[Cerebrovascular complications disclosing Conn's adenoma].
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Biomedical subjects
Publications and source records attributed to M Chatel.
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A 28-year-old woman with no history of seizure was 7 months pregnant when she developed a prolonged complex partial status epilepticus (CPSE) organized in recurrent complex partial seizures of occipital origin, which was ascertained by the presence of elementary visual hallucinations and nystagmus heralding the attacks. EEG demonstrated recurrent seizures starting from the right occipital area. This especially refractory case of CPSE resolved after treatment with antiepileptic drugs and termination of pregnancy by cesarean section.
Aphasia as the sole manifestation of focal status epilepticus in adult is rarely described. Such a case is reported here. Two separate episodes were studied: 1) prolonged mixed aphasia with continuous epileptic patterns; 2) Epileptic aphasia organized in shortly spaced temporal seizures marked by total suspension of speech, paraphasic utterances characterizing the interictal period. The clinical and EEG criteria and nosographic situation of this syndrome are reviewed. We conclude that epileptic aphasia exists if a fixed and lasting epileptic condition is created, even if a post-ictal phenomenon accounts for the symptomatology.
Ependymoblastoma tumour bearing mice were treated with sarcosinamide-CNU and compared with untreated mice with regard to tumour growth, pathological examinations and levels of phosphorylated metabolites measured by in vivo nuclear magnetic resonance spectroscopy. The response to the drug was demonstrated by its effect on tumour growth and with pathological examinations. Spectra evolution was different from controls for only four treated tumours out of eight.
Treatment of gliomas remains disappointing in spite of a great number of experimental biological data and of randomized therapeutic studies. This could be partly explained by the inefficiency of our conventional methods to assess the regional metabolism of these tumors. The use of positron emission tomography (PET) brings encouraging possibilities in this field. We report our preliminary experience of measuring regional cerebral methionine uptake with PET after intravenous injection of [11C]L-methionine. Twenty-two patients with histologically confirmed gliomas were studied. An ECAT II positron emission tomograph was used for scanning. The position of the plane was chosen to include a major section of the tumor in the reconstructed brain slice. The protocol required a two-step examination: 1) after injection of 15 to 25 mCi of [11C]L-methionine, 12 scans were performed over a period of 46 minutes; and 2) 18 hours later, regional cerebral blood volume was measured in the same slice after intravenous injection of 2 to 4 mCi of 68GaCl3. The tumoral region of interest was determined as being the area of maximum activity. For each patient we calculated the ratio, R, between the activity in this tumor region of interest and the activity in the contralateral healthy symmetric region of interest which was used as an "internal standard" for the same patient. We correlated the ratio R with the histological grading. In 22 patients, mean values of R were calculated for each tumor: Grade II (n = 5): R = 1.04 +/- 0.27; Grade III (n = 5): R = 1.68 +/- 0.22; and Grade IV (n = 12): R = 2.33 +/- 0.86.(ABSTRACT TRUNCATED AT 250 WORDS)
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Forty adults with recurring brain gliomas were treated with HECNU 130 mg/m2, given i.v. every 5 to 6 weeks (mean 5.4) and corticosteroids. According to the response to treatment, patients were divided in 3 groups: 1. Group 1 included 8 pts (20%) with objective remission, defined as a clear-cut clinical improvement persisting at least 4 weeks after the complete discontinuation of corticosteroids. 2. Group 2 included 14 patients (35%) who improved or remained stable yet stayed corticosteroid-dependent. 3. The 18 patients (45%) of group 3 failed to respond. There was a fair correlation between clinical and radiological response. Thus sequential CT-scans showed a 50 to 100% tumour reduction in all patients of group 1, and in 5 of group 2. CT-scans remained unchanged in 8 patients of group 2 and in one of group 3, and showed tumour progression in 10 patients of group 3. Drug toxicity appeared mild, reversible and was not cumulative. The better tolerance of HECNU could represent a real advantage of this drug over the commonly used nitrosourea derivatives such as BCNU and CCNU.
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A long-term open multicenter trial was carried out in 15 European centers with therapy-resistant epileptics to evaluate the efficacy and safety of progabide, a new antiepileptic GABA receptor agonist; 187 patients, suffering from partial epilepsy (57%), primary generalized epilepsy (20%), secondary generalized epilepsy (21%), and unclassified generalized epilepsy (2%), participated in the study. All patients had a total seizure frequency higher than one per month in spite of standard antiepileptic medication; 46% had a mean partial seizure frequency from daily to weekly. Progabide was administered at a mean daily dose of 30.5 mg/kg/day as an add-on to the standard antiepileptic drugs up to one year in 115 patients; 37 patients (19.8%) dropped out because of reasons which were not drug-related (bad compliance, lost to follow-up); in 12 patients (6.5%) progabide was withdrawn for side effects and in 20 (10.7%) for lack of efficacy. 71.3% of patients treated for one year (62% considering the 'cumulative' number of patients) experienced more than a 50% reduction in seizure frequency. This reduction was equally present in patients with partial epilepsy (63.9%) and with generalized epilepsy (62.2% of patients with primary and 57.1% with secondary generalized epilepsy). No signs of tolerance phenomena to the antiepileptic effect of progabide were observed. No side effects were reported in 56.7% of the patients. Clinical side effects were mild and transient, leading to progabide discontinuation in 6.5% of the patients only; an increase in SGPT was observed in 5.7% of the patients, these increases were transient and without any clinical symptom.(ABSTRACT TRUNCATED AT 250 WORDS)
A young man with a left hemifacial hemangioma had during a six months period about forty left hemispheric neurologic attacks suggestive of classic migraine. The neurologic examination was normal during the attack-free period. The CT scan (fig. 1) and the M.R.I. study (fig. 2) only showed a moderate interhemispheric asymmetry. The left internal carotid angiogram showed subtle anomalies of the venous system (fig. 3). All the neurologic manifestations ceased as soon as therapy by aspirin was initiated. A Positron Emission Tomography (PET) study with the oxygen 15 continuous inhalation technique was performed 7 months after the last attack in order to measure the regional Cerebral Blood Flow (rCBF), Oxygen Extraction Fraction (rOEF) and Oxygen Consumption (rCMRO2). Striking, statistically significant, alterations were observed in the left temporo-parieto-occipital area (fig. 4) consisting of a "misery perfusion" syndrome (rCBF = 28-38 ml/100 g/mn; rOEF = 0.64-0.80), without alteration in the rCMRO2 (Table). A repeated PET study 12 months later was unchanged. The association of local chronic oligemia and ipsilateral facial hemangioma, ipsilateral cerebral hypotrophy and venous anomalies suggested the diagnosis of atypical leptomeningeal angiomatosis of the Sturge-Weber type. The importance and persistence of the hemodynamic alterations suggest that chronic oligemia and, hence, tissue hypoxia may participate in the pathogenesis of the migraine-like attacks. Moreover, local circulatory stasis with thrombotic events may be implicated, as suggested by the apparent efficacy of aspirin.
137 samples of intracranial tumours have been studied in proton NMR spectroscopy. T1 and T2 relaxation times are above those of normal grey and white matter. Differential diagnosis between benign and malignant brain tumours does not seem feasible upon proton T1 and T2 alone. Histological correlations allowed us to specify secondary changes accounting for T1 and T2 variations (oedema, microcyst, stroma reaction, necrosis).
Brain protein synthesis may be evaluated in vivo by a PET three compartment methionine model. 14 human brain tumor patients were studied. Protein synthesis rate (PSR) was increased in any glial tumor even in low grades, but this increase was statistically more important in anaplastic tumor. Radiotherapy action was evaluated in two patients. Local tumoral PSR was reduced to normal brain PSR after treatment. No difference was seen in normal cortex contralateral to the lesion between pre and post radiotherapy examination. 11 C-L-Methionine incorporation measured by PET looks as a very sensitive method for studying tumor metabolism and treatment effects.
Polyamine levels have been studied in brain tumor patients. We focused our study on the relationship between tumor, cerebrospinal fluid (CSF) and red blood cell (RBC) polyamine levels. Our results are the following: Polyamine levels in CSF are consistently increased, whatever the histological type may be. The highest tumoral concentrations are found in medulloblastoma. In glioblastomas, the RBC spermidine levels are higher than in the other types of tumors and there is a highly significant correlation between the spermidine/spermine ratio in tumor and RBC. Therefore, RBC polyamine determination might be of clinical interest in the monitoring of patients with glioblastomas.
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Dissociation and centrifugal elutriation procedures were applied to subcutaneous transplants of the OTT-6050 mouse teratoma line in order to enrich the neuroepithelial cells. One of the resultant cell fractions, designated IB-21, was then implanted beneath the renal capsule of syngeneic mice and rebanked every 3 to 6 weeks for a total of 58 passages over 5 years. Sequential passages resulted in a tumor restricted to stem cells and neural cells (neuroblasts and glial cells). The primitive neural cells lost the ability to form rosettes after the early transplants. Subcutaneous or intracerebral transplantation of these tumors evinced their capacity for further neuroepithelial differentiation, with the demonstration of astrocytes and occasional mature synapse-forming neurons. Conversion of the tumor to the ascitic form resulted in unorganized clusters of neoplastic cells in contrast to the highly structured embryoid bodies that are characteristic of the parent Ott-6050 line. The absence of non-neural cells in the IB-21 tumor fraction and its ability to demonstrate divergent neural differentiation suggest that a transplantable neural-determined cell population exists in the OTT-6050 mouse teratoma.
The intranuclear filamentous inclusions of a human glioma were analysed with an electron microscope equipped with a goniometer stage. The inclusions consist of 6 to 8 filaments. Considering the organization of the constituent filaments we distinguish three basic types: 1. Filamentous bundles of more or less parallel filaments, forming a cigarshaped inclusion. 2. Crystalloid inclusions: a. Prisms. They consist of stacked layers of strictly parallel filaments. The angle formed by the filaments of adjacent layers if 60 degrees. b. Cylinders. The layers of filaments are bent up and may form either a circle or a spiral, when the inclusion is seen in cross-section. 3. Partially crystalloid or "intermediate" inclusions. We consider them to be transitional forms between types 1 and 2 inclusions. The crystalloid layers of such intermediate inclusions may form either prisms or cylinders. Finally, the similarity between the granulo-fibrillar capsules surrounding granular nuclear bodies and filamentous inclusions, as well as the existence of granular material dispersed between the filaments of some inclusions led us to investigate a relationship between these two structures.
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The authors summarize briefly the biological basis of therapeutic regiments against malignant gliomas. They outline some factors able to contribute to the poor results that are obtained with the now in use strategies: paucity of drugs, tumoral sites out of drug access, inadequate timing of prescriptions, variable and unpredictable drug sensitivity of cells in one given tumor. They encompass the eventuality of progress upon the use of new laboratory methods allowing a better under standing of tumor biology.