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Biomedical subjects

O Vuia

Publications and source records attributed to O Vuia.

At least 19 recordsLinked to original sources
PubMed1985

Intracranial sarcoma in childhood.

Three cases of intracranial sarcomas in children are presented. The children were 1 month, 8 months and 7.5 years old. In two cases the tumor was in the cerebral hemisphere and in one case (the 7.5-year-old child) in the cerebellum. The histopathological diagnosis was undifferentiated sarcoma and spindle cell sarcoma in the cases with tumor in the cerebral hemisphere and arachnoidal sarcoma of the cerebellum. There was one postoperative death. The two children subjected to postoperative X-ray therapy and cytostatic therapy survived 1.75 years and 7 months, respectively.

Brain Neoplasms↗
PubMed1983

[Polyneuropathy caused by disulfiram poisoning].

Disulfiram is known to produce toxic encephalopathy and peripheral neuropathy. The case of a 37-year-old alcoholic who attempted to commit suicide by taking 22.5 g disulfiram is described. During the first 6 days after the intoxication he was stuporous and had cerebellar ataxia and dysarthric speech. Then he became comatose, and as he recovered from coma, he showed peripheral neuropathy including diplegia faciei and severe tetraparesis. Denervation potentials were detected in both facial muscles and distal muscles of the upper and lower limbs, while conduction velocity was normal. Axonal degeneration was verified by sural nerve biopsy. In addition neurofilamentous axonopathy was documented. The recovery from his paresis lasted 2 years.

Adult↗
PubMed1980

Clinical and Endocrinological findings in ectopic pinealoma and spongioblastoma of the hypothalamus.

Cystic spongioblastoma and ectopic pinealoma occurring simultaneously were found in a 16-year-old male patient, and produced destruction of the hypothalamus. The clinical course extended for over four years. The clinical picture was characterized by a defect of osmo- and thermoregulation and by defective function of diencephalic nuclei and the sympathetic nervous system. The releasing factors for ACTH, TSH, LH, and FSH were lacking and produced corresponding disturbances of pituitary function. Because of the lack of hypothalamic inhibiting factors the prolactin level was increased, and the HGH level was stimulated by arginine loading and inhibited in the glucose test. The intact neurones in the ventromedial nucleus of the hypothalamus could be seen on microscopical examination. Clinical and endrocrinological findings were more suggestive of the diagnosis than the radiological ones. Computerized tomography showed multiple "tumour" localizations without any displacement signs. The occurrence of ectopic pinealoma and spongioblastoma in the same case would suggest, from the pathological point of view, a common dysontogenetic origin developing from the local elements of the nervous tissue.

Adolescent↗
PubMed1980

Embryonic carcinosarcoma (mixed tumour) of the pineal gland.

The present paper reports on a pineal gland tumour in an 8-year-old male child. Microscopic examination revealed a carcinomatous and a sarcomatous component. The aspect was characteristic of mixed embryonic carcino-sarcoma described in germ cell layer tumours of the gonads and kidney (Wilms' tumour). Ultrastructurally, the mixed character of the tumour was confirmed and also more differentiated elements were found differing from the appearances described in germ cell layer tumours. On the basis of the morphological examination it is concluded that at least in a proportion of the malignant mixed tumours of the pineal gland, their origin is to be found in the local neural elements and not the extraneural ones.

Brain Neoplasms↗
PubMed1978

Paraproteinosis and amyloidosis of the cerebral vessels and senile plaques.

A case is reported of progressive dementia and a terminal picture of generalized tetaniform contractures. The relationship of the generalized tetaniform contractures to the stiff-man syndrome is discussed. Morphologically, diffuse amyloid deposition was found in the pial and cortical vessels, accompanied by amyloid deposition in the senile plaques in the cortical and cerebellar cortex. Apart from the typical staining and ultrastructural aspects of amyloid, a deposition of material was observed, corresponding in optical and electron microscopy to a paraprotein. This case demonstrated not only the relationship between the deposition of amyloid and the formation of senile plaques, but also sustains the direct connection between amyloid in senile plaques and the paraprotein substances deriving from the blood. The probable relationship between the unusual deposition of paraproteins in the vessels and nervous system and the treatment with immunoglobulins is discussed.

Amyloidosis↗
PubMed1978

Malignant haemangioblastoma (haemangiosarcoma) of the meninges.

The present paper is a report of two cases of malignant haemangioblastoma (haemangiosarcoma) of the meninges. Evidence was found of a tumour made up of vascular proliferation and pleomorphic cells. In case 1 the meningeal tumour had the ultrastructural characteristic of a malignant haemangioendothelioma. In this patient, a spinal tumour found a year later presented the features of a mixed malignant haemangioblastoma. The origin of the tumour in case 2 is the capillary pericyte, the lesion being a malignant haemangiopericytoma. We discuss ultrastucture and properties of the pericyte, its relationship to the cerebral reticulohistiocytic system, and the relationship of malignant haemangiopericytoma with the tumours considered to represent cerebral reticulosis and reticulum cell sarcomas.

Adult↗
PubMed1978

Congenital Pelizaeus-Merzbacher disease (Seitelberger type), malformation and cystic degeneration of the central nervous system.

The present paper reports on the case of a neonate child with multiple malformations affecting osteogenesis and the central nervous system (microcephaly, pachygyria). Morphologically, myelin staining supplied evidence of the total lack of myelin sheaths (myelin aplasia). The cerebellar cortex was malformed and altered by cystic degeneration. Electron microscopy confirmed the aplasia of the myelin sheaths and revealed the presence of concentric multilamellar formations in the glial cells (myelination glia), and in areas of cystic degeneration. The morphologic aspects clearly showed the relationship of this congenital aplasia of the myelin sheaths with the congenital Pelizaeus-Merzbacher disease (Seitelberger type). The cystic degeneration differs from the spongy degeneration of the brain (van Bogaert-Bertrand disease) and should be considered a result of maldeveloped nervous tissue.

Cerebellum↗
PubMed1977

Leioblastoma, developing as an hour-glass tumour of the spinal canal.

The present paper describes a tumour that developed like an hour-glass tumour, both intraspinally at lumbar level and retroperitoneally. At operation the tumour was diagnosed as a malignant neurinoma. The structure of the tumour, studied by light and electron microscopy, as well as the subsequent evolution of the case, showed it to be a leiomyoblastoma with a retroperitoneal origin, that gradually infiltrated the nerve root (L1) up to the dura mater, epidural and subdural space, where it took on the appearance of a primary tumour of these formations. The author discusses the existence of the so-called primary intracranial rhabdomyosarcoma and its relationship with medullomyoblastoma.

Female↗
PubMed1977

Congenital spongy degeneration of the brain (van Bogaert - Bertrand) associated with micrencephaly and ponto - cerebellar atrophy (contributions to the pathology of glial dystrophy of intrauterin origin).

The present paper reports on two twin brothers who presented clinically at birth a syndrome characterized by progressive development of muscular hypertonia, opisthotonus, micrencephaly, amaurosis and short, localized clonic seizures. Both children died soon after one year of age. The anatomic examination performed in one case revealed a spongy degeneration of the brain of van Bogaert-Bertrand type. Associated to the cerebral degenerative syndrome was a severe malformative syndrome characterized by micrencephaly, internal hydrocephalus and pontocerebellar atrophy. The ultrastructure of the cerebellar cortex showed degenerative phenomena in the Purkinje and glial cells, demonstrating the evolutive character of the ponto-cerebellar lesions. The degenerative process consisted of the accumulation of lamellar bodies within the mitochondria and free in the cellular cytoplasm with tendency to form inclusions of the multilamellar or finger-print type. Association of the degenerative with the malformative process is not considered to be accidental but based upon the same fundamental pathologic process: glial dystrophy. It may be assumed that the pathologic defect, which as a rule produces syndromes that appear after birth (spongy degeneration of the brain, ponto-cerebellar atrophy), may in some instances act at an early date in intrauterine life, the glial dystrophy that appears so early producing both the degenerative and the malformative process.

Atrophy↗
PubMed1977

The benign form of multiple sclerosis. Anatomo-clinical aspects.

The present paper is a study of three cases presenting the pathological aspect of chronic, non-evolutive multiple sclerosis, corresponding to a clinical picture of benign multiple sclerosis. The first case was discovered at autopsy, the second patient had two subclinical attacks of multiple sclerosis, with a twenty-year evolution (forme fruste), and in the third case the course of the chronic multiple sclerosis ran parallel to that of a cerebral and visceral arteriopathy. Anatomic study of the demyelinating lesions revealed their non-evolutive aspect and the presence within the plaques of lesions of the walls of small vessels with the perivascular adventitial fibrosis. The vascular changes appear to play a primordial role in the lack of evolution of the demyelinating areas, manifested clinically by a stationary, benign picture.

Brain↗
PubMed1977

Morphologic aspects of the neurofibrosarcoma (neurogenic sarcoma).

Two cases of malignant tumours of the nerve sheaths are reported. In the first patient, who had von Recklinghausen's disease, light- and electron-microscopic investigations showed an undifferentiated mesenchymal tumour of the radial nerve with the property of producing collagen fibres, an aspect characteristic of sarcoma of the fibroblastic type. In the second case a pleomorphic cell sarcoma of the cranial nerves of the left cerebellopontine angle was present. The morphologic aspects of these tumours show them to be primary mesenchymal tumours of the nerve (neurofibrosarcoma, neurogenic sarcoma) originating in the fibroblastic-like elements of the nerve sheaths. The clinical and nosologic framework of this type of malignant tumour of the nerve sheaths is discussed.

Adult↗
PubMed1977

Neuroaxonal dystrophy, a juvenile-adult form.

This paper is an anatomoclinical study of a neuroaxonal dystrophy case, starting in childhood and evolving up to maturity, which may be considered as a juvenile-adult form of this syndrome. Several of the morphologic emphasized aspects suggest that this juvenile-adult form is a primary affection of the axon and therefore, may be considered as a separate entity, differing from the Hallervorden-Spatz disease.

Adult↗
PubMed1976

Congenital intracerebral epithelial cysts.

The problem of intracerebral congenital epithelial cysts is discussed with reference to five clinical cases. On the basis of their morphological structure the author differentiates the intracerebral cyst with a ciliated, columnar epithelium from the ependymal cyst proper (more frequently located in the lateral recess of the medulla oblongata) and the posterior cerebellar cyst of meningeal arachnoid origin. The cyst with a ciliated columnar epithelium appears to be an ectopic intracerebral tissue, whose neuroepithelial or endodermic origin still remains to be demonstrated. This cyst has been commonly described as a colloid (epithelial) cyst of the 3rd ventricle but may also be localized at other points in the central nervous system. One of the cases studied was an epithelial cyst in the frontal lobe, without any connexion with the ventricle, and evolving as a cerebral tumour.

Adult↗
PubMed1976

Progressive multifocal leucoencephalopathy and Rosenthal fibres.

The present paper is the study of a case of progressive multifocal leucoencephalopathy that appeared in the course of a malignant reticulosis accompanied by paraproteinosis. Due to the lesions in the brain stem and cerebellum, the case evolved clinically as a pure cerebellar form. The particularity of the morphologic aspect, not as yet described, was the abundant presence of Rosenthal fibres in the older demyelination foci. These fibres exhibited a typical structure both in the electron and optic microscope. Emphasis is laid on the connection between the formation of Rosenthal fibres and blastic cellular transformation of the glial system.

Aged↗
PubMed1976

[Morphological aspects (optical and ultrastructural) of hypertrophy of the olivary bulb].

The present paper is an anatomical study of 3 cases which evolved with hypertrophy of the inferior olives: a case of zoster encephalitis, a multifocal leucoencephalopathy and a post-traumatic encephalopathy. In all three cases a relationship was demonstrated between the dentate nucleus and dento-olivary pathway - a relation of topical order in some instances - and the hypertrophic lesion of the inferior olive. Examination in the electron microscope revealed in the hypertrophic olivary cell not only dystrophic lesions but also progressive intracellular phenomena, such as maintenance of the Nissl bodies, multiplication of the mitochondria and especially marked proliferation of the neuro-filaments. This shows that, at least in some of the evolutive phases, hypertrophy of the inferior olives is based upon a real hypertrophy of the olivary cell. In addition, lesions of the glial system of the hypertrophic inferior olives were noted with the presence of osmiophil degeneration whose morphology clearly differed from that of Rosenthal's fibres.

Adult↗
PubMed1975

Primary cerebral reticulosis and plasma cell diffenentiation.

Cerebral proliferative and tumour forming reticulosis are primary pathologic processes of the brain originating in the perivascular adventitial cell. In these conditions this cell can transform into a histiocyte or microglial cell with characters of a macrophage. A reactive cell with endoplasmic reticulum, basement membrane and fibril forming properties, is also present (fibril forming reticular cell). The malignant tumours of the perivascular spaces (cerebral reticulosarcoma) are characterized by the presence of the dedifferentiated cells originating from the intraadventitial cell. Under both proliferative and tumour forming conditions this cell may transform into a cell rich in endoplasmic reticulum, sometimes charged with Russell bodies and corresponds morphologically to the protein-forming plasma cell. The properties of the intracerebral periadventitial cells are identical with those of the reticulo-histiocytic system described by ASCHOFF. As its structure and the elements into which it differentiats sharply differ from those of the cells belonging to the lymphocyte series the term malignant lymphoma of the brain given to these processes does not correspond to the fundamental characters of these pathological process.

Basement Membrane↗