37 results on '"Masters, C L."'
Search Results
2. 11C-PiB PET studies in typical sporadic Creutzfeldt-Jakob disease.
- Author
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Villemagne VL, McLean CA, Reardon K, Boyd A, Lewis V, Klug G, Jones G, Baxendale D, Masters CL, Rowe CC, and Collins SJ
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- Aged, Aniline Compounds, Brain pathology, Brain Chemistry physiology, Codon genetics, Creutzfeldt-Jakob Syndrome metabolism, Creutzfeldt-Jakob Syndrome pathology, Fatal Outcome, Female, Fluorodeoxyglucose F18, Humans, Immunohistochemistry, Magnetic Resonance Imaging, Middle Aged, Positron-Emission Tomography, Radiopharmaceuticals, Thiazoles, Benzothiazoles, Creutzfeldt-Jakob Syndrome diagnostic imaging
- Abstract
Objective: Brain amyloid imaging using positron emission tomography (PET) is of increasing importance in the premortem evaluation of dementias, particularly in relation to Alzheimer disease (AD). The purpose of this study was to explore the premortem diagnostic utility of (11)C-PiB PET in sporadic Creutzfeldt-Jakob disease (CJD)., Methods: Two patients, 72 and 59 years old, underwent evaluation for rapidly progressive cognitive decline, dying after illness durations of 5 and 7 months, respectively. As part of their comprehensive assessment, (18)F-FDG PET and (11)C-PiB PET studies were performed approximately 2-4 weeks prior to death, and the brain regional distributions compared with those from cohorts of healthy controls (HC) and AD patients., Results: Routine investigations, including brain MRI scans, revealed changes typical of sporadic CJD, with the diagnosis confirmed at autopsy in both patients. The (18)F-FDG PET showed global hypometabolism in one patient and thalamic and frontal hypometabolism with unexpected hypermetabolism in the dentate nuclei of the cerebellum in the other. Neither patient displayed cerebral cortical (11)C-PiB PET retention above the levels observed in HC., Conclusions: No grey-matter (11)C-PiB retention was observed in two pathologically confirmed cases of typical sporadic CJD. We speculate that low PrP plaque density and small plaque size, as well as a relatively low affinity of the radioligand, explain the absence of (11)C-PiB retention. More studies to validate this hypothesis are warranted.
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- 2009
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3. Novel prion protein gene mutation presenting with subacute PSP-like syndrome.
- Author
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Rowe DB, Lewis V, Needham M, Rodriguez M, Boyd A, McLean C, Roberts H, Masters CL, and Collins SJ
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- Creutzfeldt-Jakob Syndrome diagnosis, Diagnosis, Differential, Female, Humans, Middle Aged, Supranuclear Palsy, Progressive diagnosis, Syndrome, Creutzfeldt-Jakob Syndrome genetics, Mutation, Prions genetics, Supranuclear Palsy, Progressive genetics
- Abstract
A 62-year-old Indonesian woman presenting with a progressive supranuclear palsy-like syndrome was confirmed post mortem as dying from a spongiform encephalopathy. Despite an illness duration of only 4 months, brain MRI, EEG, and CSF analysis for 14-3-3 proteins all failed to disclose changes typical of Creutzfeldt-Jakob disease. Neuropathologic examination revealed multicentric, prion protein-positive, amyloid plaques as typically seen in Gerstmann-Sträussler-Scheinker syndrome. Prion protein gene analysis revealed a previously unreported A133V mutation.
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- 2007
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4. Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease.
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Collins SJ, Sanchez-Juan P, Masters CL, Klug GM, van Duijn C, Poleggi A, Pocchiari M, Almonti S, Cuadrado-Corrales N, de Pedro-Cuesta J, Budka H, Gelpi E, Glatzel M, Tolnay M, Hewer E, Zerr I, Heinemann U, Kretszchmar HA, Jansen GH, Olsen E, Mitrova E, Alpérovitch A, Brandel JP, Mackenzie J, Murray K, and Will RG
- Subjects
- 14-3-3 Proteins cerebrospinal fluid, Adult, Age of Onset, Aged, Aged, 80 and over, Brain pathology, Creutzfeldt-Jakob Syndrome cerebrospinal fluid, Creutzfeldt-Jakob Syndrome genetics, Electroencephalography methods, Female, Genotype, Humans, Magnetic Resonance Imaging methods, Male, Middle Aged, Polymorphism, Genetic genetics, Prion Proteins, Prions genetics, Protein Precursors genetics, Sensitivity and Specificity, Time Factors, Creutzfeldt-Jakob Syndrome diagnosis
- Abstract
To validate the provisional findings of a number of smaller studies and explore additional determinants of characteristic diagnostic investigation results across the entire clinical spectrum of sporadic Creutzfeldt-Jakob disease (CJD), an international collaborative study was undertaken comprising 2451 pathologically confirmed (definite) patients. We assessed the influence of age at disease onset, illness duration, prion protein gene (PRNP) codon 129 polymorphism (either methionine or valine) and molecular sub-type on the diagnostic sensitivity of EEG, cerebral MRI and the CSF 14-3-3 immunoassay. For EEG and CSF 14-3-3 protein detection, we also assessed the influence of the time point in a patient's illness at which the investigation was performed on the likelihood of a typical or positive result. Analysis included a large subset of patients (n = 743) in whom molecular sub-typing had been performed using a combination of the PRNP codon 129 polymorphism and the form of protease resistant prion protein [type 1 or 2 according to Parchi et al. (Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar H. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46: 224-233.)] present in the brain. Findings for the whole group paralleled the subset with molecular sub-typing data available, showing that age at disease onset and disease duration were independent determinants of typical changes on EEG, while illness duration significantly influenced positive CSF 14-3-3 protein detection; changes on brain MRI were not influenced by either of these clinical parameters, but overall, imaging data were less complete and consequently conclusions are more tentative. In addition to age at disease onset and illness duration, molecular sub-type was re-affirmed as an important independent determinant of investigation results. In multivariate analyses that included molecular sub-type, time point of the investigation during a patient's illness was found not to influence the occurrence of a typical or positive EEG or CSF 14-3-3 protein result. A typical EEG was most often seen in MM1 patients and was significantly less likely in the MV1, MV2 and VV2 sub-types, whereas VV2 patients had an increased likelihood of a typical brain MRI. Overall, the CSF 14-3-3 immunoassay was the most frequently positive investigation (88.1%) but performed significantly less well in the very uncommon MV2 and MM2 sub-types. Our findings confirm a number of determinants of principal investigation results in sporadic CJD and underscore the importance of recognizing these pre-test limitations before accepting the diagnosis excluded or confirmed. Combinations of investigations offer the best chance of detection, especially for the less common molecular sub-types such as MV2 and MM2.
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- 2006
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5. CSF BACE1 activity is increased in CJD and Alzheimer disease versus [corrected] other dementias.
- Author
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Holsinger RM, Lee JS, Boyd A, Masters CL, and Collins SJ
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- Aged, Alzheimer Disease epidemiology, Amyloid Precursor Protein Secretases, Aspartic Acid Endopeptidases, Australia epidemiology, Biomarkers cerebrospinal fluid, Creutzfeldt-Jakob Syndrome epidemiology, Dementia cerebrospinal fluid, Dementia diagnosis, Dementia epidemiology, Diagnosis, Differential, Enzyme Activation, Female, Humans, Male, Reproducibility of Results, Risk Assessment methods, Risk Factors, Sensitivity and Specificity, tau Proteins, 14-3-3 Proteins cerebrospinal fluid, Alzheimer Disease cerebrospinal fluid, Alzheimer Disease diagnosis, Creutzfeldt-Jakob Syndrome cerebrospinal fluid, Creutzfeldt-Jakob Syndrome diagnosis, Endopeptidases cerebrospinal fluid, Nerve Tissue Proteins cerebrospinal fluid
- Abstract
To assess the diagnostic utility of CSF BACE1 activity for discriminating Alzheimer disease (AD) from other dementias, particularly Creutzfeldt-Jakob disease (CJD), the authors studied 26 patients with sporadic CJD, 21 patients with AD, and 21 patients with various non-AD, non-CJD dementias (DCs). CSF BACE1 activity was elevated in AD in comparison with DC (p = 0.01). Unexpectedly, CSF BACE1 activity was also increased in sporadic CJD (p = 0.02).
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- 2006
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6. Ethical considerations in presymptomatic testing for variant CJD.
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Duncan RE, Delatycki MB, Collins SJ, Boyd A, Masters CL, and Savulescu J
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- Animals, Cattle, Clinical Protocols, Creutzfeldt-Jakob Syndrome transmission, Decontamination methods, Disease Outbreaks, Genetic Predisposition to Disease, HIV Infections diagnosis, Humans, Huntington Disease diagnosis, Huntington Disease genetics, Prions, Zoonoses, Creutzfeldt-Jakob Syndrome diagnosis, Ethics, Clinical
- Abstract
Variant Creutzfeldt-Jakob disease (vCJD) is a fatal, transmissible, neurodegenerative disorder for which there is currently no effective treatment. vCJD arose from the zoonotic spread of bovine spongiform encephalopathy. There is now compelling evidence for human to human transmission through blood transfusions from presymptomatic carriers and experts are warning that the real epidemic may be yet to come. Imperatives exist for the development of reliable, non-invasive presymptomatic diagnostic tests. Research into such tests is well advanced. In this article the ethical implications of the availability of these tests are elaborated and comparisons drawn with predictive genetic testing for Huntington's disease and screening for HIV. Paramount to considerations is the issue of whom to test, weighing up respect for personal autonomy against obligations to benefit and protect society. A paradigm is proposed similar to that used for HIV screening but with unique features: compulsory testing of all blood/organ donors and individuals undergoing surgery or invasive procedures who have a significant risk of disease transmission.
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- 2005
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7. Australian sporadic CJD analysis supports endogenous determinants of molecular-clinical profiles.
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Lewis V, Hill AF, Klug GM, Boyd A, Masters CL, and Collins SJ
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- Adult, Aged, Aged, 80 and over, Australia epidemiology, Blotting, Western, Brain metabolism, Brain pathology, Brain physiopathology, Codon genetics, Creutzfeldt-Jakob Syndrome epidemiology, Female, Genotype, Geography, Humans, Male, Middle Aged, Neurons metabolism, Neurons pathology, Phenotype, PrPSc Proteins chemistry, Registries statistics & numerical data, Creutzfeldt-Jakob Syndrome classification, Creutzfeldt-Jakob Syndrome genetics, Genetic Variation genetics, PrPSc Proteins genetics
- Abstract
Objective: To define the protease-resistant prion protein (PrPres) types and associated clinical profiles in Australian patients with sporadic Creutzfeldt-Jakob disease (CJD) to allow comparison with those reported from other continents and concomitantly reaffirm absence of variant CJD (vCJD)., Methods: Reassessment of available clinical and neuropathologic data on patients referred to the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) who died between January 1, 1992, and June 30, 2003, was conducted. Molecular classification of PrPres was determined by immunoblot analysis of available frozen brain tissue. Brain homogenate pH and codon 129 genotype on the prion protein gene (PRNP) were established., Results: PrPres patterns in 35 of 37 patients with sporadic CJD conformed to one of three common reported types. Of a range of clinical features assessed, illness duration was the only clinical feature significantly associated with PrPres type. Two patients displayed coexistence of more than one PrP type, with one displaying a novel pattern of three PrPres types in a single brain region. The absence of vCJD was reconfirmed, supported by the lack of the typical PrPres glycoform pattern., Conclusions: Given Australia's geographic isolation and environmental uniqueness, the general congruity of these results with those reported from other continents suggests that endogenous factors predominantly determine sporadic Creutzfeldt-Jakob disease (CJD) phenotypic subtypes or "strains." These results support a clinicopathologic classification system whereby both PrPres type and codon 129 genotype are utilized to most accurately depict phenotypic subtypes or strains of sporadic CJD.
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- 2005
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8. Apparently sporadic CJD and covert health-care transmissions.
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Lewis V, Boyd A, Masters CL, and Collins SJ
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- Case-Control Studies, Humans, Risk Factors, Creutzfeldt-Jakob Syndrome etiology, Creutzfeldt-Jakob Syndrome transmission, Iatrogenic Disease, Surgical Procedures, Operative adverse effects
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- 2002
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9. Creutzfeldt-Jakob disease in Australia 1970-1999.
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Collins S, Boyd A, Lee JS, Lewis V, Fletcher A, McLean CA, Law M, Kaldor J, Smith MJ, and Masters CL
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- Adult, Aged, Aged, 80 and over, Australia epidemiology, Emigration and Immigration statistics & numerical data, Female, Humans, Incidence, Male, Middle Aged, Prospective Studies, Registries, Retrospective Studies, Sentinel Surveillance, Creutzfeldt-Jakob Syndrome epidemiology
- Abstract
Objective: To ascertain all persons who developed a transmissible spongiform encephalopathy (TSE) within Australia during the 30-year period 1970 to 1999 through a comprehensive national surveillance program and subject the group to detailed epidemiologic analysis., Methods: Cases were ascertained through reviews of morbidity separation coding data from all university-affiliated tertiary referral hospitals, as well as the centralized data bases of state and territory health departments, regular national death certificate searches, and semiannual mailout questionnaires to all neurologists and pathologists throughout Australia. Prospective monitoring commenced in September 1993., Results: A total of 387 patients were confirmed as having TSE during this epoch. The majority of cases were sporadic Creutzfeldt-Jakob disease (CJD) (90.7%), with 7.2% heredofamilial and 2.1% iatrogenic. Over this 30-year period, the national average annual sporadic CJD incidence rate per million progressively increased from 0.31 for the decade 1970 through 1979 to 0.77 for 1980 through 1989, reaching 1.03 for 1990 through 1999. Death certificates were found to have a false-positive rate of 11.5% and sensitivity of 83.0% for sporadic CJD., Conclusions: Within Australia, there has been a gradual increase in the incidence of transmissible spongiform encephalopathy over the three-decade period 1970 through 1999, peaking in 1999 at 1.4/million/year for sporadic Creutzfeldt-Jakob disease. This increase is believed secondary to improved case ascertainment. Variant Creutzfeldt-Jakob disease was not identified during this period. Age- and sex-adjusted comparisons showed a decline in incidence rates in the elderly in both sexes, usually from age 74 years. Death certificates were a useful but imperfect method of case detection.
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- 2002
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10. Transmissible spongiform encephalopathies in Australia.
- Author
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Boyd A, Fletcher A, Lee JS, Lewis V, Masters CL, and Collins SJ
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- Animals, Australia epidemiology, Cattle, Encephalopathy, Bovine Spongiform epidemiology, Encephalopathy, Bovine Spongiform transmission, Humans, Occupational Exposure, Population Surveillance, Prion Diseases classification, Prion Diseases epidemiology, Prion Diseases transmission, Registries, Risk Factors, Creutzfeldt-Jakob Syndrome epidemiology, Creutzfeldt-Jakob Syndrome transmission
- Abstract
The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) commenced surveillance in September 1993 as part of the Commonwealth's response to 4 cases of pituitary hormone (gonadotrophin)-associated Creutzfeldt-Jakob disease (CJD). With the passage of time, the Registry has become responsible for ascertaining all human transmissible spongiform encephalopathies (TSE; also known as prion diseases) within Australia since 1970. Included in the spectrum of diseases monitored are classical (sporadic, genetic, and health care acquired) CJD, and variant CJD (vCJD), first reported in 1996 in the United Kingdom. Variant CJD has not yet been diagnosed in Australia. Final classification of persons with suspected human prion disease is based upon all available clinical, investigational and pathological information. Ascertainment methods are diverse and include prompted, half-yearly personal communications from neurologists and neuropathologists, death certificate searches, and morbidity separation coding searches of major hospital, and State and Territory databases. More recently, referral for diagnostic CSF 14-3-3 protein testing (performed by the ANCJDR) has considerably increased prospective notifications of suspect cases. As at September 2001 there were 460 cases on the register; 237 definite cases, 168 probable and 55 incomplete cases awaiting final classification.
- Published
- 2001
11. The emerging European epidemic of variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy: lessons for Australia.
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Masters CL
- Subjects
- Animals, Australia epidemiology, Cattle, Encephalopathy, Bovine Spongiform epidemiology, Europe epidemiology, Humans, Creutzfeldt-Jakob Syndrome epidemiology, Creutzfeldt-Jakob Syndrome prevention & control, Disease Outbreaks prevention & control, Encephalopathy, Bovine Spongiform transmission
- Published
- 2001
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12. Novel prion protein gene mutation in an octogenarian with Creutzfeldt-Jakob disease.
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Collins S, Boyd A, Fletcher A, Byron K, Harper C, McLean CA, and Masters CL
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- 14-3-3 Proteins, Aged, Aged, 80 and over, Confusion etiology, Creutzfeldt-Jakob Syndrome diagnosis, DNA Mutational Analysis, Electroencephalography, Fatal Outcome, Female, Humans, Mutation, Occipital Lobe pathology, Prions cerebrospinal fluid, Proteins analysis, Creutzfeldt-Jakob Syndrome genetics, Prions genetics, Tyrosine 3-Monooxygenase
- Abstract
Background: The transmissible spongiform encephalopathies constitute a fascinating and biologically unique group of invariably fatal neurodegenerative disorders that affect both animals and humans. Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia represent the more common human phenotypes. Excluding the small number of iatrogenically transmitted cases, approximately 85% to 90% of patients develop CJD without identifiable explanation, with an increasing number of different mutations in the prion protein gene (PRNP) recognized as probably causative in the remainder., Objective: To report on an 82-year-old woman with pathologically confirmed CJD found unexpectedly to harbor a novel mutation in PRNP., Methods: Routine clinical investigations were undertaken to elucidate the cause of the rapidly progressive dementia and neurological decline manifested by the patient, including magnetic resonance imaging of the brain, electroencephalography, and cerebrospinal fluid analysis for the 14-3-3 beta protein. Standard postmortem neuropathological examination of the brain was performed, including immunocytochemistry of representative sections to detect the prion protein. Posthumous genetic analysis of the open reading frame of PRNP was performed on frozen brain tissue using polymerase chain reaction and direct sequencing., Results: Concomitant with the exclusion of alternative diagnoses, the presence of characteristic periodic sharp-wave complexes on the electroencephalogram in combination with a positive result for 14-3-3 beta protein in the cerebrospinal fluid led to a confident clinical diagnosis of CJD, confirmed at autopsy. There was no family history of dementia or similar neurological illness, but patrilineal medical information was incomplete. Unexpectedly, full sequencing of the PRNP open reading frame revealed a single novel mutation consisting of an adenine-to-guanine substitution at nucleotide 611, causing alanine to replace threonine at codon 188., Conclusions: In addition to expanding the range of PRNP mutations associated with human prion diseases, we believe this case is important for the following reasons. First, from an epidemiological perspective, the avoidance of occasional incorrect classification of patients manifesting neurodegenerative disorders that may have a genetic basis requires systematic genotyping, particularly when there are uncertainties regarding the family history. Second, the incidence of spongiform encephalopathy in elderly patients beyond the typical age range may be underestimated and does not preclude a genetic basis. Finally, as a corollary, this case highlights problematic issues in human transmissible spongiform encephalopathies, as illustrated by disease penetrance and age of onset in genotype-phenotype correlations.
- Published
- 2000
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13. Creutzfeldt-Jakob disease: diagnostic utility of 14-3-3 protein immunodetection in cerebrospinal fluid.
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Collins S, Boyd A, Fletcher A, Gonzales M, McLean CA, Byron K, and Masters CL
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- 14-3-3 Proteins, Adult, Aged, Aged, 80 and over, Biomarkers cerebrospinal fluid, Blotting, Western, Diagnosis, Differential, False Negative Reactions, False Positive Reactions, Female, Humans, Male, Middle Aged, Predictive Value of Tests, Prospective Studies, Sensitivity and Specificity, Cerebrospinal Fluid Proteins cerebrospinal fluid, Creutzfeldt-Jakob Syndrome cerebrospinal fluid, Creutzfeldt-Jakob Syndrome diagnosis, Proteins metabolism, Tyrosine 3-Monooxygenase
- Abstract
With the aim of improving the pre-mortem diagnostic accuracy of sporadic Creutzfeldt-Jakob disease (CJD), there has been considerable recent interest in the merit of immunodetecting 14-3-3 proteins in the cerebrospinal fluid (CSF) using Western blotting, with cumulative support for the utility of this technique. As a corollary, during a 20 month period, CSF samples from an unselected prospective series of 124 patients in whom sporadic CJD was a differential diagnostic possibility were examined by the Australian Creutzfeldt-Jakob disease Registry (ACJDR) for the presence of 14-3-3 proteins. Follow up to achieve a final diagnosis or clinical outcome was successful in 119. For definite and probable sporadic CJD combined, a positive result was 91.4% sensitive, while the sensitivity for the pathologically verified group alone was 96.0%. A negative outcome was 92.5% specific with false positive results seen in five patients with diagnoses which included inflammatory CNS disorders, cerebral ischaemia and dementia with Lewy bodies (DLB). Immunodetectable 14-3-3 proteins were present in three of four symptomatic patients with prion protein gene (PRNP) mutations. CSF samples containing significant amounts of blood were confirmed as suboptimal, with weak or qualitatively unusual positive results found in greater than 50% of such specimens, with only one of 14 such cases ultimately classified as definite or probable CJD., (Copyright 2000 Harcourt Publishers Ltd.)
- Published
- 2000
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14. Recent advances in the pre-mortem diagnosis of Creutzfeldt-Jakob disease.
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Collins S, Boyd A, Fletcher A, Gonzales MF, McLean CA, and Masters CL
- Subjects
- Biomarkers cerebrospinal fluid, Biopsy, Brain diagnostic imaging, Brain pathology, Brain physiopathology, Creutzfeldt-Jakob Syndrome diagnostic imaging, Creutzfeldt-Jakob Syndrome pathology, Creutzfeldt-Jakob Syndrome physiopathology, Diagnosis, Differential, Electroencephalography, Humans, Magnetic Resonance Imaging, Palatine Tonsil pathology, Prions cerebrospinal fluid, Tomography, Emission-Computed, Tomography, Emission-Computed, Single-Photon, Creutzfeldt-Jakob Syndrome diagnosis
- Abstract
Included in the spectrum of human transmissible spongiform encephalopathies are Creutzfeldt-Jakob disease (CJD) and the new variant form (vCJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, kuru and various less distinct neuropsychiatric disorders. Progress in our understanding of this group of disorders continues at a prodigious rate, although important vexing practical issues persist. The definitive confirmation of symptomatic prion disease still requires pathological examination, most reliably performed post-mortem. However, paralleling the recent advances in the molecular biological understanding of normal prion protein (PrP(c)) function and the pathophysiology of prion diseases, there have been worthwhile developments in the pre-mortem diagnosis of CJD. Efforts to develop less invasive but very reliable ante-mortem diagnostic tests have received an additional impetus because of the potential epidemic of vCJD. Historically, the ancillary investigation of most merit has been the EEG, whereas the recent advances have encompassed a broader range of technologies, including both magnetic resonance and radioisotopic neuroimaging, and immunoassays for a range of non-specific marker proteins in both CSF, and less commonly, blood. However, given the recent refinement of sophisticated immunoassays, it is envisaged that the pathognomonic, protease-resistant, disease-associated isoforms of the prion protein (PrPres) may soon be directly detectable in the blood and tissues of patients manifesting or incubating a spongiform encephalopathy., (Copyright 2000 Harcourt Publishers Ltd.)
- Published
- 2000
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15. Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study.
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Collins S, Law MG, Fletcher A, Boyd A, Kaldor J, and Masters CL
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- Adolescent, Adult, Aged, Australia epidemiology, Case-Control Studies, Creutzfeldt-Jakob Syndrome etiology, Environmental Exposure, Female, Humans, Logistic Models, Male, Middle Aged, Occupational Exposure, Registries, Risk Factors, Transfusion Reaction, Creutzfeldt-Jakob Syndrome epidemiology, Postoperative Complications
- Abstract
Background: Apart from the small number of iatrogenic and familial cases, the cause of most cases of Creutzfeldt-Jakob disease (CJD) is not known. We aimed to identify risk factors for sporadic CJD., Methods: In a case-control study, we compared the medical history and selected demographic characteristics of 241 definite (neuropathologically confirmed) and probable (clinically likely) patients with CJD, ascertained from the Australian National Creutzfeldt-Jakob Disease Registry between Jan 1, 1970, and October 31, 1997, and of 784 controls, recruited from the community by random telephone interview in August, 1997. Standard logistic regression was used for the comparisons., Findings: Surgical procedures were significantly associated with the development of sporadic CJD. This risk progressively increased with the number of surgical treatments to a maximum for three procedures (odds ratio 2.13 [95% Cl 1.34-3.41], p=0.002). There was also a significant association between risk of CJD and residence or employment on a farm (p<0.001) or market garden (p=0.002) for longer than 10 years. We found no significant risk associated with a history of blood transfusion, organ transplantation, major dental work, or occupation., Interpretation: Our findings accord with the hypothesis that a range of surgical treatments may serve as unrecognised contamination events and account for a proportion of cases of sporadic CJD. Possible biases in different methods and times for the acquisition of data on cases and controls suggest our findings need to be replicated in independent studies with community controls.
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- 1999
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16. Comparative neuropathology of Kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host.
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McLean CA, Ironside JW, Alpers MP, Brown PW, Cervenakova L, Anderson RM, and Masters CL
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- Adolescent, Adult, Creutzfeldt-Jakob Syndrome genetics, DNA analysis, Female, Genotype, Humans, Immunoenzyme Techniques, Kuru genetics, Male, Middle Aged, Creutzfeldt-Jakob Syndrome pathology, Kuru pathology, Prions genetics
- Abstract
The three major influences on the phenotype of the transmissible spongiform encephalopathies are believed to be strain of agent, route of infection and host genotype. We have compared the pathologic profiles and genotypes of the new variant of Creutzfeldt-Jakob disease (vCJD) and kuru. The comparison reveals that there are distinct lesional differences particularly in the prion protein (PrP) load and distribution as seen by immunohistochemistry. The clinico-pathologic phenotypes and the genotypes of these two diseases are sufficiently different to suggest that the strain of agent may play a greater role than any presumptive common route of peripherally acquired infection.
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- 1998
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17. Iatrogenic and zoonotic Creutzfeldt-Jakob disease: the Australian perspective.
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Collins S and Masters CL
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- Animals, Creutzfeldt-Jakob Syndrome genetics, Dura Mater transplantation, Humans, Pituitary Hormones therapeutic use, Postoperative Complications, Zoonoses transmission, Creutzfeldt-Jakob Syndrome transmission, Iatrogenic Disease
- Abstract
The transmissible brain diseases of humans and animals, the spongiform encephalopathies, continue to stimulate interest, and the announcement that exposure to "mad cow disease" (bovine spongiform encephalopathy [BSE]) is a possible explanation for more than 10 cases of a variant Creutzfeldt-Jakob disease in humans in the United Kingdom is a recent example. Cases of iatrogenic Creutzfeldt-Jakob disease (from previous use of human cadaveric tissues for pituitary hormone therapy and neurosurgical grafts) are still being identified, and the unique nosological status of this group of disorders-that they are both transmissible and inherited and that the only known component of their infectious agent is protein-alone makes these diseases remarkable.
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- 1996
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18. Transmissibility of Creutzfeldt-Jakob disease and related disorders.
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Collins SJ and Masters CL
- Subjects
- Animals, Creutzfeldt-Jakob Syndrome transmission, DNA Mutational Analysis, Humans, Iatrogenic Disease, Molecular Biology, Prion Diseases transmission, Prions genetics, Risk Factors, Creutzfeldt-Jakob Syndrome genetics, Prion Diseases genetics
- Abstract
The spongiform encephalopathies occur in both animals and humans, with kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome, and Fatal Familial Insomnia constituting the currently recognised spectrum of such disorders in man. All have proven to be transmissible, including familial cases. Important determinants of transmissibility include the route of inoculation (intracerebral is most effective) and the titre of the inoculated material (brain and spinal cord have greatest infectivity). However, in familial cases, the pathogenic mutations in the PrP gene additionally influence the efficacy of transmission. Recent progress in the molecular biological basis of these diseases suggests that most of the infectivity resides in an abnormal, relatively protease resistant, isoform of the constitutively expressed PrP. The abnormal isoform is postulated to serve as a template for auto-catalytic polymerisation and the consequent deposition of amyloid. Extensions of this hypothesis attempt to reconcile the paradox of how these disorders can be both infectious and also inherited in an autosomal dominant fashion.
- Published
- 1995
19. The nosology of Creutzfeldt-Jakob disease and conditions related to the accumulation of PrPCJD in the nervous system.
- Author
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Richardson EP Jr and Masters CL
- Subjects
- Brain metabolism, DNA Mutational Analysis, Female, Gerstmann-Straussler-Scheinker Disease classification, Gerstmann-Straussler-Scheinker Disease genetics, Gerstmann-Straussler-Scheinker Disease pathology, History, 20th Century, Humans, Male, PrPSc Proteins metabolism, Prion Diseases genetics, Prion Diseases history, Prion Diseases pathology, Brain pathology, Creutzfeldt-Jakob Syndrome classification, Creutzfeldt-Jakob Syndrome genetics, Creutzfeldt-Jakob Syndrome history, Creutzfeldt-Jakob Syndrome pathology, PrPSc Proteins genetics, Prion Diseases classification
- Abstract
Although typical cases of Creutzfeldt-Jakob disease are readily recognized pathologically and clinically, variant forms often pose a diagnostic challenge. From the 1920's, when this disease was first characterized, until quite recently diagnosis relied heavily on morphologic changes. New advances in immunoassays and PrP gene analysis now provide important adjuncts in recognizing the spectrum of disorders of PrP metabolism associated with these transmissible encephalopathies.
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- 1995
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20. Creutzfeldt-Jakob disease and slow infections: a review.
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Irving WL, Crimmins DS, Masters CL, and Cunningham AL
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- Humans, Creutzfeldt-Jakob Syndrome diagnosis, Creutzfeldt-Jakob Syndrome epidemiology, Creutzfeldt-Jakob Syndrome transmission, Slow Virus Diseases diagnosis, Slow Virus Diseases epidemiology, Slow Virus Diseases transmission
- Published
- 1990
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21. Neuropathology of unconventional virus infections: molecular pathology of spongiform change and amyloid plaque deposition.
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Masters CL and Beyreuther K
- Subjects
- Alzheimer Disease complications, Amyloid classification, Amyloidosis complications, Amyloidosis metabolism, Creutzfeldt-Jakob Syndrome complications, Humans, Kuru complications, Alzheimer Disease pathology, Amyloid biosynthesis, Amyloidosis pathology, Brain pathology, Creutzfeldt-Jakob Syndrome pathology, Kuru pathology
- Abstract
To the triad of neuronal loss, gliosis and spongiform change as characteristic morphological changes associated with infection of the central nervous system, one can now add the presence of scrapie-associated filaments (SAF)/PrP rods. While the host's immune response is conspicuous by its absence, the vigorous astrocytic response is presumptive evidence of the host's ability to recognize and respond to the primary neuronal insult. We assume that the spongiform change and vacuolation of neurons are of fundamental importance in the pathogenesis of the disease, realizing that neither is specific or essential for the replication of the infectious agent. The topographical distribution of lesions is partly explained by the portal of entry and retrograde spread of the virus. The temporal progression of the lesions is more clearly determined by the host genes, best illustrated by studies of the incubation period. The molecular basis of the spongiform change is unknown but it is presumed to involve some disturbance of membrane metabolism. The recognition of PrP as a membrane glycoprotein invites proposals for its role in the development of these spongiform lesions. Extracellular amyloid occurs as plaques or congophilic angiopathy in some instances, and provides the best evidence that Alzheimer's disease (AD) is in some way related to the unconventional virus diseases. However, the protein subunit (A4) of the amyloid fibril in AD and its precursor are quite distinct from the PrP subunit which constitutes the amyloid fibril in these infectious diseases. It is still unclear whether the PrP subunit in the SAF has exactly the same composition as in the extracellular amyloid fibril. Our results suggest that only a fragment of the PrP molecule is the major constituent of the extracellular fibril. Since both PrP and A4 are derived from membrane glycoproteins, the elucidation of their normal function is likely to lead to a better understanding of the spongiform and amyloidogenic lesions in these diseases.
- Published
- 1988
- Full Text
- View/download PDF
22. Transmissible dementias: current problems in tissue handling.
- Author
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Baringer JR, Gajdusek DC, Gibbs CJ Jr, Masters CL, Stern WE, and Terry RD
- Subjects
- Brain surgery, Electroencephalography, Electromyography, Humans, Specimen Handling, Alzheimer Disease transmission, Creutzfeldt-Jakob Syndrome transmission, Dementia transmission
- Published
- 1980
- Full Text
- View/download PDF
23. Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
- Author
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Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, and Gajdusek DC
- Subjects
- Animals, Brain microbiology, Cricetinae, Female, Goats, Humans, Kidney microbiology, Pan troglodytes, Saimiri, Sheep, Spleen microbiology, Creutzfeldt-Jakob Syndrome transmission, Eating, Kuru transmission, Scrapie transmission
- Abstract
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
- Published
- 1980
- Full Text
- View/download PDF
24. Rapidly progressive dementia with ataxia.
- Author
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Harper CG, Day TJ, and Masters CL
- Subjects
- Aged, Ataxia pathology, Dementia pathology, Electroencephalography, Female, Humans, Neurologic Examination, Brain pathology, Creutzfeldt-Jakob Syndrome pathology
- Published
- 1986
- Full Text
- View/download PDF
25. [Experimental transmission of Creutzfeldt-Jakob disease to the guinea pig (author's transl)].
- Author
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Gálvez S, Gajdusek DC, and Masters CL
- Subjects
- Animals, Creutzfeldt-Jakob Syndrome pathology, Female, Guinea Pigs, Humans, Middle Aged, Saimiri, Cerebral Cortex pathology, Creutzfeldt-Jakob Syndrome transmission
- Published
- 1980
26. Transmissible spongiform encephalopathy (Creutzfeldt-Jakob disease). Atypical clinical and pathological findings.
- Author
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Schoene WC, Masters CL, Gibbs CJ Jr, Gajdusek DC, Tyler HR, Moore FD, and Dammin GJ
- Subjects
- Brain Stem pathology, Ethmoid Bone pathology, Humans, Male, Middle Aged, Paresthesia etiology, Skin pathology, Sleep Wake Disorders etiology, Spinal Cord pathology, Sural Nerve pathology, Creutzfeldt-Jakob Syndrome pathology
- Abstract
A middle-aged neurosurgeon had an 18-month illness characterized by abnormal sleep patterns, paresthesias, and necrotizing cutaneous lesions with vasculitis and signs of cerebral, brainstem, vestibulocerebellar, and progressive spinal cord involvement. Biopsy specimens of nerve and skin showed an acute vasculitis with endovascular cellular proliferation in the pattern of a Köhlmeier-Degos lesion and focal epidermal necrosis. Mental changes and cranial-nerve signs developed. Myoclonus occurred occasionally during sleep. Akinetic mutism ensued. At autopsy, major abnormalities were limited to the nervous system and skin. Spongiform encephalopathy typical of Creutzfeldt-Jakob disease was found with amyloid kuru plaques. A cribriform change distinct from the spongiform change was seen focally in the white matter. Scarred skin lesions and a healed, partially obliterative arteritis were noted. Inoculation of brain and lung into nonhuman primates resulted in a spongiform encephalopathy.
- Published
- 1981
- Full Text
- View/download PDF
27. Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change.
- Author
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Masters CL and Richardson EP Jr
- Subjects
- Adult, Aged, Cerebellum pathology, Cerebral Cortex pathology, Corpus Striatum pathology, Female, Gliosis pathology, Hippocampus pathology, Humans, Male, Medulla Oblongata pathology, Middle Aged, Nerve Degeneration, Pons pathology, Spinal Cord pathology, Telencephalon pathology, Thalamic Nuclei pathology, Brain pathology, Creutzfeldt-Jakob Syndrome pathology
- Published
- 1978
- Full Text
- View/download PDF
28. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering.
- Author
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Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, and Asher DM
- Subjects
- Adolescent, Adult, Age Factors, Aged, Animals, Boston, Creutzfeldt-Jakob Syndrome genetics, Creutzfeldt-Jakob Syndrome transmission, Female, Humans, Male, Middle Aged, Occupations, Retrospective Studies, Scrapie epidemiology, Sex Factors, Sheep, Space-Time Clustering, Creutzfeldt-Jakob Syndrome epidemiology
- Abstract
The worldwide epidemiology of Creutzfeldt-Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal-spatial clustering of cases was not found in the United States, but reports from other countries indicate that such clustering does occur. Fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection. Iatrogenic transmission by corneal transplantation and neurosurgical operations has occurred, and the possibility is raised that previous surgery or preexisting neurological operations has occurred, and the possibility is raised that previous surgery or preexisting neurological disease may be associated with an increased risk of developing CJD. It remains to be determined whether the virus of CJD is maintained only by patient-to-patient transmission, has a zoonotic reservoir such as scrapie, or causes widespread latent infection of man that is occasionally activated.
- Published
- 1979
- Full Text
- View/download PDF
29. The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer's disease.
- Author
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Masters CL, Gajdusek DC, and Gibbs CJ Jr
- Subjects
- Adult, Aged, Aging, Alzheimer Disease complications, Alzheimer Disease etiology, Alzheimer Disease transmission, Creutzfeldt-Jakob Syndrome complications, Creutzfeldt-Jakob Syndrome transmission, Female, Humans, Male, Middle Aged, Pedigree, Alzheimer Disease genetics, Creutzfeldt-Jakob Syndrome genetics, Dementia genetics
- Abstract
We have analysed the familial occurrence of Creutzfeldt-Jakob disease (CJD) in 27 families selected from a total of 73 families. Fifteen per cent of all cases of CJD have a family history of disease consistent with autosomal dominant transmission. The onset of disease in familial cases is significantly earlier than in sporadic cases. A maternal effect was not found, nor was there evidence for prenatal vertical transmission of the virus. Temporal and spatial separations between affected members demonstrates that incubation periods ranging at least from one to four decades are to be expected. Affected siblings tend to die at the same age, and not at the same time, which is consistent with some form of vertical transmission (either prenatal or early postnatal), assuming rather uniform incubation periods. CJD occurred in four families in members related by marriage, evidence in favour of horizontal or common source transmission in occasional cases. The familial occurrence of CJD and Alzheimer's disease (AD) were compared using data on 52 families with AD. The age at death and duration of disease in familial AD is greater than in familial CJD. Familial AD also occurs in a pattern of autosomal dominant transmission, without maternal effect. There were four families with AD in which one or more members died from CJD. There were an additional 17 families with AD in which one or more members presented with clinical features resembling CJD. Although virus causing an experimental spongiform encephalopathy was isolated from the brain of two cases of familial AD, most cases of sporadic and familial AD tested failed to cause disease when brain tissue was inoculated into nonhuman primates. The precise mechanism of spread of the virus in familial CJD remains unknown. The results of the present study are consistent with the hypothesis of a genetically inherited susceptibility to infection which is acquired in early infancy or childhood. Other proposed mechanisms such as prenatal vertical transmission or a common environmental source of infection seem less likely.
- Published
- 1981
- Full Text
- View/download PDF
30. Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease.
- Author
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Salazar AM, Masters CL, Gajdusek DC, and Gibbs CJ Jr
- Subjects
- Adult, Aged, Basal Ganglia Diseases pathology, Brain pathology, Creutzfeldt-Jakob Syndrome transmission, Female, Humans, Male, Middle Aged, Myelin Sheath ultrastructure, Neurofibrils ultrastructure, Spinal Cord pathology, Amyotrophic Lateral Sclerosis pathology, Creutzfeldt-Jakob Syndrome pathology, Dementia pathology
- Abstract
A review of over 2,000 cases of Creutzfeldt-Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical-pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt-Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt-Jakob disease and do not deserve the label of "amyotrophic Creutzfeldt-Jakob disease." When lower motor neuron involvement occurs in transmissible Creutzfeldt-Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.
- Published
- 1983
- Full Text
- View/download PDF
31. Preclinical lesions and their progression in the experimental spongiform encephalopathies (kuru and Creutzfeldt-Jakob disease) in primates.
- Author
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Masters CL, Kakulas BA, Alpers MP, Gajdusek DC, and Gibbs CJ Jr
- Subjects
- Animals, Astrocytes pathology, Brain Stem pathology, Cerebellum pathology, Cerebral Cortex pathology, Corpus Striatum pathology, Disease Models, Animal, Haplorhini, Hippocampus pathology, Humans, Neurons pathology, Primates, Time Factors, Brain pathology, Creutzfeldt-Jakob Syndrome pathology, Kuru pathology
- Abstract
Creutzfeldt-Jakob disease and kuru were studied in experimental primates. Eight animals with clinical disease lasting from 1/2 to 12 1/2 months were evaluated for histological evidence of progression of the pathological triad of neuronal vacuolation, neuronal loss and fibrous astrocytosis. The first change to appear was neuronal vacuolation, in both the body of neurones and in the neuropil. Fibrous astrocytosis was found subsequent to neuronal damage and necrosis. Neuronal loss was apparent when clinical signs were present. As the clinical disease progressed, so did the severity of neuronal loss and astrocytosis. Five animals, 1 1/2-10 1/2 months after intracerebral inoculation, before they had shown any signs of clinical disease, had histological evidence of neuronal vacuolation and astrocytosis.
- Published
- 1976
- Full Text
- View/download PDF
32. Golgi and electronmicroscopic studies of spongiform encephalopathy.
- Author
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Landis DM, Williams RS, and Masters CL
- Subjects
- Adult, Animals, Astrocytes ultrastructure, Axons ultrastructure, Cerebral Cortex pathology, Cricetinae, Dendrites pathology, Dendrites ultrastructure, Humans, In Vitro Techniques, Male, Middle Aged, Neurons pathology, Neurons ultrastructure, Scrapie pathology, Sheep, Staining and Labeling, Cerebral Cortex ultrastructure, Creutzfeldt-Jakob Syndrome pathology
- Abstract
Golgi impregnations of cerebral biopsies from two patients suffering from Creutzfeldt-Jakob disease (subacute spongiform encephalopathy) revealed striking loss of dendritic spines of pyramidal neurons and unusual focal spherical distensions of dendritic and axonal processes. Light and electronmicroscopic studies disclosed spongiform changes, which seemed to be caused by intracytoplasmic vacuoles and expansion of the plasmalemmal membranes of neurons and astrocytes. Although the diagnostic biopsies were performed at a markedly symptomatic stage of the disease, there was no evidence of neuronal cell loss. Neuronal changes in Golgi impregnations of cerebral cortex from hamsters infected with scrapie were essentially identical to those in the human biopsies. The loss of dendritic spines of pyramidal cells and spherical swellings of axons and dendrites thus seem to be characteristic of spongiform encephalopathies, and probably account in part for the clinical neurologic manifestations, which may be severe in the relative absence of neuronal death.
- Published
- 1981
- Full Text
- View/download PDF
33. Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies.
- Author
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Masters CL, Gajdusek DC, and Gibbs CJ Jr
- Subjects
- Adult, Aged, Cerebellar Ataxia complications, Cerebellar Ataxia pathology, Cerebellar Diseases genetics, Cerebellar Diseases transmission, Dementia etiology, Female, History, 20th Century, Humans, Kuru pathology, Male, Middle Aged, Time Factors, Viral Plaque Assay, Amyloidosis etiology, Cerebellar Ataxia microbiology, Creutzfeldt-Jakob Syndrome microbiology, Viruses, Unclassified isolation & purification
- Published
- 1981
- Full Text
- View/download PDF
34. Creutzfeldt-Jakob disease: its origins.
- Author
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Masters CL
- Subjects
- History, 20th Century, Creutzfeldt-Jakob Syndrome
- Published
- 1989
- Full Text
- View/download PDF
35. Decontamination of formaldehyde-fixed cerebral tissues of Creutzfeldt-Jakob disease by autoclaving, without the loss of microscopic visual quality.
- Author
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Masters CL, Jacobsen P, and Kakulas BA
- Subjects
- Humans, Microbiological Techniques, Brain pathology, Creutzfeldt-Jakob Syndrome pathology
- Published
- 1985
36. Creutzfeldt-Jakob disease in an adolescent.
- Author
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Monreal J, Collins GH, Masters CL, Fisher CM, Kim RC, Gibbs CJ Jr, and Gajdusek DC
- Subjects
- Adolescent, Cerebral Cortex pathology, Corpus Striatum pathology, Creutzfeldt-Jakob Syndrome diagnosis, Follow-Up Studies, Humans, Male, Brain pathology, Creutzfeldt-Jakob Syndrome pathology
- Abstract
A 16-year-old boy was stricken with a progressive neurologic disorder characterized primarily by dementia progressing to severe neurologic debility in 12 months and death 28 months following the first symptoms. Pathologic examination showed a spongiform encephalopathy, consistent witha clinical diagnosis of Creutzfeldt-Jakob disease (CJD). The noteworthy features of the case are the age of onset, the somewhat prolonged course an the amount of white matter change. These are discussed within the frame of reference of CJD and the spongiform encephalopathies of infancy and childhood. Animal inoculation studies employing post-mortem embalmed brain as inoculum are currently in progress to determine the transmissibility of this patient's disease.
- Published
- 1981
- Full Text
- View/download PDF
37. Experimental kuru in the gibbon and sooty mangabey and Creutzfeldt-Jakob disease in the pigtailed macaque. With a summary of the host range of the subacute spongiform virus encephalopathies.
- Author
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Masters CL, Alpers MP, Gajdusek DC, Gibbs CJ Jr, and Kakulas BA
- Subjects
- Animals, Creutzfeldt-Jakob Syndrome transmission, Haplorhini, Humans, Kuru transmission, Slow Virus Diseases transmission, Creutzfeldt-Jakob Syndrome veterinary, Hominidae, Hylobates, Kuru veterinary, Macaca, Monkey Diseases transmission, Slow Virus Diseases veterinary
- Abstract
The experimental host range for the slow virus infections causing subacute spongiform virus encephalopathies is enlarged in primates to include the gibbon for kuru, the pigtailed macaque for Creutzfeldt-Jakob disease, and the sooty mangabey for both diseases. The report is based on neuropathological evidence of the diseases in animals with preclinical lesions. A table lists all the species to which the subacute spongiform virus encephalopathies have been transmitted.
- Published
- 1976
- Full Text
- View/download PDF
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