Wernicke's encephalopathy, Korsakow's amnesic state and thiamine deficient encephalopathy.
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Biomedical subjects
Publications and source records attributed to A O Vortmeyer.
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We report the first case of a congenital intracranial chordoma. Hydrocephalus, sixth and seventh cranial nerve palsy, and torticollis were observed shortly after birth. The tumour was delineated by sonography, CT scans and MRI and the diagnosis confirmed after subtotal resection at the end of the newborn period.
Neuropathological examination of three patients who were maintained on parenteral nutrition without substitution of thiamine demonstrated an acute haemorrhagic encephalopathy. The lesions differed substantially from the classic features of thiamine deficient encephalopathy regarding the histopathological alterations and the topographical distribution. The extreme rapidity of thiamine deprivation may have been responsible for the abrupt clinical onset of the disease and the intensity of the morphological alterations.
Dietary deprivation of thiamine combined with pyrithiamine administration in rats was used for pathophysiological and morphological investigations. The animals passed through three different symptomatic stages, ranging from slight neurological abnormalities to generalized seizures from day 8 up to day 11. Hypothermia was a consistent finding during the second week. Histological examination revealed two types of neuropathological lesions in the rats. Those in the colliculi inferiores and the vestibular nuclei were characterized by a bullous spongiform appearance of the neuropil with severely damaged, pale and oedematous nerve cells. Alterations in the thalamus and inferior olives, however, showed eosinophilic nerve cell necrosis of the ischemic type which resembles the thalamic pathology found in human cases of Wernicke's encephalopathy.
Formalin-fixed paraffin-embedded material of 158 diffuse malignant pleural mesotheliomas (DMPMs) was used in order to determine the differential diagnostic value of immunocytochemical probes against 9 different antigens. While vimentin expression was found in only 50% of cases, regardless of their histological subtype all tumours were found to be cytokeratin-positive when an antibody with broad-spectrum cytokeratin reactivity was used. Conversely, none of the cases was immunostained by antisera against carcinoembryonic antigen (CEA), Leu-M1 antigen, chromogranin, S-100 protein, lysozyme and a T-cell associated antigen. The density of inflammatory cell infiltrates reactive with antisera against the three latter antigens was not associated with the clinical behaviour of the neoplasms examined. Eight DMPM cases showed immunoreactivity with HEA-antibodies against Egp 34, an antigen previously supposed only to be expressed by carcinomas. On the basis of these findings, the consistent cytokeratin reactivity, also of the sarcomatous type of DMPM, may help to exclude metastatic involvement of the pleura by a mesenchymal neoplasm of other origin. CEA and Leu-M1 staining of a given pleural tumour, on the other hand, is indicative of a carcinoma secondarily afflicting the pleura, thus making the diagnosis of primary DMPM unlikely.
In order to test the hypothesis that Wernicke's encephalopathy is of topographic rather than of pathogenetic specificity we examined the brains of 49 patients without any evidence of chronic alcoholism. They had died at least four days after an event of severe hypoxia-ischemia. They all showed extensive lesions in the cortex, in the thalamus and in other regions. In 19 of them there was additional necrosis in the mamillary bodies which apparently was of the same age as the associated cortical and thalamic lesions and which could not be distinguished from Wernicke's encephalopathy. In three of the 19 cases there was a total necrosis within the mamillary bodies. By re-examining the mamillary bodies of 12 known alcoholics without any evidence for an ischemic impact we could affirm that total necrosis may fit into the spectrum of Wernicke's encephalopathy. Our findings demonstrate that the morphological changes in the mamillary bodies due to thiamine deficiency and those due to hypoxia-ischemia may be identical.
The authors report a case of a congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus within a few days after birth. The tumor was well demonstrated by sonography, CT, and MR; the preoperative diagnosis was histologically confirmed after subtotal resection.
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A major obstacle to apply molecular biologic techniques effectively to the somatic genetic analysis of neoplastic tissue is the presence of abundant nonneoplastic elements in the analyzed specimen. These nonneoplastic elements, including reactive fibrous cells, vascular cells, and a variety of infiltrating white blood cells, may mask genetic alterations that otherwise would be easily detectable if the neoplastic cells were procured selectively. We have developed a microdissection technique which allows to selectively procure and genetically analyze small populations of neoplastic cells from the glass slide. In this report, we describe the microdissection technique and genetic analysis of archival frozen and paraffin-embedded tissue in general. Then, we review some implications of this approach of genetic analysis for mapping and cloning of new genes, identification of premalignant lesions, and differential analysis of different histologic components within the same tumor.