[Posner-Schlossman syndrome].
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Biomedical subjects
Publications and source records attributed to C Thomas.
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Two cases of pleomorphic xanthoastrocytoma (PXA) involving the temporal lobe are reported, both occurring in adolescents. This neoplasma is a recently described, rare variant of astrocytoma affecting the superficial cerebral cortex and meninges of children and young adults. The tumor is considered to arise from the subpial astrocytes of the superficial cortex. Electron microscopic examination and immunoperoxidase stains for glial fibrillary acidic protein (GFAP) are helpful in making a definitive histologic diagnosis. Despite its pleomorphic appearance, this type of supratentorial glioma appears to have a much better prognosis than other tumors from which it must be distinguished, such as high grade gliomas and meningeal sarcomas. In contrast to malignant gliomas, the pleomorphic xanthoastrocytoma does not appear to require aggressive postoperative radiation therapy or chemotherapy. Therefore, it is important to recognize and identify this type of glioma as a distinct entity.
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We report a kindred of French/Alsatian origin with symptoms of Gerstmann-Sträussler-Scheinker disease over 3 generations. In the propositus, cerebellar signs and memory disturbance were the presenting features, followed by other neurological manifestations. Biopsy of the cerebral cortex showed numerous multicentric and "kuru"-type amyloid plaques that on immuno-light and electron microscopy stained with antibody to prion protein. Molecular genetic analysis revealed an A117V mutation in the open reading frame of the prion protein gene. Questions as to pathology and spread of this mutation are discussed.
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Hepatitis C virus types were investigated by using samples from eight sero-reactive and PCR positive patients attending our Hemodialysis Unit en Paysandú, Uruguay. After HCV RNA detection by reverse transcription and polymerase chain reaction, HCV genotyping was carried out by a nested PCR amplification, using type specific primers of HCV core region. These results were confirmed using a method based upon reverse hybridation of amplified products by enzyme-labeled type-specific probes to portions of the 5' UTR region. HCV genotypes were assigned according to Simmonds' classification. Type 1b was found in five patients, type 3a was found in one and one patient was not classified. There was a patient who became PCR negative at the moment the genotyping was carried out.