[Urologic manifestations in Wegener's disease].
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Biomedical subjects
Publications and source records attributed to A Verier.
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We report 2 cases of occlusion of the internal carotid artery in young, non-atherosclerotic patients with essential thrombocythemia (ET). This complication of ET seems to be rare, but is probably underdiagnosed since transient ischemia, which is frequent in ET patients, is seldom explored by angiography. The excess of thrombocytes and the thrombopathy may account for the microvascular complications, but in the absence of any other contributory factor, occlusions of large arteries, such as the ICA, is more difficult to explain.
A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.
A 21 year-old man presented with a history of sudden onset of aphasia and headache. CT showed a left parietal hypodensity and pallidal calcifications. The ECG showed a Wolff-Parkinson-White's syndrome. The patient then developed successively focal epileptic seizures, temper disorders, a cardiomyopathy, a pepper and salt retinopathy with hemeralopia, a left hemiplegia, deafness, and fever of unexplained origin. Left carotid angiography showed thin, irregular or occluded branches of the middle and anterior cerebral arteries. Blood muscle enzymes, lactate and pyruvate, were elevated with acidosis. Muscle biopsy revealed a mitochondrial myopathy and blood chemistry showed a severe deficiency of respiratory chain enzymes. Death occurred after 28 months. This case showed the diagnostic features of Melas, with some elements of the Kearns-Sayre syndrome. To our knowledge, this is the first case were serial angiographies allowed demonstration of arterial changes capable of explaining cerebral infarctions.
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A woman presented with a history of three regressive comas of undetectable etiology between the age of 52 and 57 years. An IgG lambda benign monoclonal dysglobulinemia was combined with a papular mucinosis (myxedematous lichen or the generalized form of Arndt-Gotton's scleromyxedema). In the 6 analogous cases documented in the literature the onset of coma occurred generally several weeks after an aggravation of the cutaneous lesions. The coma was preceded by an influenza-like syndrome followed by asthenia, malaise with vertigo and frequently epileptic seizures. During recovery, hallucinations and transient hepatic disorders were noted. Pruritus with pronounced hypereosinophilia preceded desquamation and regression of dermatologic lesions. These comas can lead to a fatal outcome (2 of 7 cases) or regress in 2 to 20 days usually without sequelae. The disease is probably of immunologic origin. The paraprotein or a serum factor could exert a direct toxic effect on brain. As in neurologic manifestations of malignant dysglobulinemia, explained initially by a "toxic encephalosis, clinical, angiography, biologic and immunologic data exist in favor of blood hyperviscosity. This hyperviscosity could result from polymer formation through intermediates immunoglobulins and other protein chains, or again from alteration of deformability of red cells by binding of paraprotein. Hyperviscosity syndromes are frequent in system diseases that are often associated with papular mucinosis. Whatever the exact mechanism of these "comas due to papular mucinosis", a logical choice is their treatment by immunosuppressants and plasmapheresis: in the case reported, the use of plasmapheresis as soon as premonitory signs had appeared probably prevented a fourth coma.
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Two cases of intraspinal meningeal hemangiopericytomas are reported, one of which cervical and the other one dorsal with relapse. The anatomopathological features of the hemangiopericytomas are recorded and the nosological problems of their meningeal localizations are discussed. As in the case of intracranial meningeal hemangiopericytomas, the clinical, radiological and macroscopic features of intraspinal hemangiopericytomas--to the exception of a few particular cases--resemble those of other localizations of these tumours more than those of meningiomas, in the light of the review of twenty-eight cases to be found in the relevant literature. Treatment of such tumours hasn't as yet been well circumscribed. Surgical treatment may be facilitated by preoperative embolization. Radiotherapy must be associated to it, at least when removal is incomplete, because of frequent recurrence.
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A critical study of 11 cases with neurological complications resulting from damage to the cervical spine during rheumatoid arthritis prompts the authors to reconsider their therapeutic strategy. After recalling the various neurological complications and their mechanisms, they propose an extension of the range of indications for surgical treatment. The limits of this extension are defined according to neurological and radiological criteria and the risks inherent in each method.
SEP contribution to diagnosis is interesting but limited to hardly examined complete syndromes in an emergency care unit. The predictive value of SEP is high if electrophysiological data are correlated the 10th. day with the clinical status. A good prognostic value is shown when the clinical posterior column and pyramidal tract dysfunctions are equal in intensity and distribution, i.e. in complete syndromes or central spinal cord syndromes or Brown-Sequard's ones. However, not any correlation exists in cases of anterior spinal cord syndromes. Dg.EMG. is an easy, atraumatic useful tool for the clinician. It must be a systematic approach of the brain stem and cervical spinal cord phrenic centers vitality that may be involved by the trauma. When a respiratory deficiency occurs, it allows the diagnosis of a "peripheral" or "neurological" etiology. Its high prognostic value for m tor diaphragmatic function (increase or decrease) must be discussed before any therapeutic decision.
A 36 year-old caucasian woman was operated and then irradiated for a pituitary adenoma. Two years later, a left anterior temporal lobectomy for a grade I astrocytoma was performed. Four years later, she experienced right hemiparesis and aphasia. CT scan showed a left temporo-occipital low density area. A left carotid angiogram showed a narrowing of the left carotid artery beginning in the lower part of the siphon and progressing to a complete supraclinoid occlusion. There was a collateral circulation of the Moyamoya type. Radiation-induced narrowing or occlusion of the intracranial internal carotid artery is an infrequent finding. Most cases appear in young subjects, several years after a high dose of radiation therapy (30 to 60 grays). Some cases may show a network of the Moyamoya type. Usually, the absence of vascular abnormalities prior to radiation cannot be demonstrated. In our case, as in 3 other cases of the literature, the intracranial vessels were of normal appearance before irradiation. The vascular lesions can thus be considered as acquired and secondary to radiation therapy.
On the grounds of studies carried out in the last ten years, the authors criticize the usual classification of comas and question the Glasgow scale (opening of eyes, verbal answer, motor response). Their description of brain stem injury by axial herniation and explanation of the role of diffuse encephalic lesions causing intracranial hypertension is based upon anatomo-clinical findings and results of investigations in comatose patients with head injury. For prognostic purposes, a simple and fairly reliable classification of post-traumatic comas is proposed. Comas are divided into five stages of rostro-caudal destructuration (cortico-sub-cortical, diencephalic, meso-diencephalic, mesencephalic and pontic) by studying the response to pain (unadapted, adapted, absent) and four brain stem reflexes (fronto-orbicular, vertical and horizontal oculocephalic, light reflexes).
Severe head injury often produces complex intracranial displacements of the brain, resulting in widespread, often microscopic lesions. These are responsible for two types of edema: vasogenic edema, with outflow of molecules and fluid into the extracellular spaces by rupture of the blood-brain barrier and vasoplegia, and cytotoxic edema, with swelling of astrocytes due to membrane lesions. The connexions between these two types of edema are still obscure. Alterations in membrane phospholipids may impede function of Na-K pump enzymes, causing accumulation of water in the cell. Cerebral edema is responsible for intracranial hypertension and tentorial herniation, which in turn increase edema through venous compression, ischemia, and hypoxia. The least controversial anti-edema therapeutic measures include relative fluid and salt restriction, mannitol if called for, neuroplegia, in particular with diazepam and Gamma-OH, and assisted ventilation.
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