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Biomedical subjects

J J Kepes

Publications and source records attributed to J J Kepes.

At least 55 records · Page 3Linked to original sources
PubMed1990

Mesolimbocortical dementia: clinico-pathological studies on two cases.

The clinicopathological findings are presented of two cases of mesolimbocortical dementia. Both cases were characterised by late onset slowly progressive personality changes and progressive intellectual deterioration without clinical Parkinsonism. Neuropathological findings revealed non-specific neuronal degeneration, Holzer and GFAP positive gliosis primarily affecting the limbic system, caudate, thalamus and substantia nigra. The pathological findings coincide with the distribution of the non-striatal dopaminergic pathways and suggest an intrinsic involvement of these pathways to account for the clinical and pathological manifestations.

Aged↗
PubMed1990

HIV-related neuromuscular syndrome simulating motor neuron disease.

A young homosexual man presented with slowly progressive weakness and diffuse fasciculations. Muscle biopsy was compatible with clinical diagnosis of motor neuron disease. The patient died from opportunistic infections related to acquired immunodeficiency syndrome. Autopsy revealed evidence of myelopathy, patchy fiber loss from anterior nerve roots, marked demyelination within intramuscular twigs of peripheral nerves, and primary myopathic features superimposed on advanced neurogenic atrophy of the skeletal muscles.

Adult↗
PubMed1990

Ganglioglioma of the optic chiasm: case report and review of the literature.

We describe a case of ganglioglioma of the optic chiasm and tract in a 33-year-old man. Review of the literature discloses only seven cases of tumors of the anterior visual pathway that meet the histological criteria for such neoplasms. A detailed histological description of our case and a review of the literature is provided.

Adult↗
PubMed1989

Dominantly inherited dementia and parkinsonism, with non-Alzheimer amyloid plaques: a new neurogenetic disorder.

A family is described in which a dominant form of inheritance, probably autosomal dominant, expresses severe dementia and parkinsonism as the major clinical features. Neuropathological correlates in two autopsied members of this family consisted of extracellular hyaline eosinophilic, congophilic amyloid plaques in decreasing order of frequency in the cerebral cortex, basal ganglia, thalamus, and substantia nigra, and atrophy and gliosis of the basal ganglia and substantia nigra. The extracellular plaques did not stain with antibody raised against the prion protein nor with two separate anti-amyloid A4 antibodies. The combination of dominantly inherited dementia with parkinsonism and extracellular plaques in this distribution that are amyloid and prion protein antibody negative has not been previously reported and thus may represent a new neurological genetic disorder.

Alzheimer Disease↗
PubMed1989

Histopathological features of recurrent pleomorphic xanthoastrocytomas: further corroboration of the glial nature of this neoplasm. A study of 3 cases.

Pleomorphic xanthoastrocytoma (PXA), a tumor most often presenting superficially over the cerebral hemisphere of young subjects, has certain morphological similarities to fibrous histiocytoma (or fibrous xanthoma) of the meninges and brain, namely the occurrence of lipid-laden neoplastic cells and, frequently, a dense reticulin fiber network. The detection of glial fibrillary acidic (GFA) protein in the tumor cells helped to establish its astrocytic derivation, but it has been advanced that, in spite of this agreed observation, the tumor should still be regarded as a fibrous xanthoma of meningeal origin. Although many patients have a long symptom-free postoperative survival, local recurrences at varying intervals after surgery have been noted in some instances. Weldon-Linne et al. first reported that such a recurrence had the morphology of a small-cell glioblastoma. We are reporting three further examples of locally recurrent neoplasms in patients whose original meningocerebral tumors had the typical features of PXA; the recurrences (developing 7 months, 7 years and 15 years, respectively, after surgery) were small-cell glioblastomas. The rich reticulin network present in the initial tumor was mostly lost in the recurrences. This anaplastic evolution further confirms the astrocytic nature of the PXA.

Adolescent↗
PubMed1989

Cerebellar astrocytomas in elderly patients with very long preoperative histories: report of three cases.

Three patients, ages 69, 67, and 74 years, respectively, underwent surgical removal of cystic cerebellar astrocytomas. All three had past histories pointing to the existence of a cerebellar lesion for many decades prior to surgery: Patient 1 had had nystagmus on lateral gaze on the side of the tumor since early childhood; Patient 2 had had sensorineural hearing loss on the side of her neoplasm for 38 years preceding the operation; and Patient 3 was diagnosed as having a brain tumor 51 years before the operation. (He has been blind because of pressure hydrocephalus for half a century, but otherwise managed to live a productive farming and family life until he sustained a head injury in a car accident, which forced him to undergo removal of his cerebellar tumor.) The neoplasms in all three instances were found by histological examination to be low-grade astrocytomas. These cases indicate that low-grade cerebellar astrocytomas, which are well known for their characteristically long postoperative courses, may at times manifest a slow growth potential with an exceptionally long preoperative course.

Aged↗
PubMed1988

"Chordoid" meningeal tumors in young individuals with peritumoral lymphoplasmacellular infiltrates causing systemic manifestations of the Castleman syndrome. A report of seven cases.

Seven young patients ranging in age from 8 to 19 years had surgically removed meningeal neoplasms with a peculiar myxoid-chordoid pattern. The tumors were surrounded by massive polyclonal lymphoplasmacellular infiltrates with follicles and germinal centers. The patients preoperatively manifested iron-resistant hypochromic microcytic anemia, and one of them had dysgammaglobulinemia and stunted growth. After the masses were removed, the blood picture of the patients normalized, and the adolescent with retarded somatic development resumed normal growth, but two patients developed local recurrence with identical histology and again became anemic. It appears that the peritumoral lymphoplasmacellular infiltrates, which in these instances may be regarded as reactive rather than primary cell proliferations, nevertheless brought about the type of systemic manifestations known as the Castleman syndrome, characteristically seen in patients with angiofollicular lymphoid hyperplasia (hamartoma) of soft tissues, or Castleman's tumor. In one of our patients the inflammatory infiltrate extended far into the surroundings of the meningioma, resulting in the erroneous initial diagnosis of "encephalitis" from a needle biopsy of the brain near the tumor.

Adolescent↗
PubMed1988

Glial fibrillary acidic protein in chondrocytes of elastic cartilage in the human epiglottis: an immunohistochemical study with polyvalent and monoclonal antibodies.

Glial fibrillary acidic protein (GFAP), an intermediate filament, was first discovered in the cytoplasm of reactive astroglia and to this date is regarded as a rather characteristic component of both non-neoplastic and neoplastic astrocytes. It has, however, been found in other glial elements also, as well as in a few types of tissues outside the central nervous system. Chondrocytes in hyaline and fibrocartilage do not express GFAP, but in the elastic cartilage of the human epiglottis we found GFAP to be regularly present when tested with either polyclonal or monoclonal antibodies. While most types of cartilage in humans are believed to be of mesenchymal origin, embryologic relationship between epiglottal cartilage and the neural crest may play a role in the curious phenomenon of the regular presence of GFAP in the chondrocytes of the epiglottis.

Adult↗
PubMed1988

Patterns of epithelial metaplasia in malignant gliomas. I. Papillary formations mimicking medulloepithelioma.

Adenoid-like formations resembling ducts and glands or forming a cribriform pattern have previously been described in malignant gliomas, resulting in some cases in a confusion with metastatic adenocarcinoma. The interpretation of these structures as being composed of anaplastic glial cells rests partly on the presence of transitions to more differentiated neoplastic astrocytes and partly on the positivity of some of these cells for glial fibrillary acidic protein. In this report two cases are presented in which the adenoid pattern was associated with papillary formations mimicking the arrangement of a medulloepithelioma. These structures represent a form of aberrant neoplastic differentiation in a malignant glioma rather than the expression of an embryonal neuroepithelial neoplasm.

Adult↗
PubMed1988

Patterns of epithelial metaplasia in malignant gliomas. II. Squamous differentiation of epithelial-like formations in gliosarcomas and glioblastomas.

Six cases are reported (four gliosarcomas and two glioblastomas) in which the epithelial-like areas of glial anaplasia showed focal squamous cell differentiation, characterized by the development of epithelial whorls, keratin pearls and immunopositivity for cytokeratin. The expression of glial fibrillary acidic protein and the development of squamous metaplasia usually were mutually exclusive. Autopsy findings in two patients and clinical work-up in five failed to disclose a primary extraneural malignancy. It is suggested that squamous differentiation may represent an extreme form of epithelial metaplasia in a malignant glioma. This possibility should be kept in mind in the diagnostic evaluation of such cases, especially in view of the current emphasis on the immunomorphologic demonstration of intermediate filament tumor markers.

Adult↗
PubMed1988

Segmental duplication of the basilar artery with thrombosis.

Duplication or fenestration of the basilar artery, a result of an embryologic malformation, has an incidence of up to 5.3% in the general population. The most common complication of this anomaly is the formation of aneurysms. Thrombosis of a partially duplicated basilar artery developed in a 43-year-old man who complained of visual disturbances followed by seizures and coma, and who eventually died. Autopsy showed a partially organized thrombus occluding both halves of a duplicated portion of the basilar artery, old infarcts in the calcarine cortices, and a recent large infarct in the basis pontis. There was only minimal atherosclerosis of other intracranial arteries, including the vertebral arteries. Hemodynamic disturbances and turbulent blood flow at the site of fenestration may be the cause of the thrombosis that occurred in this artery.

Adult↗
PubMed1988

Multiple sulfatase deficiency: bridge between neuronal storage diseases and leukodystrophies.

A fatal case of multiple sulfatase deficiency in a 10-year-old girl is reported. In this rare disease, which is inherited as an autosomal recessive, features of metachromatic leukodystrophy and of mucopolysaccharidoses occur together. The white matter suffers progressive destruction with sulfatides accumulating in macrophages; these stain metachromatically as golden brown granules with acetic acid thionin stain. Cortical and subcortical neurons are distended by lipids and mucopolysaccharides, and fibrous thickening of the leptomeninges leads to severe obstructive hydrocephalus. This entity can be regarded as a bridge between leukodystrophies and neuronal storage diseases both conceptually and in its morphological manifestations.

Brain↗
PubMed1988

Intracranial malignant fibrous histiocytoma with abscess-like tumor necrosis. Case report.

A case of a malignant fibrous histiocytoma (MFH) in the right temporal lobe of a 75-year-old man is reported. The tumor involved the brain and the overlying dura as one continuous mass and had undergone extensive liquefaction necrosis of its center that had a gross appearance similar to that of an abscess. The preoperative computerized tomography scan with injection of contrast material showed the lesion to have central and peripheral enhancement suggestive of metastatic tumor, glioblastoma multiforme, or abscess. Microscopically, the tumor showed the typical features of a MFH with marked acute inflammation in the areas of necrosis. No extracranial sources for this tumor were found clinically. This is the 15th reported case of MFH involving the central nervous system as a primary neoplasm and the first case with gross pathological and radiological features resembling in many respects those of an abscess.

Aged↗
PubMed1987

Astrocytomas: old and newly recognized variants, their spectrum of morphology and antigen expression.

The relationship between the morphology of astrocytomas and their prognosis is complex, with the localization of the tumour, the predominant cell type and the degree of anaplasia all playing an important part in determining the patient's future. Since many astrocytomas have a diversity of patterns, small needle biopsies taken from just one or a few areas may fail to elucidate the principal features of a given tumour. In addition to the astrocytoma subtypes listed in the WHO's International Histological Classification of brain tumours, new entities have been observed in the last few years. These include lipidized forms, such as the relatively benign pleomorphic xanthoastrocytoma and the highly malignant lipidized glioblastoma. Neoplastic astrocytes are capable of forming basal laminae and of phagocytosis, and often contain alpha-1-antitrypsin, features that may lead to confusing them with histiocytes. They may be arranged in a whorled pattern imitating meningiomas, their myxoid intercellular stroma may assume the morphology of cartilage and closely packed tumour cells in "epithelioid" astrocytomas come close to imitate metastatic carcinoma. Some astrocytomas contain cells indistinguishable from those of granular cell tumours of other tissues. The presence of reactive lymphocytes in astrocytomas and reactive astrocytes in malignant lymphomas can be the source of confusion between those two kinds of neoplasms.

Animals↗