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Biomedical subjects

H D Mennel

Publications and source records attributed to H D Mennel.

At least 55 records · Page 3Linked to original sources

[Menkes' kinky hair disease: a clinical and pathological case report with special reference to glial changes in the brain damage].

Menkes' kinky hair disease (Kinky hair disease, trichopoliodystrophy) is a rare hereditary disease of copper metabolism. In this study we report about the case of a boy with a long course of disease who died at the age of 6. The last phase of the disease was characterized by an almost uncontrollable excitation stage and later by an apallic syndrome. Clinical course and autopsy result are presented here. The protracted course obviously led to unusual neuropathological changes. In addition to the known neuronal damages an excessive reactive gliosis could be found. The unusual neuropathological course of this gliosis perhaps allows the conclusion that apart from neuronal damages disturbances of copper metabolism have an influence on the glia.

Brain↗

[Eosinophilic endomyocarditis post partum or pregnancy-related cardiomyopathy].

We report the case of a 28-year old asthmatic female patient, who developed an acute heart failure beginning with diarrhea, fever, and dyspnea 5 weeks after delivery. After improvement of all vital functions and dismissal from hospital care unit a marked blood hypereosinophilia, left ventricular congestive heart failure, pericardium effusion and fever up to 40 degrees C followed. Endomycardial, bone marrow and skeletal muscle biopsies and the pericardial fluid showed a marked eosinophilic infiltration or polymyositis, respectively, which could be treated successfully with steroids and azathioprin. During steroid medication cytomegalovirus-associated myocarditis developed and was diagnosed by in situ hybridization. CMV hyperimmunoglobulin treatment (Cytotect, Biotest) was started (2 ml/kg bw on day 1 and 3, and 1 ml/kg on days 5, 7 and 9), which led to the eradication of the residual infiltrate and CMV-DNA in the myocardium. After discontinuation of all medication, eosinophilia and asthma recurred so that immunosuppressive treatment was continued.

Adult↗

[Results and reliability of stereotactic and endoscopic biopsies in brain tumors].

Stereotactic neurosurgery was the first "minimal invasive method" in the field of neurosurgery, later followed and partly replaced by endoscopic techniques. One reason for such an approach is sampling of small tissue probes for diagnosis, e.g. in brain tumours not accessible to open surgery. The appropriate method in the hands of the experienced is the "Quetsch" or smear technique. Its reliability is limited by the fact, that the "architectural" or "tissular" components of tumours lack in those purely cytological preparations. Tissue architecture however is crucial for the assessment of different grades in glial tumour progression. The grade of a glial tumour is the most critical information for the patient and the therapist; grading of the supratentorialf gliomas of the adult by means of cellular and tissue pleomorphism therefore forms the basis of Zülchs system of classification and grading of all intracranial tumours by comparison of postoperative survival. The resulting four grade system--slightly modified--is part of the old and new issue of the WHO classification of brain tumours. In order to specify the possibility of correct diagnosis and grading in probes gained by the minimal invasive techniques, we present results of three diagnostic approaches: First: We report results of a study performed during the last twelve years in which a diagnosis of smear preparations had been made on neurosurgical specimens prior to conventional handling. The "blind" cytological diagnosis was then compared with the final diagnosis of the tumour using light and electron microscopy and immunohistochemistry. Second: We report results and estimates of tissue probes gained by the so called sandwich technique in which the removal of material for cytological analysis is done stepwise. By doing so, material representative for different compartments of the neoplasm is obtained. This implies collaboration between neurosurgeon and neuropathologist not only during the time of stereotactic action but also in the planing period; the correct interpretation of the different compartments delivered by imaging methods in this context is essential. Third: We present selected cases of probe sampling under direct visual control by endoscopy. This method is especially useful for tumours bordering the ventricular system. Surface structures and cyst linings can be visualized directly if the endoscopist is familiar with normal and pathological tissue appearance. The specimen for analysis may therefore be taken from the most relevant tumour region and the sandwich technic which means tissue damaging in multiple localisations can be partly or fully avoided.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗

[Primary cerebral lymphoma. Current status of diagnosis and therapy].

Primary cerebral lymphomas are rare tumors. In the last years the frequency of these space occupying lesions is increasing. Diagnosis by image-generating methods is often difficult. Stereotactic biopsy is a reliable diagnostic tool. Explorative craniotomy with tumor resection should be avoided if possible. An effective multimodal therapeutic regimen is the combination of radiotherapy and chemotherapy. Epidemiologic data, diagnostic methods and therapeutic proceedings are presented and discussed on a series of 7 patients with primary cerebral lymphomas treated in our department.

Aged↗

Gangliosides in meningiomas: correlation of Glac2 to intermediary filament.

Human meninges and 29 meningiomas were analyzed as to their glycosphingolipid composition. In the neutral fraction GSL, a mostly even distribution of mono-, di-, tri-and tetrahexoside was demonstrated. In the group of the gangliosides, Glac1 in one broad band in chromatograms occurred in almost all meningiomas; Glac2 was present in 84% of tumours. Members of the Gtn-family were only found in a small minority of tumours while various Gtet-gangliosides were detectable in nearly half of them. No constant pattern or patterns emerged and no correlation to either morphological subtype or malignancy grade could be established. Immunohistochemistry revealed focal presence of Glac2 in a pattern similar to that of vimentin expression. Semiquantitative evaluation showed good correlation between both parameters.

Biomarkers, Tumor↗

Expression of GD2-epitopes in human intracranial tumors and normal brain.

Two monoclonal antibodies (mabs) were raised against ganglioside GD2 (Gtri2) and tested on human intracranial tumors and normal brain by immunohistochemical methods both in frozen and paraffin embedded sections. In normal brain structures, astrocytes were visualized with both mabs (BW 625 and BW 704) almost exclusively in the subventricular and subpial layer. A minor amount of myelin sheaths in well defined localisation was only recognized in frozen sections. Consequently in astrocytic tumors of different grades of malignancy (WHO I-IV), astrocytes were depicted in their characteristic shape and arrangement around vessels. In addition, staining was observed in meningiomas and schwannomas, but not in pituitary adenomas or metastatic carcinomas. In meningioma und schwannoma the staining was restricted to the cellular periphery and was again present in frozen section material and absent in paraffin embedded tissue. In astrocytes, reactive and neoplastic, obviously fibrous processes and cytoplasm were distinctly stained both in frozen and paraffin embedded sections. It is concluded that some neuroectodermal derived cells as well as myelin of defined localisation express GD2 on the membrane surfaces and in the cytoplasm. The latter may primarily be the case in fibrous astrocytes, which were stained in reactive and pathologic conditions. The reaction can be used as diagnostic tool in astrocytic tumors; its positive therapeutic significance is hampered by the fact that (1) not all cells in heterogeneous tumor populations express the epitope and (2) there are normal structures which do so.

Antibodies, Monoclonal↗

Application of the AgNOR technique to neurooncology.

The silver staining of interphase nucleolar organizer regions (NORs) is a recently developed method to measure cell proliferation in tissue specimens. The major silver staining protein is nucleolin, a 92 kd nucleolar protein, which probably controls rDNA transcription. Nucleolin itself is under control of p34cdc2 kinase, which is a subunit of M phase kinase. The specific silver staining of NORs measures ribosomal gene activity and is therefore useful in measuring cell proliferation via nucleolar biosynthetic activity. Although human tumors of different malignancy grades usually can be distinguished by their AgNOR number, there is considerable overlap between different grades which significantly hampers their use in individual cases. For routine application of the AgNOR technique in histopathology we propose a standardised staining protocol with use of internal control cells and the use of image analysis for AgNOR enumeration.

Animals↗

Stereotactic-endoscopic procedures on processes of the cranial midline.

The term "midline tumour" is defined partly from the topographic and from the pathogenetic point of view. Problems of modern imagegenerating procedures in establishing the diagnosis of cerebral midline lesions are described. The role of stereotactic diagnostic and therapeutic interventions is emphasized. Stereotactic brain tumour biopsy, installation of shunts and reservoirs under visual control are performed. Interstitial radiotherapy is carried out for low-grade gliomas. As an important innovation, stereotactic procedures are combined with endoscopic techniques. Particular diagnostic and therapeutic difficulties of typical midline tumours such as craniopharyngeoma, germinoma, glioma and primary cerebral lymphoma as a local extension are discussed. A reasonable concept in diagnosis and therapy of cerebral midline lesions is proposed.

Adolescent↗

Grading of intracranial tumors following the WHO classification.

The grading scheme contained in the WHO classification "Histological Typing of CNS Tumors" has been introduced after long and controversial discussions. Of the three major systems for grading intracranial tumors which were at hand, Zülch's is closest to the WHO system. The criteria for, and general remarks about, the WHO system for the evaluation of malignancy grades are delineated. Their almost complete concordance with Zülch's "horizontal" scheme is emphasized, although some minor differences are mentioned. It is the author's belief, which may, however not be theoretically fully justified, that this system has been applied successfully since the publication of WHO classification. Opposition to WHO grading mostly reflects general pessimism about the possibility of gaining information concerning proliferation from morphology. However, the analysis of therapeutic trials in the last decades has shown that histological grade is of great predictive value. This correlation can be improved by the broad and exact application of the system.

Brain↗

Chromosome numbers and DNA-content in intracerebrally transplanted experimental gliomas.

Serially transplanted experimental tumors of the central and peripheral nervous system can be used as models to investigate open questions in human neurooncology. Altered susceptibility of higher passages to chemotherapy might be correlated with chromosome number and DNA-content variations which would be partly expressed as changes in proliferation behaviour. Karyotypes therefore were analysed in the 73rd to 90th generations of transplanted experimental gliomas. Wide variation of chromosome number was observed; 2 major types of distribution occurred, the one presenting with, the other without stemlines. Large chromosomes # 1 and # 4 were often monosomic, while small chromosomes of ## 8 to 20 were increased up to the fivefold. Lines with prominent and few markers were observed. On the whole, cells of the proliferating pool of the tumor had to be considered as hypotetraploid. Comparison of chromosome numbers and DNA content gave good correlation; differences between the 2 were explained by the fact that only the number of chromosomes was taken into account, regardless of whether small or large chromosomes were lacking or in excess. When intracerebrally transplanted tumors had been previously treated by administration of BCNU, the DNA content was altered, indicating an increased share of diploid cells in the proliferation pool. Results are at variance with earlier findings in tissue cultures of directly induced malignant gliomas and neurinomas in rats. The findings in transplanted tumors can be interpreted as a result of increased malignancy in transplantation tumors, documented by rapid growth in the animal and dedifferentiated histologic morphology.

Animals↗

Tissue architecture and glycosphingolipid content in human gliomas II-IV.

Contradictory results have been reported claiming either none, partial or almost complete correlation between the complexity of GSL compound profiles and the assumed glial tumor differentiation. Therefore an attempt was made to compare GSL patterns with both the general (final) tumor diagnosis and malignancy grade (WHO) as well as the regional evaluation of the histology and the grading in the tumor tissue pieces directly subjected to biochemical analysis. Regional and general (final) diagnosis did not always correspond, especially when more than one tissue sample of a given tumor was analyzed. Four GSL component patterns were identified by TLC: GSL-type I with gangliosides primarily of the simple Glac-family lacking sulfatide and the more complex Gtri- and Gtet-gangliosides, GSL-type II with ganglioside of the Glac- and Gtri-families, also without sulfatide, and GSL-type III, with more complex gangliosides of the Gtri- and Gtet-families in addition to Glac-gangliosides and sulfatide, similar to the normal brain pattern, and the pattern of normal brain. There was only insufficient correlation between these GSL-type patterns and final diagnoses. However, between regional diagnosis of astrocytoma II and GSL-type III on the one hand and glioblastoma multiforme IV and GSL-type I on the other hand, a coincidence of more than 85% was found. In only 50% the intermediate GSL-type II and glioma III were associated. There was no relation between GFAP or vimentin expression and histology or GSL-type both with regard to final and regional diagnoses. Regional astrocytoma architectures exhibiting GSL-type III were mostly fibrillary, whilst glioblastomas with GSL component pattern I had often a giant cell make up.(ABSTRACT TRUNCATED AT 250 WORDS)

Antigens, Differentiation↗

[Morphology and prognosis of intracranial tumors].

Traditionally, prognosis of intracranial tumors is considered to be correlated with histology. Today a four grade scale is increasingly used, in which the grades II through IV are attributed to gliomas depending upon their cellular and tissue pleomorphism. These tumors will regrow after one to five years. Tumors which will not relapse after successful operation are considered to be of grade I. This grading, which is part of the WHO histological typing of intracranial tumors, can be supplemented by the proliferation markers. These may allow better evaluation of the prognosis.

Brain↗

[Stereotactic intervention as a contribution to diagnosis and therapy of paramedian processes].

In open brain surgery, access to processes in the midline region is difficult. Exact diagnosis of the tumour type is crucial to the choice of therapeutic modality. In many cases, modern imaging procedures such as CT and MRI are useful diagnostic tools. The diagnosis is confirmed by direct biopsy and histological examination. Selected cases of tumours close to the midline (germinoma, pilocytic astrocytoma, primary cerebral malignant lymphoma) are presented. CT and MRI are helpful in demonstrating space-occupying processes. The tumour's histological type is determined by CT-stereotactic biopsy. The histopathological diagnosis is established by means of a crush preparation. Appropriate forms of therapy are proposed on the basis of the diagnosis of tumour type. The results are impressive, due to the efficacy of stereotactic procedures as an additional means of diagnosing and treating space-occupying processes of the midline region.

Astrocytoma↗

[Epithelial cyst of the central nervous system. A rare abnormality].

A cystic process in the right frontal lobe of the brain in a man of 35 years of age, and an intramedullary cyst in the upper cervical spinal cord in a 29-year old woman, are described. In both cases, the wall of the cyst consisted of mucosal cells and cells having cilia pointing to the inside, located on a thin tissue layer, the structure of which resembled that of a meningioma. Central nervous cysts of this kind are usefully classified together with other, similar cysts (bronchogenic, ependymal and others) as "epithelial cysts". They must be differentiated from neoplastic tumours. Analysis of the cystic contents can prove helpful.

Adult↗

Cell proliferation in intracranial tumours: selective silver staining of nucleolar organizer regions (AgNORs). Application to surgical and experimental neuro-oncology.

A novel tool in diagnostic and experimental pathology, the AgNOR-technique, which consists of visualization of ribosomal gene activity by selective silver staining, was applied to 144 cytological specimens of human tumours of the nervous system. The number of silver-stained nucleolar organizer regions (AgNORs) was correlated with the biological behaviour of the tumours investigated; low AgNOR number were observed in benign neoplasms such as meningiomas and schwannomas and higher AgNOR numbers in glioblastomas and metastases. The mean AgNOR number per cell was 3.15 in astrocytomas, 4.5 in anaplastic astrocytomas and 5.86 in glioblastoma multiforme. Benign and malignant lesions showed different distribution patterns of AgNORs, with few but centrally located AgNORs in benign, and multiple but scattered AgNORs in malignant tumours. AgNOR number per cell and AgNOR area revealed an inverse relationship (correlation coefficient -0.15, linear regression). In addition to the human tumours, two N-nitroso-N-ethyl-urea (NEU) induced tumors in BD-IX rats a mixed glioma (G-XIII) and a malignant schwannoma (N-XII), were investigated. Twelve G-XIII gliomas revealed homogenous AgNOR-counts (standard error of the mean less than 10%), with absolute values between the values obtained for human glioblastomas and metastases. Seven N-XIII subcutaneously transplanted schwannomas revealed higher AgNOR values than human schwannomas, but lower than experimental gliomas. It is concluded that the AgNOR method, as a technique for visualization of ribosomal gene activity, is valuable for assessing proliferative activity and malignancy in both diagnostic and experimental neuropathology.

Animals↗

[Capillary microscopy controlled nail fold biopsy in the diagnosis of collagen disease and vasculitis].

In cases of suspected microangiopathies morphologic abnormal capillaries were identified and marked by nailfold capillary microscopy (NCM). The marked nailfold areas were precisely biopsied and the biopsies were examined by electronic-microscopy and light microscopy (HE and immune-histochemical). Histological findings well correlated with clinical and capillaroscopical findings. It is concluded that NCM-assisted nailfold biopsy helps to achieve histologic proof of microangiopathies and improves diagnostic accuracy in collagen vascular diseases and vasculitides.

Biopsy↗

Glycosphingolipids of human gliomas.

Histologically characterized human gliomas of various grades of malignancy obtained during surgery were extracted, and their glycolipids were isolated and partially identified. Among the gliomas analyzed, three types of glycolipid component distribution could be identified. The glycosphingolipid (GSL) type I pattern correlated closely with that of the most malignant gliomas (Grade IV). Its neutral GSLs consisted of glucosyl- and, as a major component, dihexosylceramide, in addition to globo- and neolactotetraosylceramide. Galactosylceramide and sulfatide were absent. The gangliosides of GSL type I were almost exclusively of the GLac family, aside from small amounts of neolacto-series-derived species. The neutral components of GSL type II were similar to those of GSL type I. The acidic compounds of GSL type II were gangliosides of the Gtri family and trace amounts of neolacto-series sialoglycolipids, in addition to GLac1 and GLac2. GSL type II contained no Gtet gangliosides and no sulfatide. The GSL type III pattern was that of the most benign gliomas, with all glycolipids present that are found in normal brain and, in addition, those of the GSL type II.

Astrocytoma↗

Neuroprotective effect of memantine demonstrated in vivo and in vitro.

The purpose of the present study was to test whether the anticonvulsant, memantine (1-amino-3,5-dimethyladamantane), can protect neurons against hypoxic or ischemic damage. To this end, we used a rat model of transient forebrain ischemia and cultured neurons from chick embryo cerebral hemispheres. Ischemia was induced for 10 min by clamping both carotid arteries and lowering the mean arterial blood pressure to 40 mm Hg; the rats were allowed to recover for 7 days. Cultured neurons were made hypoxic with 1 mmol/l NaCN added to the incubation medium for 30 min followed by a recovery period of 3 days. The possible effects of memantine were compared with those produced by a typical non-competitive NMDA antagonist, dizocilpine. Similar effects were obtained with both drugs. The drugs reduced the damage caused by transient ischemia to neurons of the hippocampal CA1 subfield. Memantine (10 and 20 mg/kg) had a dose-dependent effect when administered intraperitoneally to the rats 1 h before ischemia. Dizocilpine was active in this model at a dosage of 1 mg/kg. When administered after ischemia, 10 mg/kg memantine significantly protected CA1 neurons against ischemic damage. Furthermore, the drugs protected cultured neurons against hypoxic damage. The lowest effective concentration was 0.1 mumol/l for dizocilpine and 1 mumol/l for memantine. Thus, memantine possesses neuroprotective activity but is less potent than dizocilpine.

Adenosine Triphosphate↗