoimmune diseases (3), Moschowitz disease, antithrombocytic antibodies and hepatic coma (1 each). The technique was modified to make it possible routinely to exchange 121 of plasma in 4 hours with no major untoward side effects even in anuric or thrombopenic patients. Complete removal of circulating antibodies was possible and often followed by impressive clinical benefit.
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Experience with 17 allogeneic bone marrow transplantation (BMT) patients with refractory acute leukemia is reported. The authors show that it is extremely difficult to cure patients with end stage disease by this procedure. Based on experience that, if performed early, BMT can bring about over 50% potential cures, it is postulated that marrow grafts should be performed much earlier than is done at present. From the results in 45 patients with severe aplastic anemia it is concluded that the majority do not have a defect at the pluripotent stem cell itself. There is disturbed maturation to functional end cells which can be corrected in most instances by ALG, infusion of allogeneic marrow and low dose androgens. BMT between HLA-identical siblings need not cause major problems, but it involves a considerable risk of fatal complications such as irreversible marrow rejection and GvH.