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Biomedical subjects

R C Janzer

Publications and source records attributed to R C Janzer.

At least 19 recordsLinked to original sources

Induction of the blood-brain barrier specific HT7 and neurothelin epitopes in endothelial cells of the chick chorioallantoic vessels by a soluble factor derived from astrocytes.

Some blood-brain barrier properties of microvascular endothelial cells have been shown to be inducible by astrocytes. We tested the hypothesis that this cellular interaction is mediated by a soluble factor(s). Chick chorioallantoic vessels in ovo were constantly exposed to astrocyte-conditioned medium. We found that endothelial cells exposed to astrocyte-derived factors, but not to glioma- or endothelial cell-derived factors, expressed the HT7 antigen and neurothelin, two specific markers of the blood-brain barrier phenotype. These results indicate that a soluble factor(s) secreted by astrocytes is capable to induce specific blood-brain barrier properties in endothelial cells of non-neural origin.

Animals

A modified Hortega-Globus stain is superior to Bielschowsky and Bodian stains for demonstrating neuritic plaques.

The quantitative assessment of the age-dependent number of neuritic plaques is essential for the diagnosis of Alzheimer type dementia. This study reports the superiority of a modified Hortega-Globus stain compared to Bielschowsky and Bodian stains applied to samples obtained from ten brains of patients with a clinical history of progressive dementia. In two of ten cases only the modified Hortega-Globus stain allowed confirmation of the diagnosis of senile dementia of the Alzheimer type (SDAT). The counts of neuritic plaques in sections stained by other methods were not sufficient to establish the histological diagnosis of SDAT. These results indicate that the choice of the most sensitive staining method is critical for the correct histopathologic diagnosis of the Alzheimer type dementia.

Aged

Acute fatal myeloencephalopathy after combined intrathecal chemotherapy in a child with acute lymphoblastic leukemia.

We report a case of a fatal toxic encephalomyelopathy in a 12-year-old girl due to prophylactic intrathecal injection of methotrexate and cytosine arabinoside, with a characteristic progressive symptomatology leading to death after 28 days. The location and type of neuropathological changes support the hypothesis of a direct toxic effect of methotrexate and/or cytosine arabinoside on structures directly exposed to the cerebrospinal fluid.

Antineoplastic Combined Chemotherapy Protocols

Arteriovenous malformation in meningothelial meningioma: case report.

An unusual association of a meningioma and an arteriovenous malformation is reported. A 68-year-old man developed left homonymous hemianopsia, left hemiparesis, and gaze palsy. Magnetic resonance imaging showed a right occipital mass lesion containing multiple signal-void areas with tubular and honeycomb appearance, suggesting a marked vascular component. An angiogram showed abnormal vasculature in the mass supplied by the posterior cerebral artery and a dural arteriovenous malformation on the tentorium. Neuropathological examination after total removal of the mass revealed a meningothelial meningioma including major portions of an arteriovenous malformation that extended from the dura and leptomeninges, through the meningioma, and into the occipital lobe, where the tumor was located.

Aged

Meningovascular form of neuroborreliosis: similarities between neuropathological findings in a case of Lyme disease and those occurring in tertiary neurosyphilis.

Recent observations have delineated the neurological manifestations of Lyme disease, but, to our knowledge, no detailed neuropathological study from autopsy cases has been reported. In this report we describe the neuropathological findings in a case of Lyme neuroborreliosis. The chronic meningitis, the occlusive meningovascular and secondary parenchymal changes that we found are similar to those occurring in the meningovascular form of neurosyphilis. Thus, we suggest that the case described here represents the meningovascular form of tertiary Lyme neuroborreliosis.

Humans

In vivo metabolism and reaction with DNA of the cytostatic agent, 5-(3,3-dimethyl-1-triazeno)imidazole-4-carboxamide (DTIC).

The cytostatic drug dacarbazine [DTIC, 5-(3,3-dimethyl-1-triazeno)imidazole-4-carboxamide] is strongly carcinogenic in rats. Bioactivation of DTIC yields a methylating intermediate but the extent of interaction with cellular macromolecules has not previously been reported. Following a single i.p. injection of [14C-methyl]DTIC, exhalation of 14CO2 occurred with a t1/2 max of approximately 2 hr (0.95 mg/kg) and 2.5 hr (95 mg/kg). Of the total radioactivity administered, 8.5% was exhaled as 14CO2; 54% was excreted via the urine, predominantly as unchanged DTIC. In liver, kidney and lung, formation of 7-[14C]methylguanine in DNA and RNA was directly proportional with dose. DNA methylation by a single dose of DTIC (9.8 mg/kg; 5 hr survival time) was highest in liver (35 mumoles 7-methylguanine/mole guanine), followed by kidney (25 mumoles) and lung (20 mumoles). The remainder tissues showed 7-methylguanine concentrations approximately 50% of those in liver DNA, with the exception of the brain which had a very low extent of DNA modification (approximately 1 mumole/mole guanine). At the specific radioactivity used (48 mCi/mmole), the promutagenic base O6-methylguanine was only detectable in liver, kidney, lung, and stomach DNA (0.6-0.8 mumoles/mole guanine). Autoradiographic studies revealed a diffuse distribution of reaction products in rat liver. In contrast, N-nitrosodimethylamine and related carcinogens known to be bioactivated by the hepatic cytochrome P-450 system show a predominantly centrilobular distribution. This difference may be due to the greater stability of proximate carcinogens generated by alpha-C hydroxylation at one of the methyl groups of DTIC.

Animals

Progressive myoclonic epilepsy (Unverricht type) with atypical Lafora bodies. Case report.

A patient with advanced progressive myoclonic epilepsy (Unverricht type) with Lafora bodies is presented. Although the clinical history and symptoms were classical, the regional distribution of the cerebral involvement differed from the classical picture: the corpora mamillaria, the nucleus subthalamicus, and the nucleus ruber, which are normally reported to be spared, contained multiple Lafora bodies, whereas the lateral geniculate body, which is usually involved, was intact. The number of inclusions per cell, up to 25, was extremely high and correlated with the marked cortical atrophy and the prolonged clinical course. Using electron microscopy, type I and type II Lafora bodies were found, but the latter lacked the typical filamentous ultrastructure in the peripheral zone. The lack of visceral Lafora bodies in this case suggests that liver, muscle, and skin biopsies, which are widely used for the diagnosis, may lead to false negative results and cannot always replace a stereotactic brain biopsy. The differential diagnosis on polyglucosan bodies is emphasized.

Adult

Early and late effects on the normal dog brain of permanent interstitial iridium-192 irradiation.

To investigate the effects of a permanent interstitial source of gamma-irradiation on normal brain tissue, single iridium-192 (Ir-192) wires (1.05 mCi) were stereotactically implanted into the left centrum semiovale of adult dogs (survival times, 25, 46, 74, 230 and 362 days). Within 25 days, a coagulation necrosis developed in the immediate vicinity of the radioactive source. In later stages, the necrosis increased in size and became progressively mineralized. Staining for extravascular immunoreactive serum proteins revealed the presence of a chronic perifocal vasogenic edema, which extended into the white matter of the entire ipsilateral hemisphere. This edema persisted through all stages and showed a significant decrease only in the animal with a 1-year survival. A reactive gliosis with formation of a dense network of glial fibrillary acidic protein-positive astrocytes developed around the central necrosis in the adjacent white matter and, at later stages, in the contralateral hemisphere. Demyelination was restricted to the ipsilateral centrum semiovale without affecting the internal capsule or the contralateral hemisphere. It was present as early as 25 days and showed no tendency to increase at later stages. Analysis of the sequential morphological changes following Ir-192 implantation suggests that the central coagulation necrosis represents a direct radiation effect, the sharp focal delineation of which can be explained by the physical characteristics of the radiation source, i.e., rapid fall-off of the dose at short distances. Due to the continuous emission of radiation energy, there is a perifocal zone with overlapping of progressive radiation damage and tissue organization. This focus becomes the source of a chronic vasogenic edema, which in turn is most likely to be responsible for the partial demyelination of the adjacent centrum semiovale. The widespread reactive gliosis observed at all stages may also, in part, be a consequence of chronic vasogenic edema, but its distribution suggests that direct radiation effects may also be involved.

Animals

Severe neonatal centronuclear (myotubular) myopathy: an X-linked recessive disorder.

Prenatal onset and rapidly fatal course of centronuclear myopathy are described in four male newborns including two brothers. Diagnosis was established by muscle biopsy within the first week of life in two and at autopsy in the two other patients: Central nuclei, central aggregation of oxydative enzyme activity in the majority of muscle fibers and type 1 fibre hypotrophy were demonstrated. Prenatal manifestation included polyhydramnios, reduced fetal movements and breech presentation. All four newborns developed respiratory insufficiency requiring artificial ventilation immediately after birth. Severe muscular weakness and hypotonia as well as hardly elicitable grasping, deep tendon reflexes and Moro response were noticed. Additional findings included high arched palate, joint contractures, thin ribs, lung hypoplasia, abundant skin and cryptorchidism. In two families, the pedigree contains other affected males, suggesting X-linked inheritance. Seven female carriers were clinically healthy and one of them showed normal muscle histology. Fourteen previously published neonatal cases of centronuclear myopathy are reviewed and compared with our findings. This severe perinatal form of centronuclear myopathy has to be considered in male fetuses and newborns with polyhydramnios and respiratory failure due to muscular weakness or in infants who died of unexplained postnatal asphyxia. Diagnosis should be established by muscle biopsy.

Genetic Carrier Screening

The capillary bed in the choroid plexus of the lateral ventricles: a study of luminal casts.

Micro-angioarchitecture of the choroid plexus of the lateral ventricles is investigated in microcorrosion casts of animal and human preparations studied with the scanning electron microscope. The capillary bed in the diverse regions of the tissue belongs to one of three patterns: (1)-a network of capillary meshes that envelop the larger arteries and veins predominates in the central segment. (2)-in the villous regions a "leaf-like" organization of sinusoids is found together with (3)-fronds of "glomerular" formations. "Glomeruli" are formed when arterial afferents and venous efferents converge in a quasi hilar structure before branching in arterio-venous loops. Nodular thickenings are observed on glomerular capillaries. The preparations studied (rat, dog, human) are remarkably similar and differ mostly in degree of occurrence of common architectural patterns. Arterio-venous communications are found at the hilus of human glomerular formations.

Adult

An unusual association of dentato-rubral degeneration with spinal ataxia, ophthalmoplegia and multiple cranial nerve palsies.

Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the dentato-rubral system, of the posterior columns and of several cranial nerve nuclei. The problems of differential diagnosis and classification are discussed.

Ataxia

Primary intracranial germ-cell tumors. A clinicopathological study of 14 cases.

Fourteen cases of primary intracranial germ-cell tumors are presented. Histologically, there were eight germinomas, three teratomas, and three germ-cell tumors of more than one histological type. Immunohistochemical studies revealed alpha-fetoprotein in yolk-sac tumor components in two cases and beta human choriogonadotropin in syncytiotrophoblastic giant cells in one case. One teratoma contained an unusual pleomorphic sarcomatous portion with features of early myoblastic differentiation. Comparison of intracranial with gonadal germ-cell tumors shows that the same subtypes are found in both locations with comparable incidence and similar biological behavior. The detailed World Health Organization classification of testicular germ-cell tumors should be applied to the histopathological classification of intracranial germ-cell tumors. Despite the critical location of intracranial germ-cell tumors, a good outcome can be achieved by optimal surgical excision. A primary microsurgical approach provides a histopathological diagnosis, which is indispensable for the proper choice of postoperative management.

Adolescent

The relation between solid cell nests and C cells of the thyroid gland: an immunohistochemical and morphometric investigation.

Thyroid tissue of 300 routine autopsies was processed in a standardized manner. So-called solid cell nests (SCN) were found in 21 patients (7%). These cases were investigated carefully by serial step sectioning. In order to explore the correlation of SCN to the C-cell system, the sections were stained by silver impregnation and the immunoperoxidase method. Morphometric analyses revealed a significant increase in the density of C cells in the proximity of the SCN. With progressive distance from the SCN, the C-cell density decreased and reached normal values. In 30% of the cases argyrophilic and calcitonin-positive cells were found lying within the SCN. Occasionally, mixed follicles could be discerned: These were lined on the one side by a multilayered squamous epithelium, on the other side by normal monolayered cubic follicular epithelium, and contained a peculiar granular material. In one case, SCN were associated with intrathyroid portions of the parathyroids and adult adipose tissue, in a second case with adipose tissue only. Most probably SCN are vestiges of the ultimobranchial body and should be interpreted as such, despite the fact that other authors have expressed different views. The lack of disturbances in the calcium metabolism of the patients and the absence of medullary carcinoma in their family histories led us to interpret locally confined C-cell hyperplasia not as reactive nor premalignant, but rather as normal.

Adipose Tissue

Perisulcal infarcts: lesions caused by hypotension during increased intracranial pressure.

A pattern of cortical necrosis surrounding the cerebral sulci and similar to ulegyria was found in 5 patients. The lesions were widely disseminated in all parts of the hemispheric cortex, affecting mostly the deep cortex of several adjacent sulci. They were hemorrhagic in 3 patients, ischemic in the others. Each patient had suffered a severe brain injury and became comatose thereafter. Increased intracranial pressure was evident from clinical findings, necropsy changes, or both. While in coma, each patient had at lease one episode of hypotension. The data suggest that perisulcal infarcts are a manifestation of diminished vascular perfusion during a period of increased intracranial pressure.

Adult

[C-cells of the thyroid gland].

In the human thyroid gland, the C-cell system reacts in physiologic and pathologic conditions as an entity and represents an independent endocrine organ. Its special features are its embryology, which relates it to other derivatives of the neural crest, the special histologic methods needed to demonstrate its disseminated C cells, and the obscure physiologic role of its hormone, calcitonin. Although there exist many histopathologic findings, the only C-cell disease of clinical interest is medullary carcinoma, which in its familial form is usually associated with pluriglandular syndromes.

Calcitonin