Journal ArticleDOI
Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease.
Inga Zerr,Maurizio Pocchiari,Steven J. Collins,J.-P. Brandel,J de Pedro Cuesta,Richard Knight,H. Bernheimer,Franco Cardone,N. Delasnerie-Laupretre,N. Cuadrado Corrales,Anna Ladogana,Monika Bodemer,Ashley Fletcher,T. Awan,A. Ruiz Bremón,Herbert Budka,Jean-Louis Laplanche,Robert G. Will,Sigrid Poser +18 more
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TLDR
In this paper, a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoassay were conducted.Abstract:
Objective: To improve diagnostic criteria for sporadic Creutzfeldt–Jakob disease (CJD). Methods: Pooled data on initial and final diagnostic classification of suspected CJD patients were accumulated, including results of investigations derived from a coordinated multinational study of CJD. Prospective analysis for a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoassay were conducted. Results: Data on 1,003 patients with suspected CJD were collected using a standard questionnaire. After follow-up was carried out, complete clinical data and neuropathologic diagnoses were available in 805 cases. In these patients, the sensitivity of the detection of periodic sharp wave complexes in the EEG was 66%, with a specificity of 74%. The detection of 14-3-3 proteins in the CSF correlated with the clinical diagnosis in 94% (sensitivity). The specificity (84%) was higher than that of EEG. A combination of both investigations further increased the sensitivity but decreased the specificity. Conclusions: Incorporation of CSF 14-3-3 analysis in the diagnostic criteria for CJD significantly increases the sensitivity of case definition. Amended diagnostic criteria for CJD are proposed.read more
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Journal ArticleDOI
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Inga Zerr,Kai Kallenberg,David Summers,C. Romero,Ana Lia Taratuto,Uta Heinemann,Maren Breithaupt,Daniela Varges,Bettina Meissner,Anna Ladogana,Maaike Schuur,Stéphane Haïk,Steven J. Collins,Gerard H. Jansen,G. B. Stokin,José Pimentel,Ekkehard Hewer,D. Collie,Pete Smith,H. Roberts,Jean-Philippe Brandel,C M van Duijn,Maurizio Pocchiari,C. Begue,Patrick Cras,Robert G. Will,Pascual Sánchez-Juan +26 more
TL;DR: Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt–Jakob disease.
Journal ArticleDOI
Recommendations for the diagnosis and management of Alzheimer's disease and other disorders associated with dementia: EFNS guideline.
Gunhild Waldemar,Bruno Dubois,Murat Emre,Jean Georges,Ian G. McKeith,Martin N. Rossor,Philip Scheltens,P. Tariska,Bengt Winblad +8 more
TL;DR: This international guideline on dementia covers major aspects of diagnostic evaluation and treatment, with particular emphasis on the type of patient often referred to the specialist physician, and revised the recommendations for clinical diagnosis, blood tests, neuroimaging, electroencephalography, and cerebrospinal fluid analysis.
Journal ArticleDOI
Sporadic and familial CJD: classification and characterisation
TL;DR: This review attempts to classify and characterise sporadic and familial Creutzfeldt-Jakob disease (CJD) as a function of these two disease determinants: PrP genotype and PrP(Sc) type.
Journal ArticleDOI
Iatrogenic Creutzfeldt-Jakob disease at the millennium.
Patrick O. Brown,Michael A. Preece,J.-P. Brandel,Takeshi Sato,Lisa M. McShane,I. Zerr,Ashley Fletcher,Robert G. Will,Maurizio Pocchiari,Neil R. Cashman,J.H. d’Aignaux,Larisa Cervenakova,J. Fradkin,Lawrence B. Schonberger,Steven J. Collins +14 more
TL;DR: Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.
Journal ArticleDOI
Genetic prion disease: the EUROCJD experience.
Gabor G. Kovacs,Maria Puopolo,Anna Ladogana,Maurizio Pocchiari,Herbert Budka,Cornelia M. van Duijn,Steven J. Collins,Alison Boyd,Antonio Giulivi,Mike Coulthart,N. Delasnerie-Laupretre,Jean Philippe Brandel,Inga Zerr,Hans A. Kretzschmar,Jesús de Pedro-Cuesta,Miguel Calero-Lara,Markus Glatzel,Adriano Aguzzi,Matthew Bishop,Richard Knight,Girma Belay,Robert G. Will,Eva Mitrova +22 more
TL;DR: The authors' results show that clinicopathological phenotypes include genetic Creutzfeldt–Jakob disease (gCJD), fatal familial insomnia (FFI), and Gerstmann–Sträussler–Scheinker disease (GSS).
References
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Journal ArticleDOI
Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects
Piero Parchi,Armin Giese,Sabina Capellari,Paul Brown,Walter J. Schulz-Schaeffer,Otto Windl,Inga Zerr,Herbert Budka,Nicolas Kopp,Pedro Piccardo,Sigrid Poser,Amyn M. Rojiani,Nathalie Streichemberger,Jean Julien,Claude Vital,Bernardino Ghetti,Pierluigi Gambetti,Hans A. Kretzschmar +17 more
TL;DR: The present data demonstrate the existence of six phenotypic variants of sCJD, and the physicochemical properties of PrPSc in conjunction with the PRNP codon 129 genotype largely determine this phenotypesic variability, and allow a molecular classification of the disease variants.
Journal ArticleDOI
Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease
Paul D. Brown,C. J. Gibbs,Pamela Rodgers-Johnson,D. M. Asher,Sulima Mp,A. Bacote,L. G. Goldfarb,D. C. Gajdusek +7 more
TL;DR: Incubation periods as well as the durations and character of illness showed great variability, even in animals receiving the same inoculum, mirroring the spectrum of clinical profiles seen in human disease.
Journal ArticleDOI
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples
Andrew F. Hill,RJ Butterworth,Susan Joiner,Graham S. Jackson,Martin N. Rossor,Dafydd Thomas,Adam Frosh,N Tolley,JE Bell,Michael D. Spencer,Andrew T. King,S Al-Sarraj,James W. Ironside,Peter L. Lantos,John Collinge +14 more
TL;DR: If, in the appropriate clinical context, a tonsil biopsy sample was positive for PrPSc, variant CJD could be diagnosed, which obviates the need for a brainBiopsy sample to be taken, and the results show that variants CJD has a different pathogenesis to sporadic CJD.
Journal ArticleDOI
The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies.
TL;DR: In patients with dementia, a positive immunoassay for the 14-3-3 brain protein in cerebrospinal fluid strongly supports a diagnosis of Creutzfeldt-Jakob disease, but this finding does not support the use of the test in patients without clinically evident dementia.
Journal ArticleDOI
Creutzfeldt‐Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering
TL;DR: The worldwide epidemiology of Creutzfeldt‐Jakob disease (CJD) is presented from an analysis of 1,435 patients, and fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection.
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Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects
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