Biomedical subjects
J Ironside
Publications and source records attributed to J Ironside.
Type of prion protein in UK farmers with Creutzfeldt-Jakob disease.
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Molecular assessment of the potential transmissibilities of BSE and scrapie to humans.
More than a million cattle infected with bovine spongiform encephalopathy (BSE) may have entered the human food chain. Fears that BSE might transmit to man were raised when atypical cases of Creutzfeldt-Jakob disease (CJD), a human transmissible spongiform encephalopathy (TSE), emerged in the UK. In BSE and other TSE diseases, the conversion of the protease-sensitive host prion protein (PrP-sen) to a protease-resistant isoform (PrP-res) is an important event in pathogenesis. Biological aspects of TSE diseases are reflected in the specificities of in vitro PrP conversion reactions. Here we show that there is a correlation between in vitro conversion efficiencies and known transmissibilities of BSE, sheep scrapie and CJD. On this basis, we used an in vitro system to gauge the potential transmissibility of scrapie and BSE to humans. We found limited conversion of human PrP-sen to PrP-res driven by PrP-res associated with both scrapie (PrP[Sc]) and BSE (PrP[BSE]). The efficiencies of these heterologous conversion reactions were similar but much lower than those of relevant homologous conversions. Thus the inherent ability of these infectious agents of BSE and scrapie to affect humans following equivalent exposure may be finite but similarly low.
Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy.
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Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD.
Strains of transmissible spongiform encephalopathies are distinguished by differing physicochemical properties of PrPSc, the disease-related isoform of prion protein, which can be maintained on transmission to transgenic mice. 'New variant' Creutzfeldt-Jakob disease (CJD) has strain characteristics distinct from other types of CJD and which resemble those of BSE transmitted to mice, domestic cat and macaque, consistent with BSE being the source of this new disease. Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype.
Squamous cell carcinoma of the eyelid and the acquired immunodeficiency syndrome.
PURPOSE: We studied a case of squamous cell carcinoma of the eyelid in a 42-year-old homosexual man with the acquired immunodeficiency syndrome (AIDS) to explore the link between squamous cell carcinoma and human Papillomavirus infection. METHOD: A rapidly growing, 3-mm lesion was removed from the right lower eyelid by excisional biopsy. RESULTS: Examination of the lesion confirmed squamous cell carcinoma. Polymerase chain reaction for human Papillomavirus 16 was positive. CONCLUSION: This case suggests a role for human Papillomavirus infection in the cause of squamous cell carcinoma in AIDS patients.
Metachronous lymphoma of the breast and conjunctiva.
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Transmission of fatal familial insomnia to laboratory animals.
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Intracerebral distribution of infectious amyloid protein in spongiform encephalopathy.
We studied the regional distribution of infectious amyloid protein by western immunoblots of brain tissue extracts from 37 patients with different forms of spongiform encephalopathy, i.e., 16 sporadic cases, 18 familial cases with a variety of mutations, and 3 iatrogenic cases. In sporadic and familial Creutzfeldt-Jakob disease, amyloid protein concentrations were usually highest in the frontotemporal regions of the cerebral cortex, whereas iatrogenic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome had as high or higher concentrations in the deep cerebral nuclei and cerebellum. As a group, familial cases had lower amyloid protein concentrations than either sporadic or iatrogenic cases, and fatal familial insomnia patients had the lowest concentrations found in any form of disease. This hierarchy of amyloid protein concentrations corresponds to the experimental transmission rates observed for each form of disease and is consistent with the concept that the protein molecule is an integral component of the infectious agent. Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy.
Acquired immunodeficiency syndrome-related primary central nervous system lymphoma.
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Progressive paraparesis, lymphocytic meningitis, and stroke in a 37-year-old man.
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A case of Creutzfeldt-Jakob disease presenting with cortical deafness.
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Teaching image-guided stereotactic methodology and functional neuroanatomy of the thalamus and pallidum: a simple ex vivo technique.
A simple and cheap model that enables on site, ex vivo, but very practical, learning of frame-based image-guided stereotactic technique and methodology, and the functional anatomy of the pallidum and thalamus is described. Using a cadaver skull, a specially prepared, formalin-fixed cadaver brain, and a modified stereotactic probe application of a stereotactic frame to the cranium, fiducial point acquisition, target point acquisition, computation of both arc and probe depth settings, and verification of target point accuracy can all be practiced. If diencephalic targets are selected for targeting with ball bearings then section of the cadaver brains, and study of a human thalamic stereotactic atlas provides an excellent and clinically relevant method of learning functionally important thalamic and pallidal anatomy. The method and techniques are described for CT imaging using the Brown-Roberts-Wells frame, but they are equally applicable to other frame types.
Stereotaxic brain biopsy in AIDS patients: does it contribute to patient management?
The authors analysed all cases of brain biopsy done in AIDS or HIV+patients at the Department of Clinical Neurosciences, Western General Hospital, Edinburgh, during the period 1989-92 to determine the clinical utility of the procedure. Thirteen lesions were biopsied revealing toxoplasmosis in five, lymphoma in four, progressive multifocal leucoencephalopathy in three, HIV encephalitis in two and encephalitis of unknown aetiology in one. One biopsy specimen contained abnormal tissue but was non-diagnostic. The median survival after biopsy was only 7 weeks, but preoperative immunological (median CD4 count 45) and clinical status of the patients were poor. Although the impact of tissue diagnosis on patient survival was minimal it did enable modification of drug therapy. The authors preliminary experience with biopsy in AIDS patients suggests that the diagnostic information obtained is of limited value in prolonging the lives of patients in poor immunological status, but may be useful in refining medical therapies for toxoplasmosis and progressive multifocal leucoencephalopathy. Whether biopsy should be performed earlier in the course of cerebral disease in an effort to prolong survival time remains unclear.
Nucleolus organiser regions on mitotic and meiotic chromosomes from infertile males investigated using a specific silver stain.
Mitotic preparations from 30 subfertile males and meiotic preparations from 3 normal and 2 subfertile males were examined by means of the Ag-I technique of Bloom and Goodpasture (1976) to reveal nucleolus organiser regions (NORs). In the mitotic preparations, each subject was found to have a characteristic number of Ag-positive NORs per cell, within a range of 6--10. Analysis of satellite associations showed that the mean number of satellite associations per cell was related to the modal number of Ag-positive NORs for each subject. In the meiotic preparations, silver deposition was observed throughout meiotic prophase, but disappeared totally during diakinesis and metaphase II. It was seen again in early spermatids, and disappeared again as nuclear elongation took place. This pattern was observed in both normal and subfertile subjects, and may provide indirect evidence for the activation of rRNA genes during spermatogenesis.
Proceedings: Viral and bacterial antibodies in primary biliary cirrhosis and other chronic liver disease.
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