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Biomedical subjects

M Kornfeld

Publications and source records attributed to M Kornfeld.

At least 55 records · Page 3Linked to original sources
PubMed1986

Granular cell glioblastoma: a malignant granular cell neoplasm of astrocytic origin.

Granular cell tumors in the cerebral hemispheres are rare and of unknown origin. Two large aggressive cerebral neoplasms composed mainly of granular cells and also containing neoplastic astrocytes were studied histologically, ultrastructurally and immunohistochemically. In both instances, transitional forms between astrocytes and typical granular cells were demonstrated. In the second case which consisted of a central core of typical glioblastoma surrounded by a thick shell of granular cells, the latter spread in a fashion that is characteristic of intrinsic tumors of the central nervous system by forming abundant secondary structures of Scherer, particularly under the pia and around cortical blood vessels. All the evidence indicates that in these two cases granular cells were transformed neoplastic astrocytes. Paradoxically, many granular cells exhibited benign cytological features that belied their malignant nature.

Astrocytes↗
PubMed1985

Neuropathology of ornithine carbamyl transferase deficiency.

Neuropathologic findings in two cases of ornithine carbamyl transferase (OCT) deficiency are presented. In one, a boy 3 days old, the only abnormality related to the enzyme defect was metabolic gliosis which was unusual in distribution being present mainly in the brain stem. In the other case, a girl who died at the age of 2 3/12 years, the brain showed metabolic gliosis in typical location, widespread ulegyria, and moderate atrophy of the internal granular layer in the cerebellum accompanied by development of expansions of Purkinje cell dendrites. Case 2 demonstrates how rapidly changes previously demonstrated in the cerebral cortex after a few months of illness can proceed to massive hemispheric destruction. By now ulegyria has been observed in several disorders of the urea cycle.

Brain↗
PubMed1984

Cisplatin neuropathy. Clinical, electrophysiologic, morphologic, and toxicologic studies.

Ten of 11 patients with ovarian cancer receiving cisplatin developed a distal sensory neuropathy, manifested early by decreased vibratory sensibility in toes and depressed ankle jerks and later by uncomfortable paresthesias. Eleven patients receiving cisplatin, 50 mg/m2 monthly (mean total, 580 mg/m2) were studied prospectively with monthly neurologic examinations and conduction velocity determinations of median, peroneal, and sural nerves. Early signs were decreased vibratory sensibility in toes (mean dose, 417 +/- 132 mg/m2 [SD]) and loss of ankle jerks (mean dose, 455 +/- 86 mg/m2). With continued therapy, four developed paresthesias. Strength was unaffected. Sural nerve responses abruptly disappeared in six of peripheral nerves from four patients showed axonal degeneration and secondary myelin breakdown. Platinum concentrations in three patients were similar in tumor (3.3 micrograms/g), sural nerves (3.5 micrograms/g), and spinal ganglia (3.8 micrograms/g), but lower in brain (0.17 microgram/g). This may explain the cisplatin toxicity of peripheral nerves with relative sparing of the central nervous system.

Adult↗
PubMed1984

Meningeal carcinomatosis from an ovarian primary: a clinicopathologic study.

Meningeal carcinomatosis due to primary ovarian carcinoma is an uncommon metastasis that presents difficult problems in diagnosis and management of patients known to have this disease. There is evidence that extended survival due to advances in treatment of ovarian carcinoma has improved our ability to diagnose meningeal carcinomatosis of this tumor more often. Herein the first case of meningeal metastasis of primary ovarian cancer with cytologic and postmortem examination is discussed.

Adenocarcinoma↗
PubMed1984

Glycogenosis type VIII.

Glycogenosis Type VIII, characterized ultrastructurally by an accumulation of rosettes (alpha-particles) of glycogen in the central nervous system, is an extremely rare condition; only two sporadic cases are on record. The first complete autopsy on a patient with cerebral alpha-particle glycogenosis, a 20-year-old American-Indian female, is the subject of this report. The case was clinically unique because of long survival and presumable familial incidence. The gross pathology was characterized by severe brain atrophy but preserved thickness of the cortical mantle. Vacuolation of the neuropil was the main histological abnormality and was most extensive in the striatum and less so in the cerebral cortex and some brain stem nuclei. Biochemical analysis showed glycogen levels elevated fiftyfold in the striatum and eightfold in the cerebral cortex in comparison with control tissue. Ultrastructural observations and evidence obtained from the Golgi method suggest that the distal axon was the principal site of storage. Two prominent additional abnormalities, spheroids, not previously observed in this disease, and massive accumulation of lipofuscin, were probably both related to the prolonged course of illness. The viscera, including the liver were morphologically free of storage.

Adult↗
PubMed1983

Time course of blood nerve barrier reconstitution after sectioning: implications for xenograft studies.

The time course of blood-nerve barrier recovery in mouse nerves was studied after proximal and distal segmental sciatic sections. Transfer constants for uptake of isotopically labeled sucrose and urea were determined. Sectioned segments were examined ultrastructurally. Marked alterations in isotope uptake for 2 months with gradual restoration of blood-nerve barrier by 6 months was found. Ultrastructure of sectioned segments showed increased numbers of perineurial compartments. Human nerve xenografts to immunosuppressed animals remain hypomyelinated for 6 months which may be related to damage to the blood-nerve barrier.

Animals↗
PubMed1983

Experimental influenza B virus toxicity in mice. A possible model for Reye's syndrome.

Mice intravenously injected with concentrated infectious influenza B/Lee/40 virus (LD50 = 6400 hemagglutinin units) developed lethargy, seizures, coma, and death 1 to 3 days later. The cerebrospinal fluid cell count was normal. Serum glutamic oxaloacetic transaminase levels increased 19-fold and plasma ammonia levels elevated 2.6-fold over control values. Serum bilirubin levels remained normal. Microvesicular fatty metamorphosis developed in the liver, whereas the brain showed mild cerebral edema without inflammatory changes. Viral propagation did not occur in liver or brain, but viral hemagglutinin, neuraminidase, and probably nucleoprotein antigens were produced in hepatocytes. Many of the clinical, biochemical, and pathologic features of the mouse illness are similar to those seen in Reye's syndrome.

Animals↗
PubMed1981

Acute thallium poisoning: toxicological and morphological studies of the nervous system.

Nine days following ingestion of 5 to 10 gm of thallium nitrate, a young man died with severe cranial and peripheral neuropathy, anuria, and heart failure. Ultrastructural examination of nerves obtained on days 7 and 9 demonstrated axonal degeneration with secondary myelin loss. Axons were swollen and contained distended mitochondria and vacuoles. Thallium levels in more than twenty organs and body fluids ranged from below 1.0 to 178 microgram/gm; concentrations in twenty areas of the nervous system ranged from 29 to 140 microgram/gm. The highest brain levels of thallium were found in gray matter. In the thalamus, 87% of the thallium was present in cell sap. Tissue concentrations of thallium did not parallel those reported for potassium, suggesting that thallium distribution differs from potassium distribution in human beings.

Acute Disease↗
PubMed1981

Morphometric analysis of secretory granules and prolactin levels in chromophobe pituitary adenoma.

Morphometric analysis of secretory granules was performed in 16 chromophobe adenomas: seven undifferentiated cell adenomas (group A), two of which were accompanied by mild hyperprolactinemia and nine prolactin cell sparsely granulated adenomas (group B), all associated with hyperprolactinemia. The results of morphometry were correlated with levels of prolactin in tumor and serum. The range of granule diameters was similar in both groups but granule size frequency was different resulting in a characteristic histogram for each group. Poor relationship existed between granule mean diameter and prolactin in tissue of the group B. In the same group there was virtually no linear relationship between serum prolactin on one side and granule mean diameter, granule density or tissue prolactin on the other. Also in the group B a paradoxical inverse relationship was found between granule density and tumor prolactin. Apparently, in prolactin cell adenoma a sizable part of hormone is located outside of mature secretory granules. An abundance of immature granules, misplaced exocytosis, crinophagia or defect in packaging of the hormone could all explain this paradox but the last possibility seems most plausible.

Adenoma, Chromophobe↗
PubMed1981

Cytomegalovirus labyrinthitis in an infant: morphological, virological, and immunofluorescent studies.

The inner ears obtained from an infant who died of severe congenital cytomegalovirus infection were examined using virological and morphological methods. The techniques of microdissection and surface preparations, immunofluorescent microscopy, and transmission and scanning electron microscopy were employed. Cytomegalovirus was isolated from the perilymph. Injury to the cochlea was minor and was confined to the first half of the basal turn. There were signs of a severe labyrinthitis of the vestibular endolymphatic system. Vestibular organs, especially the saccule and utricle, showed severe damage, with collapse of the saccular membranous wall. Inclusion-bearing cells containing cytomegalovirus antigen, as shown by immunofluorescent staining, and cytomegalovirus virions, as observed by electron microscopy, were found on the endolymphatic surface of the membranous walls, mainly in the saccule and utricle. In the utricle, the infected cells appeared to be concentrated in the regions where the so-called dark cells were located. These dark cells are similar to the proximal tubule cells in the kidney, another common target for cytomegalovirus infection.

Antigens, Viral↗
PubMed1980

Pure axonal neuropathy: nerve xenografts and clinicopathological study of a family with peripheral neuropathy, hereditary ataxia, focal necrotizing encephalopathy, and spongy degeneration of brain.

Three family members had the unusual combination of severe peripheral neuropathy, atypical hereditary ataxia, spongy degeneration of cerbral hemispheres, and cerebellar and brainstem foci of necrotizing encephalopathy, proved pathologically in one autopsied case. A sural nerve from a patient, devoid of myelinated fibers, was transplanted into thymectomized, lethally irradiated, and bone marrow reconstituted adult mice. A normal number of myelinated fibers was present in the grafts eleven weeks later. Eighteen weeks after grafting, mice were reconstituted with syngeneic thymus to return immunocompetence. Schwann cells in the graft were rejected and axons became totally denuded of myelin after thymus reconstitution. The peripheral neuropathy is thus due to axonal disease since human Schwann cells were capable of normally myelinating regenerating mouse axons. A puzzling feature after rejection was the absence of myelin debris containing macrophages in the grafts. It is suggested that part of the rejection process in this model is mediated by antibody rather than by cellular mechanisms.

Adolescent↗
PubMed1980

Mixed nemaline-mitochondrial "myopathy".

A 4-year-old boy suffering from a nonprogressive muscular weakness had a muscle biopsy which ultrastructurally showed large aggregates of nemaline bodies and mitochondria in myofibers; occasional concentric lamellated bodies were present as well. The mitochondria were mostly at the periphery of collections of nemaline bodies, less commonly in their central portions. The light microscopic features differed from those of either nemaline or mitochondrial myopathy and were somewhat reminiscent of multicore disease. Evidence suggestive of possible neurogenic origin of both rods and mitochondria is reviewed.

Biopsy↗
PubMed1980

Influenza A virus and Reye's syndrome in adults.

We report fatal Reye's syndrome in two adults following proven influenza A viral infections. Reye's syndrome is, therefore, not confined to children but may also occur in adults. Many reported cases of postinfluenza A encephalopathy have clinical and pathological features of Reye's syndrome suggesting that they are not due to postinfectious perivenous demyelination.

Adolescent↗