Results 51 to 60 of about 35,863 (248)

Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993–2002

BMC Public Health, 2006
Background The objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries. Methods From data collected for surveillance purposes, we describe annual proportions of deaths due to ...
Jansen Gerard H, Lewis Victoria, Collins Steven, Sanchez-Juan Pascual, Van Duijn Cornelia, Alpérovitch Annick, Delasnerie-Lauprêtre Nicole, Brandel Jean-Philippe, Kretszchmar Hans A, Zerr Inga, Pocchiari Maurizio, Puopolo Maria, Mellina Vittorio, Stoeck Katharina, Almazán Javier, Glatzel Markus, de Pedro-Cuesta Jesús, Coulthart Michael B, Gelpi Ellen, Budka Herbert, Mitrova Eva   +20 more
doaj   +1 more source

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease [PDF]

, 2017
Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype.
Begue, C., Brandel, J. P., Breithaupt, M., Collie, D., Collins, S. J., Cras, P., Haik, S., Heinemann, U., Hewer, E., Jansen, Gerard H., Kallenberg, K., Ladogana, A., Meissner, B., Pimentel, J., Pocchiari, M., Roberts, H., Romero, C., Sanchez-Juan, P., Schuur, M., Smith, P., Stokin, G. B., Summers, D. M., Taratuto, A., van Duijn, C., Varges, D., Will, R. G., Zerr, I.   +26 more
core  

Creutzfeldt-Jakob disease and homocysteine levels in plasma and cerebrospinal fluid [PDF]

, 2005
Background: There is evidence that homocysteine contributes to various neurodegenerative disorders. Objective: To assess the values of homocysteine in patients with Creutzfeldt-Jakob disease (CJD) in both cerebrospinal fluid (CSF) and plasma.
Bottiglieri T, Halliwell B, Hans A. Kretzschmar, Inga Zerr, Jens Wiltfang, Markus Otto, Otto M, Stefan Bleich, Stefan Kropp, Ubbink JB, Zerr I, Zerr I   +11 more
core   +1 more source

ZNRD2 Mediated Nucleoprotein Aggregation Impairs Respiratory Syncytial Virus Replication

Advanced Science, EarlyView.
During RSV infection, nucleoprotein (N) forms RNA‐bound oligomers. The host protein ZNRD2 binds to these oligomers, promoting their transition into insoluble aggregates. These aggregates simultaneously sequester functional N to restrict viral production and disrupt chaperonin assembly quality control by interfering with ZNRD2's role as an adaptor ...
Haiwu Zhou, Mingbin He, Jinhong Du, Cong Liu, Weiwei Wang, Yuewen Han, Zhifei Li, Yali Qin, Mingzhou Chen   +8 more
wiley   +1 more source

The human spongiform encephalopathies [PDF]

Romanian Journal of Neurology, 2018
The human spongiform encephalopathies are a group of heterogenous, usually fatal diseases, characterized by a unique pathogenetic mechanism and distinct clinical presentation. They are classified into sporadic, familial and acquired forms.
Mavroudis Ioannis, Petrides Foivos, Kazis Dimitrios   +2 more
doaj   +1 more source

Significant association of a M129V independent polymorphism in the 5\prime UTR of the PRNP gene with sporadic Creutzfeldt-Jakob disease in a large German case-control study [PDF]

, 2006
Background: A single nucleotide polymorphism (SNP) in the coding region of the prion protein gene (PRNP) at codon 129 has been repeatedly shown to be an associated factor to sporadic Creutzfeldt-Jakob disease (sCJD), but additional major predisposing DNA
Bickeböller, H, group, the KORA, Illig, T, Kretzschmar, H A, Rosenberger, A, Vollmert, C, Wichmann, H‐E, Windl, O, Xiang, W, Zerr, I   +9 more
core   +2 more sources

Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6

Annals of Neurology, EarlyView.
Objective An enduring puzzle in many inherited neurological disorders is the late onset of symptoms despite expression of function‐impairing mutant protein early in life. We examined the basis for onset of impairment in spinocerebellar ataxia type 6 (SCA6), a canonical late‐onset neurodegenerative ataxia which results from a polyglutamine expansion in ...
Haoran Huang, Taylor L. Charron, Min Fu, Miranda Dunn, Deborah M. Jones, Praveen Kumar, Satoshi Ishishita, Allan‐Hermann Pool, Ashwinikumar Kulkarni, Genevieve Konopka, Vikram G. Shakkottai   +10 more
wiley   +1 more source

THE CLINICAL CASE OF PRIMARY PROGRESSIVE APHASIA

Учёные записки Санкт-Петербургского государственного медицинского университета им. Акад. И.П. Павлова, 2019
The article described the rare clinical case of the patient with probable transmissible encephalopathy (Creutzfeldt–Jakob disease) in whom rapidly forming primary progressive aphasia was the herald clinical feature, and motor and sensory functions were ...
Natalya V. Shuleshova, Aleksey V. Sizov, Irina V. Kupriianova, Victoria V. Zvereva   +3 more
doaj   +1 more source

Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years [PDF]

, 2017
To date, cerebrospinal fluid analysis, particularly protein 14-3-3 testing, presents an important approach in the identification of Creutzfeldt-Jakob disease cases.
Aguzzi, Adriano, Calero, Miguel, Gawinecka, Joanna, Green, Alison, Karch, Andre, Knight, Richard, Kulczycki, Jerzy, Ladogana, Anna, Laplanche, Jean-Louis, Mitrova, Eva, Peoc'h, Katell, Pocchiari, Maurizio, Saiz, Albert, Sanchez-Juan, Pascual, Sanchez-Valle, Raquel, Schelzke, Gabi, Sklaviadis, Theodor, Slivarichova, Dana, Stoeck, Katharina, van Duijn, Cornelia M., Zerr, Inga   +20 more
core  

The Phenotypic Spectrum of Sporadic Creutzfeldt‐Jakob Disease Cortical Subtype

Annals of Neurology, EarlyView.
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Simone Baiardi, Claudia Marina Vargiu, Brian S. Appleby, Marcelo Barria, Giuseppe Mario Bentivenga, Ignazio Calì, Benedetta Carlà, Mark Cohen, Armin Giese, Jochen Herms, Aino‐Minerva Kortelainen, Anna Ladogana, Angela Mammana, Diane Ritchie, Otto Windl, Sabina Capellari, Piero Parchi   +16 more
wiley   +1 more source