Posttraumatic common carotid artery dissection.
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Biomedical subjects
Publications and source records attributed to F Woimant.
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At the acute stage of cerebral ischemia, the "therapeutic window" probably does not last more than 6-12 hours. Despite similar treatments, patients admitted in stroke specialist units are more likely to survive and to have a good functional outcome than patients treated in general wards. In most cases acute arterial hypertension should not be treated. Thrombolytic agents given within 6 hours after onset, are now under evaluation in several clinical trials. There is no scientific evidence to support the use of anti-coagulation as a curative treatment of acute cerebral ischemia; however, clinical trials remain necessary, especially in progressing stroke. Neuroprotective drugs protect neurons against the consequences of hypoxia in animals: most clinical trials with oral nimodipine led to negative results but the meta-analysis suggests that patients receiving nimodipine within 12 hours after stroke onset might have a lower mortality rate and a better functional outcome. Other clinical trials with neuroprotective drugs are currently running: anti-NMDA drugs, chlomethiazol, tirilazad, ganglioside GM 1, etc Most therapeutic agents are now under evaluation. An early admission of patients with acute stroke is required to evaluate therapeutics agents which probably cannot be effective if started more than 6-12 hours after stroke onset.
OBJECTIVE: Free glycoprotein hormone alpha-subunit plasma levels have been reported to be increased in glycoprotein hormone-secreting adenomas and in acromegaly, but rarely in prolactinomas and in only two cases of Cushing's disease. The prevalence of elevated plasma alpha-subunit levels in patients with non-functioning adenomas is still unclear. In addition, no previous work has described plasma alpha-subunit levels in a comprehensive series of adenomas characterized by in-vivo secretion and/or immunocytochemistry. PATIENTS: Thirty-seven patients with definite prolactinomas and 48 with non-functioning tumours characterized by immunocytochemistry were studied, from a series of 145 consecutive patients including 33 acromegalics, 18 patients with glycoprotein hormone-secreting adenomas and 9 with Cushing's disease. MEASUREMENTS: Plasma free alpha-subunit was measured by radioimmunoassay in all patients and in a large sample of normal subjects to establish normal ranges according to sex, age and menstrual status. Tumour volume index was the product in cm3 of length, width and height of the adenoma as assessed by computerized tomography or magnetic resonance imaging. RESULTS: Twelve of the 37 (32%) patients with prolactinomas had increased plasma alpha-subunit levels; their tumours were significantly larger with significantly higher plasma PRL levels than those of patients without increased plasma alpha-subunit levels (P < 0.02). All prolactinomas above 50 cm3 were associated with alpha-subunit secretion, whereas only 6 of 29 smaller tumours were similarly associated. Twelve of the 48 'non-functioning' adenomas actually secreted alpha-subunit in vivo: 8 gonadotrophin-secreting, 2 'pure' alpha-secreting, one with negative immunocytochemistry and one necrotic adenoma. Their volumes were significantly higher than those of adenomas without increased plasma alpha-subunit levels (P < 0.04). Plasma alpha-subunit levels were increased in the 6 patients with TSH-secreting adenomas, 8 of 12 with FSH-secreting adenomas, 11 of 33 acromegalics and none of those with Cushing's disease. CONCLUSION: Plasma free alpha-subunit levels were increased in 49 of 145 patients (34%). For prolactinomas and 'non-functioning' adenomas, alpha-subunit hypersecretion was seen more often with larger tumours. Half of the cases with increased free alpha-subunit in this series were patients harbouring an adenoma which did not stain for an intact glycoprotein hormone.
The authors report on a Portuguese family with 3 adult brothers affected with GM2-gangliosidosis (B1 variant) in a sibship of 4, and more specifically on one of these brothers with neurological onset at the age of 17. Psychosis, lower motoneuron involvement and dysarthria were predominant in two of the cases; the third had a cerebellar symptomatology. Hexosaminidase A activity, studied in leukocytes, was profoundly deficient when measured using the specific sulfated substrate, but nearly normal using a conventional assay (non-sulfated substrate). These results established the diagnosis of the unusual enzymological form of GM2-gangliosidosis known as the B1 variant, which had so far not been associated with an adult phenotype. Molecular studies are in progress to study genotype/phenotype correlations in this family in comparison with known mutations in the B1 variant and in adult GM2-gangliosidosis. This report also emphasizes that a metabolic etiology, leading to genetic counselling, should be considered in some familial degenerative neurological disorders.
Subependymomas are uncommon, benign, slowly growing lesions usually located in the fourth ventricle. Their morphology is characteristic, but their histogenesis remains controversial. Seven cases observed in our laboratory over a period of 7 years are reported. Most tumors were located axially in the brain stem, and symptoms were more variable than usually reported. The histological appearance was fairly uniform. Immunohistochemistry showed that the fibrillary component was composed of glial fibers. Ependymary differentiation is inconstant on electron microscopy. The pathogenesis of the lesion (tumor, hamartoma or reactive proliferation) is debated in the literature as is the histogenesis.
Although no treatment has yet been shown to be effective during the acute phase of cerebral ischaemia, several treatments are currently under investigation: treatments designed to improve cerebral perfusion (fibrinolytics) or limit cellular lesions and protect cerebral metabolism (neuroprotectors, antioxidants). However, these treatments can only be effective when they are started very early, during the first hours following onset of ischaemia. At the present time, simple measures must be rapidly applied to limit extension of ischaemia and to prevent the development of complications, in order to improve the functional prognosis.
OBJECTIVES: To determine whether migraine is a risk factor for ischaemic stroke. DESIGN: A case-control study. SETTING: Two hospitals in Paris. SUBJECTS: 212 patients with stroke (137 men and 75 women) and 212 controls matched for sex, age (to within five years), and history of hypertension. MAIN OUTCOME MEASURES: Ischaemic stroke, confirmed by brain computed tomography or magnetic resonance imaging, and history of headache, recorded with structured questionnaire during interview. RESULTS: Prevalence of migraine did not differ between patients with stroke and controls: 18/137 v 17/137 for men (odds ratio 1.1 (95% confidence interval 0.5 to 2.2), p = 0.86); 23/75 v 17/75 for women (odds ratio 1.6 (0.7 to 3.5), p = 0.24); and 41/212 v 34/212 for both sexes (odds ratio 1.3 (0.8 to 2.3), p = 0.33). When subjects were split into two age groups, however, prevalence of migraine was significantly higher among younger women (aged < 45) with stroke compared with their controls (13/20 v 6/20, odds ratio 4.3 (1.2 to 16.3), p = 0.03). Furthermore, the risk of ischaemic stroke was higher among younger women who smoked (7/20 v 1/20, odds ratio 10.2 (1.1 to 93.3)). CONCLUSIONS: Prevalence of migraine was not different between patients with stroke and matched controls except among women aged < 45, when migraine and stroke were significantly associated.
Transient myasthenia gravis was observed in an infant whose mother had no signs of neuro-muscular disease. The case presented with severe polyhdramnios at 31 weeks. Sonographic and invasive work-up showed only an absence of fetal swallowing. At birth, the infant had severe muscle weakness and respiratory distress. He had high titres of anti-acetylcholine receptor (anti-AChR) antibodies of maternal origin. Anti-AChR antibodies were also found in stored samples of fetal serum and amniotic fluid. To our knowledge, this is the first case of neonatal myasthenia gravis for which neither past nor present evidence of disease could be obtained in the mother. Anti-AChR antibody testing should be considered in cases of unexplained polyhydramnios.
The aim of this study was to evaluate the role of endovascular treatment for intracranial mycotic aneurysms. The clinical and angiographic features of three patients with endocarditic vegetation (two with Streptococcus viridans and one with Staphylococcus) were reviewed retrospectively. Patients were selected for this treatment according to the clinical setting and aneurysm location. In two cases, selective catheterization of a distal middle cerebral and posterior cerebral artery branch with a microcatheter followed by superselective amobarbital testing of the parent vessel was preliminary to the occlusion of that vessel with autologous clot or glue. The third patient was treated by selective occlusion of the aneurysm by intra-aneurysmal placement of platinum minicoils. Two patients presented with intracranial hemorrhage and in one the lesion was found on computerized tomography. All three aneurysms had been excluded from the circulation at the 6-month follow-up review. The only complication from the procedure, despite the septic nature and distal localization, was balloon deflation in one patient, who was successfully retreated with coils. Endovascular embolization is indicated in patients who are at risk of hemorrhage and cannot undergo the standard procedure. The superselective amobarbital test allows selection of patients who will tolerate distal vessel occlusion. This endovascular procedure represents a safe and effective treatment for these lesions.
A case of HILV1-associated adult T cell leukaemia/lymphoma (ATLL) in à 21-year olf African woman is reported. The patient presented with lymphomatous meningoradiculopathy. The usual clinical features of ATLL were absent. Lumbar MRI showed a pial enhancement by DTPA-gadolinium of the conus medullaris which extended to the proximal cauda equina. Under systemic chemotherapy coupled with intrathecal chemotherapy the patient progressively improved, and at the second MRI examination complete disappearance of the lumbar enhancement was observed. MRI of the brain using axial and coronal T2-weighted sequences detected multifocal lesions of high-intensity signal in the subcortical white matter. ATLL is unusual in people of African origin. The ATLL-strongyloïdes infestation association has previously been reported, suggesting that parasitic infestation may be an important co-factor leading to the development of ATLL.
A patient, aged 26, had a large parietal tumor, clinically revealed by intracranial hypertension and left sized paresthesia. Removal of tumor was followed by a rapid recurrence. Rhabdomyosarcoma was diagnosed on the second surgical specimen. These tumors are rare in the central nervous system. They occur mostly, though not exclusively, in young adults. Their prognosis is poor and their histogenesis is still unclear.
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Internal carotid artery dissection is a major cause of ischemic stroke in the young. Pain is the leading symptom and is associated with other focal signs such as Horner's syndrome and painful tinnitus or with signs of cerebral or retinal ischemia. We report two patients with angiographically confirmed extracranial internal carotid artery dissection presenting with cephalic pain as the only manifestation. The first patient had a diffuse headache and a latero-cervical pain lasting for 12 days, reminiscent of carotidynia. The second patient experienced an exploding headache suggestive of subarachnoid hemorrhage, which was ruled out by computed tomography of the head and cerebrospinal fluid study. These patients demonstrate that recognition of carotid artery dissection as a cause of carotidynia and headache suggestive of subarachnoid hemorrhage may permit an earlier diagnosis and possibly the prevention of a stroke through the use of anticoagulation.
Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
Fifteen patients were observed between 1987 and 1990: there were six with angiographically confirmed vertebral artery dissection, and 9 with carotid artery dissection. Results showed concordance of MRI and angiographic findings, in all cases but one. The dissected portion consistently showed a semilunar hyperintensity narrowing the residual eccentric signal void of the lumen when the artery was not completely occluded. In one angiographically occluded vessel, MR detected a small signal void within the hyperintensity, indicating that the artery was not completely occluded. The length of the dissected portion was clearly demonstrated by MR. Follow up MR and angiographic studies confirmed the regression of the dissection, and also allowed examination of the cerebral parenchyma.
A woman, who presented with clinical and radiological signs of a right temporal mass suggestive of a brain tumour, was found to have granulomatous angiitis associated with cerebral amyloid angiopathy; the diagnosis was confirmed by biopsy. She is still well 13 years after excision of the lesion. The association of granulomatous angiitis and cerebral amyloid angiopathy constitutes a peculiar variety of central nervous system micro-angiopathy. Only a few similar cases have been described.
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Three patients from a single family of six siblings had homocystinemia and homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency and had severe recurrent strokes in adult life. Two of the patients died 1 year after clinical onset.