[Schwannoma of the second part of the duodenum. A case operated on as an emergency (author's transl)].
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Biomedical subjects
Publications and source records attributed to B Pasquier.
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The authors report two cases of "sinus pericranii" a rare vascular tumor of the skull, communicating with the meningeal veins or with the dural sinuses. The partial reduction in size of the tumor in the sitting position may aid the clinical diagnosis. The X ray diagnosis is highly enhanced by the direct injection of contrast medium into the tumor, leading to the visualization of the outflow in the miningeal veins, while external carotid angiography is normal. Step by step coagulation of the verious supply coming through the skull allows a non-bleeding surgical excision.
The authors report a fairly typical clinico-pathological case of acute hemorragic leucoencephalitis (A.H.L.E.). Both clinical and histological features appeared particularly acute. At autopsy a visceral evolving tuberculosis was diagnosed. Such an etiological circumstance has not been, apparently, reported in cases of A.H.L.E. published as such. Three other etiological circumstances were noticed: pyuria, treatment by cephalotin and treatment by gentamycine. The physiopathogenesis of A.H.L.E. remains obscure and will not be clarified before detailed immunological studies can be performed. But the disease is rare and brisk and diagnosis usually post-mortem.
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The authors report the first two French cases of primary rabdomyosarcoma of the central nervous system. They then review the literature on muscle tumours or primary myosarcomas of the nervous system of which 29 cases have now been published. From the point of view of morphology and classification, one should distinguish firstly, purely mesenchymatous tumours (rhabdomyosarcomas, leiosarcoma, mesenchymoma) and secondly, composite tumours of connective tissue or spino-epithelial tumours (medullomyoblastomas, neuromyoblastomas, gliomyosarcomas). Their histogenesis occurs from the ectomesenchyme of the neural crests.
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The authors report the pathological and clinical findings of a dissecting aneursym of the basilar artery in a 32 year old man up till then in good health, who died two months later. The pathological study showed bilateral lesions of osteo-malacia in the neighborhood of the pons due to hemo-dissection affecting the right vertebral artery just before it enters the dura-mater, then its intra-cranial portion, the basilar trunk and the first part of the right posterior cerebral artery. The originality of this case resides in the finding of segmental stenosing panarteritis of the same right vertebral artery in the segment which precedes dissection. On this occasion, the authors carried out a review of the literature and considered a few comments on the etiology, the pathology, clinical findings and classification of this unusual disease.
The authors describe the case of a patient of 22 with hypertension and livedo reticularis who, after presenting with a left brachial monoparesis became progressively demented over a period of five years and died at the age of 27 from a cerebro-meningeal haemorrhage. Angiographically, diffuse distal obliteration of the cerebral arteries was seen with deep networks of the moyamoya type involving the lenticulostriate arteries; similar changes were present in the upper left limb. Histopathological investigations showed obliterative thromboangiitis affecting not only the cerebrum, the brain stem and the cerebellum, but also the viscera. Analysis of this case and consideration of the theoretical possibilities leads the authors to urge that thromboangiosis together with its cerebral manifestations should be considered a nosological entity.
A case of primary rhabdomyosarcoma in the brain stem is described in an 8 year old girl. The clinical data showed a right side hemiplegia then a total paralysis of the left sixth cranial nerve and a paraplegia which became lethal in 3 months. The necrospy revealed a tumoral nodule in the left medulla oblongata and pons with diffuse subarachnoidal extension from the cranial nerves to the cauda equine roots. Histologically the tumor appeared to be polymorph with numerous rhabdomyoblasts which had a clear cross striation and which were sometimes less differentiated without any neuronal or glial elements. Perivascular tumoral cells and blood vessels were closely linked, the Virchow-Robin spaces were clearly involved. The electron microscopic study confirmed that the less differentiated cells were of a rhabdomyoblastic nature. A review of the litterature indicates that these malignant neoplasias are highly exceptional, and can be classified within the group of primary tumors of the neuraxis with muscular elements. The histogenetic origin of these tumors appears to be the ectomesenchyme of neural crests.
The authors report 4 cases of the morbid familial association revealing itself late in life (average age 37.7 years) including multiple basal cell carcinomas of the face and neurological and psychiatric symptoms, the most complete examples of which were severe, including mixed cerebello-spinal ataxia, involvement of the anterior horns of the spinal cord, a pyramidal syndrome and extra-pyramidal syndrome, abolition of the osteo-tendinous reflexes, dementia, paralysis of certain cranial nerves. These are associated constantly with increased glucose concentration in the cerebro-spinal fluid and dilatation of the cerebral ventricles. The course is unusual. The skin signs always occur first. There is a definite parallel between the severity of the skin involvement and that of the central nervous system. A neuropathological study of one case (case 2) showed lesions of degenerative type resembling spino-cerebellar degeneration of Menzel type with supramedullary diffusion to the locus niger, locus coeruleus, cranial nerves and thalamus. From these clinical and pathological findings, the authors noted an original pathological and clinical entity and consider certain diagnostic and pathological problems. The precise relationship between the skin and nervous lesions is unknown, but may be due to abiotrophic processes. Concerning the classification, this disease should not be included among the phacomatoses for there is no biastema tendency, and should be included among the more general group of neuro-cutaneous dystrophies or genoneurodermatoses.
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Two complications of Paget's disease are presented : they include an osteogenic sarcoma and a giant-cell tumour (GCT) of the lumbar spine with a radiculo-medullary compression syndrome. The rarity of sarcomatous degeneration of the spine affected by Paget's disease is emphasized and also the unusual character of the spinal GCT which develop in the presence of Paget's disease. As in the present case, it is often difficult to confirm the benign or malignant character of a GCT and the principal criteria of this classification are discussed.
The authors report an observation in which four siblings were affected by both multiple cutaneous epitheliomatosis and complex but relatively stereotyped neurological disorders. Clinically, the main syndrome was cerebello-spinal ataxia with involvement of the anterior horns of the spinal cord with less marked pyramidal and extra-pyramidal features. Neuropathological examination of one of the cases revealed lesions of essentially cerebello-spinal degeneration suggestive of Menzel's disease. The possible connection between the neural and cutaneous lesions is discussed. All the various etiological categories possible have been ruled out; not one being entirely satisfactory, except for the very broad category of genetic neuro-dermatoses.
The authors report an angiographic observation, where a stenosis of the left middle cerebral artery, with Moya-Moya networks, is described. The anatomical study has shown an atresy of the middle cerebral artery, and has confirmed the hypothesis of a supplying role played by the Moya networks. Most of their anatomical findings are in agreement with a malformative aspect, which is speculated by the authors to be related to the failure, at the embryon level, of a good development of the middle cerebral artery; in this way, Moya might represent the remaining features of primitive plexiform networks. From this particular anatomical observation, the authors discuss some nosological problems and propose the hypothesis of several groups, which may be related to the moment when the stenosis is suspected to occur.