[Recklinghausen's neurofibromatosis. Pathogenesis: current concepts].
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Biomedical subjects
Publications and source records attributed to L Picard.
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Epidural venous system of the human corps are injected by a fortacryl-minium mixture then dissected with detail. These study confirms existence of two longitudinal venous plexus: one anterior and internal, one another anterior and external. There is no posterior longitudinal venous plexus.
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158 pneumoencephalographies of infants less than 2 years old were examined and 53 of them had a large cavum veli interpositi. A neuroradiological description of this cistern is given and a comparison made with injected anatomical specimens. A statistical comparison with 105 pneumoencephalographies without a cavum veli interpositi does not shown any correlation between the presence of this cistern and pathological conditions. This investigation indicates that a large cavum veli interpositi in the newborn has no relation to hydrocephalus or cerebral atrophy. The younger the child, the more frequent is the presence of this cistern, especially under eight months of age.
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The authors describe a technique which permits the extraction of intra-vascular foreign bodies by the intermediary of femoral catheterization. By this method it was possible to extract a catheter below Holter's valve, after accidental migration into the cardiac cavities, in four cases.
In connection with a personal case, 100 chemodectomas of the vagus described in the literature have been collected: revealing themselves in 75p. 100 of the cases in the form of cervical and pharyngeal tumours, 50p. 100 of the tumours of the vagal glomus involve some neurological manifestations mainly in the form of lesion of the lower cranial nerves. These appear to be either affected in isolation (41 p. 100) or in a dissociated manner (59 p. 100). Intra-cranial extension is rare (7 p. 100 of the cases) and delayed. Carotid angiography is characteristic. 76 p 100 are intra-vagal, 24 p. 100 are para-vagal and 60 p. 100 are located in the plexiform ganglion. Multifocal forms which account for 17 p. 100 of the cases are more usually, but not exclusively, familial. Surgical treatment is followed by sequelae or complications in more than half the cases. Close attention has been paid to pathogenetic theories: chemodectomas of the vagus may develop from type I chemoreceptor cells of the carotid glomera or from distinct cells with different properties (S.I.F. cells). They may equally well develop from nonchromaffin paraganglionic cells which have not migrated in the normal manner. The finding of such cells in the nerves of new-born babies and adults supports this theory, at least for some chemodectomas of the vagus.
Experiences from therapeutic embolization by way of superselective catheterization of the external carotid artery, in 40 cases of cranio-cerebral, maxillo-facial and cervical tumours are reported. Using Spongel, embolization offered definite advantages compared to simple arterial ligation: it is easily performed as a supplementary procedure to diagnostic superselective angiography; a meticulous technique will provide superselective arterial occlusion with obstruction of vessels down to the precapillary size (diameter 150 to 200 mu). Embolization is advocated as a preoperative measure in meningeomas (particularly at the skull base), naso-pharyngeal angiofibromas, intracranial and cervical chemodectomas, in which cases profound bleeding during surgery may be expected and as a palliative measure in cases where surgery and radiation therapy fail to control recurrence of a tumour.
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Case report of an 8 days old girl who had a discrete increase in head circumference associated with moderate EEG abnormalities. Familial and obstetrical histories were negative. Skull transillumination, contrast ventriculography and carotid arteriography led to the diagnosis of hydranencephaly. The cerebral lobes whose blood supply is dependent from the internal carotids were missing and were replaced by cavities. The normal neonatal neurological behaviour which did not change till death at 3 months, is a stiking phenomenon.
Intra- and para-bulbomedullary hemangioblastomas are a relatively frequent topographic variety and represent about one third of the spinal cord cases. The surgical approach is particularly difficult in this location, because of adhesion or infiltration of the brain stem by the hemangioblastoma. The authors correlate the angiographic signs and the anatomical findings obtained during surgery. Four topographic types are described: intra-bulbomedullary tumors, usually allowing partial resection only; tumors of the floor of the IVth ventricle extending between the cerebellar tonsils without any infiltration of the brain stem, allowing complete resection; tumors of the cerebellar tonsils with a lateral para-bulbar extension which should allow complete resection, but present with difficult diagnostic problems on angiography; tumors of the cerebellar tonsils with a midline extension present with a particular difficult angiographic diagnosis, but should also allow complete resection.
The total cerebral blood flow was measured by the Xe 133 in 9 patients, before and during a pure Alfatesine induced anaesthesia. With a perfusion flow of 0,04 ml/kg/mn, the total flow underwent a mean fall of 38 p. 100 that was linked neither to any systemic pressure variations nor to variations of the PaCO2. This fall was linked to a cerebral arterio vasoconstriction as it was evidenced by arteriographic images.
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