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J J Kepes

Publications and source records attributed to J J Kepes.

At least 91 records · Page 5Linked to original sources
PubMed1982

Hypertrophic mononeuropathy. A report of two cases and review of the literature.

Two cases of hypertrophic mononeuropathy were studied. This entity is characterized grossly by focal enlargement of a single peripheral nerve, and microscopically by loss of nerve fibers, fibrosis, and formation of numerous onion bulbs. Our cases were diagnosed by exploratory surgery, biopsy, and light microscopy. Electron microscopic evaluation was performed in one case. Recognition of this entity by the surgical pathologist is important, so that it can be distinguished from generalized forms of onion bulb neuropathies, most of which give a much less favorable long-term prognosis. Hypertrophic mononeuropathy has usually resulted in irreversible local neurologic deficit but, unlike the polyneuropathies, in all the previously reported cases (as well as in our two cases to date) it has remained a localized process.

Adolescent↗
PubMed1981

Malignant gliomas with heavily lipidized (foamy) tumor cells: a report of three cases with immunoperoxidase study.

Three cases of heavily lipidized malignant glioma are reported. Large amounts of intracellular lipid gave the tumor cells a vacuolated, foamy appearance that generally obscured their glial nature. The latter was, however, confirmed by the demonstration of glial fibrillary acidic protein (GFAP) by immunoperoxidase. These tumors, which were situated deeply in the brain and contained areas of necrosis, must be distinguished from the relatively benign pleomorphic meningocerebral xanthoastrocytomas, which are characteristically superficial in their location and lack necrotic foci. The malignant nature of the presently described tumors is shown by the death of all three patients within one year of surgery. Thus, in the biologic sense these tumors represent glioblastomas; however, their morphologic peculiarities in the form of massive lipidization of tumor cells, apparently not caused by necrobiosis, set them aside as a histologic subgroup.

Adult↗
PubMed1980

Cockayne syndrome: an atypical case.

We report a case of Cockayne syndrome with several atypical features. The patient displayed many of the typical features, including cachectic bird-headed dwarfism, photodermatitis, normal pressure hydrocephalus, and extraphyramidal and pyramidal tract signs. However, she also displayed the unusual and previously unreported features of late age at onset, relative stability until age 19, and fertility, with a successful pregnancy. Brain biopsy showed hypomyelination, no active demyelination, and fibrillary gliosis.

Adult↗
PubMed1980

Suggested pathological basis of "redundant nerve root syndrome" of the cauda equina.

A clinical entity described as "redundant nerve root syndrome" of the cauda equina is reviewed. Eighteen reported cases with typical gross features are analyzed. Considerable speculation has existed regarding the pathogenesis of this syndrome, chiefly because the involved nerve root was not biopsied in the majority of instances. In one reported case, when the redundant root was examined histologically, a plexiform neurofibroma was found to be the cause of the thickening and redundancy of the nerve. In the case presented here, the enlarged nerve roots were part of a malignant plexiform neurofibroma with a short clinical course, and the tumor led to the death of the patient. It is suggested that biopsy of the involved nerve root is crucial in establishing the definitive diagnosis.

Adult↗
PubMed1980

Cerebellar astrocytoma invading the musculature and soft tissues of the neck. Case report.

A case of cerebellar astrocytoma with unusual growth pattern in an 8-year-old boy is reported. The tumor had some histological hallmarks of malignancy (focally high cellularity, mitotic figures, necroses, and calcifications) and, 17 months after subtotal removal, recurred as a nuchal mass in the area of the incision, infiltrating first the skeletal musculature and later other soft tissues of the neck, including lymph nodes. Although cerebellar astrocytomas may recur locally and some may even seed through the cerebrospinal fluid, this is the first reported case of a tumor of this group to infiltrate skeletal muscle and other soft tissues of the neck. The patient underwent five operations to relieve the cervical organs from pressure.

Astrocytoma↗
PubMed1979

Astrocytes in hemangioblastomas of the central nervous system and their relationship to stromal cells.

Thirteen cases of CNS hemangioblastoma were examined with the immunoperoxidase technique for glial fibrillary acidic protein (GFAP) to determine if there were astrocytic elements among the "stromal cells" of these tumors. In six cases, includinng two leptomeningeal hemangioblastomas, none of the stromal cells were positive on GFAP stain. Seven cases, however, showed variable presence of Gfap positive cells, including clusters of heavily lipidized cells deep within cerebellar hemangioblastomas. These GFAP positive cells were indestinguishable by other stains from interstitial or stromal cells. Thus, it appears that in at least some hemangioblastomas of the CNS parenchyma, a few or many "stromal cells" are lipidized astrocytes. All stromal cells, however, cannot be of astrocytic origin, as proposed by Jakobiec et al. (1976), in view of our six cases where no GFAP-positive cells were found in the tumors. It is suggested that cells identified as "stromal cells" of hemangioblastomas on light microscopy are a heterogeneous group of cells including astroyctic as well as other elements and that they resemble each other on ordinary stains because of the "equalizing effect" of cell lipidization.

Adult↗
PubMed1979

Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases.

Twelve cases of a distinctive form of supratentorial astrocytoma occurring in young subjects (ages 7 to 25) are reported. The tumors were superficial and involved the leptomeninges extensively. The tumor cells display marked pleomorphism, including bizarre giant cells and a number of mitotic figures, but no necrosis. Many contain large amounts of lipid in their cytoplasm and are surrounded by reticulin fibers, thus simulating a mesenchymal tumor. For these reasons, some examples of this tumor have been previously interpreted to represent meningocerebral fibrous xanthomas. Immunoperoxidase technique performed in nine of the twelve cases has, however, established the presence of glial fibrillary acidic protein in the tumor cells, which are therefore considered to be astrocytic. By electron microscopy many tumor cells are surrounded by basal laminae, accounting for the abundant reticulin network demonstrable in silver preparations. Since subpial astrocytes are known to be partly covered by a basal lamina, it is likely that they are the cells of origin for this neoplasm. In contrast to its pleomorphic cytology, the biological behavior of this tumor appears to be relatively favorable, and long survival times (up to 25 years) have been recorded in some cases. (These tumors are distinct from intracranial fibrous xanthomas of mesenchymal derivation. Cells of the latter are negative on GFAP stain.)

Adolescent↗
PubMed1979

Cystic lesions associated with intracranial meningiomas.

Three unusual cases of large, peritumoral cystic lesions associated with intracranial meningiomas are reported. In each case, the cyst caused difficulty in the interpretation of the computed tomogram when the latter was considered as a diagnostic test by itself, but the composite information obtained from the clinical history, cerebral angiography, and a radionuclide brain scan led to the correct preoperative diagnosis. The cyst was extrinsic to the tumor and contained xanthochromic fluid with a high protein content. The cyst wall consisted of brain parenchyma that showed glial cell proliferation (confirmed by the presence of glial fibrillary acidic protein by immunoperoxidase staining).

Aged↗
PubMed1979

Extraspinal ependymomas. Report of three cases.

Spinal ependymomas may rarely arise from heterotopic ependymal cell clusters and thus occur in an extraspinal location. Presentation of three cases and a review of the literature reveal that these tumors have characteristic radiographic and clinical features. They occur mainly in patients in the third decade of life, and present either in the soft tissue posterior to the sacrum or in the pelvis. In the case of posterior tumors, the patient exhibits a mass which is usually mistaken for a pilonidal cyst. Patients whose tumor is pelvic in location present with sphincter disturbances or dysfunction of the sacral nerve roots. Conventional and computerized tomographic studies will reveal erosion of the sacrum. Myelography will demonstrate an extradural mass indenting the thecal sac from below. The protein in the cerebrospinal fluid will be normal. A combined posterior and anterior approach with the goal of complete tumor removal is the procedure of choice. If this is not feasible, then radiation therapy should be employed. Because of the increased incidence of systemic metastases, the average postoperative survival is approximately 10 years.

Adolescent↗
PubMed1978

Transitional cell tumor of the pituitary gland developing from a Rathke's cleft cyst.

A tumor of the pituitary gland is reported which developed from the wall of a Rathke's cleft cyst. This was an incidental autopsy finding in a 79-year-old diabetic woman. The cyst wall was lined by ciliated, mucus producing columnar epithelium with areas of squamous metaplasia, the solid portion of the tumor was composed of cells with light and electron microscopic characteristics of squamous cells, mucus producing cells and anterior lobe cells of the pituitary: they displayed intercellular bridges, intracellular mucus droplets and on electron microscopy varying numbers of intracytoplasmic secretory granules in the 220-450 millimicron range. The cells thus correspond to an early developmental stage of the pituitary anterior lobe, when the still squamous and columnar Rathke's cleft cells begin to develop their endocrine granulation. For this reason it was felt that "transitional cell tumor of the pituitary" would best characterize this hitherto undescribed neoplasm.

Aged↗
PubMed1978

Neurotoxicity of intrathecal gentamicin: a case report and experimental study.

Distinctive lesions occurred in the brainstem of a 59-year-old patient who had had recent Pseudomonas aeruginosa meningitis treated with parenteral and intrathecal gentamicin sulfate. The lesions were multiple, minute, and discrete, and were characterized by loss of axons, spongiosis, axonal swelling with frequent calcification, loss of astroglia and oligodendroglia, and slight inflammatory response. These lesions were restricted to the myelinated fiber bundles of the pons and mesencephalon. Because similar lesions can occur with other intrathecally administered medications and emboli to the brain, an experimental study in rabbits was done. Similar lesions were produced in normal adult rabbits after a single intracisternal injection of gentamicin sulfate with or without preservative at doses equivalent to 50 and 100 times the human therapeutic dose. Lesions were not seen after injection of normal saline, preservative, or gentamicin sulfate with preservative at doses equivalent to 1 and 10 times the human therapeutic dose. A direct relationship was observed between the cerebrospinal fluid concentrations of gentamicin, brain tissue concentrations of gentamicin, and occurrence of the lesions.

Animals↗
PubMed1978

Supratentorial leptomeningeal hemangioblastoma.

A familial case of von Hippel-Lindau syndrome with a supratentorial leptomeningeal hemangioblastoma is reported. This is the first case in which the meningeal origin of a supratentorial hemangioblastoma has been histologically verified in von Hippel-Lindau syndrome.

Brain Neoplasms↗
PubMed1977

Some important radiological aspects of the kidney in Hippel-Lindau syndrome: the value of prospective study in an affected family.

Eighty-three per cent of the affected members of a family with Hippel-Lindau syndrome had hypernephromas and cortical cysts of the kidney. Two of the asymptomatic members proved to have multiple hypernephromas by a prospective study. The carcinomas are frequently multifocal or bilateral. The cortical cysts are usually multiple, bilateral and small, and often contain carcinoma in the wall. The association of multifocal or bilateral hypernephromas with renal cortical cysts represents an important aspect of the Hippel-Lindau syndrome. The value of prospective renal studies in affected families is stressed.

Adult↗
PubMed1977

Role of the radionuclide brain image in the diagnosis of brainstem gliomas.

The noninvasive detection of brainstem gliomas remains difficult. Eleven of our patients with proven brainstem gliomas had radionuclide brain imaging prior to the initiation of therapy or confirmation of the diagnosis; six studies were positive. Pneumoencephalography remains the most reliable diagnostic test for brainstem glioma, and is invariably required for confirmation. Although angiography is useful in the evaluation of vasocularity it may not detect small infiltrating lesions. Radionuclide brain imaging is useful in the initial workup of patients with suspected brainstem gliomas.

Adult↗