Published in Ann Neurol on November 01, 2004
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Diagnostic value of CSF protein profile in a Portuguese population of sCJD patients. J Neurol (2009) 0.94
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The EEG in E200K familial CJD: relation to MRI patterns. J Neurol (2011) 0.83
A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease. BMC Med Genet (2011) 0.81
Detailed electroencephalographic long-term follow-up study in Lewy body dementia with periodic sharp wave complexes. J Neurol (2007) 0.80
Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles. J Neurol (2008) 0.80
Clinical overlap between Jakob-Creutzfeldt disease and Lewy body disease. Can J Neurol Sci (2012) 0.79
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No evidence for association between tau gene haplotypic variants and susceptibility to Creutzfeldt-Jakob disease. BMC Med Genet (2007) 0.77
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Re: Creutzfeldt-Jacob disease misdiagnosed as dementia with Lewy bodies. J Neurol (2006) 0.75
Creutzfeldt-Jakob Disease-Like Periodic Sharp Wave Complexes in Voltage-Gated Potassium Channel-Complex Antibodies Encephalitis: A Case Report. J Clin Neurophysiol (2016) 0.75
Related or not? Development of spontaneous Creutzfeldt-Jakob disease in a patient with chronic, well-controlled HIV: A case report and review of the literature. SAGE Open Med Case Rep (2016) 0.75
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Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis. Exp Ther Med (2017) 0.75
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (2013) 6.34
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol (2007) 5.02
A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain (2009) 4.40
α-Synuclein and tau concentrations in cerebrospinal fluid of patients presenting with parkinsonism: a cohort study. Lancet Neurol (2011) 3.12
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol (2007) 2.97
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies. J Neuropathol Exp Neurol (2008) 2.96
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol (2009) 2.76
Mutations in the colony stimulating factor 1 receptor (CSF1R) gene cause hereditary diffuse leukoencephalopathy with spheroids. Nat Genet (2011) 2.76
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol (2009) 2.68
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
Misfolded proteinase K-resistant hyperphosphorylated alpha-synuclein in aged transgenic mice with locomotor deterioration and in human alpha-synucleinopathies. J Clin Invest (2002) 2.47
Direct quantification of CSF alpha-synuclein by ELISA and first cross-sectional study in patients with neurodegeneration. Exp Neurol (2008) 2.31
ALDH1A1 is a marker of astrocytic differentiation during brain development and correlates with better survival in glioblastoma patients. Brain Pathol (2012) 2.14
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol (2008) 2.02
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions. J Neuropathol Exp Neurol (2007) 2.02
Synapse formation and function is modulated by the amyloid precursor protein. J Neurosci (2006) 1.99
Hyperphosphorylation and insolubility of alpha-synuclein in transgenic mouse oligodendrocytes. EMBO Rep (2002) 1.91
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins. Acta Neuropathol (2013) 1.82
LPS receptor (CD14): a receptor for phagocytosis of Alzheimer's amyloid peptide. Brain (2005) 1.81
The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication. EMBO J (2011) 1.80
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol (2008) 1.78
FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations. Brain (2011) 1.76
Intratumoral homogeneity of MGMT promoter hypermethylation as demonstrated in serial stereotactic specimens from anaplastic astrocytomas and glioblastomas. Int J Cancer (2007) 1.67
Screening of innate immune receptors in neurodegenerative diseases: a similar pattern. Neurobiol Aging (2007) 1.62
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations. Acta Neuropathol (2013) 1.59
Interleukin 4 and interleukin 10 levels are elevated in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. Arch Neurol (2005) 1.53
Identification of differentially expressed genes in scrapie-infected mouse brains by using global gene expression technology. J Virol (2004) 1.50
Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J Biol Chem (2004) 1.50
Beta-amlyoid 1-42 and tau-protein in cerebrospinal fluid of patients with Parkinson's disease dementia. Dement Geriatr Cogn Disord (2006) 1.49
FET PET for the evaluation of untreated gliomas: correlation of FET uptake and uptake kinetics with tumour grading. Eur J Nucl Med Mol Imaging (2007) 1.47
Different apolipoprotein E, apolipoprotein A1 and prostaglandin-H2 D-isomerase levels in cerebrospinal fluid of schizophrenia patients and healthy controls. World J Biol Psychiatry (2010) 1.45
Increase in CD230 (cellular prion protein) fluorescence on blood lymphocytes in bovine spongiform encephalopathy-infected nonhuman primates. Transfusion (2009) 1.41
Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem (2008) 1.40
The Hedgehog receptor Patched controls lymphoid lineage commitment. Blood (2007) 1.35
Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD. Brain (2006) 1.35
Gamma-secretase inhibition reduces spine density in vivo via an amyloid precursor protein-dependent pathway. J Neurosci (2009) 1.35
Chloroquine activates the p53 pathway and induces apoptosis in human glioma cells. Neuro Oncol (2010) 1.33
Upregulation of cellular prion protein (PrPc) after focal cerebral ischemia and influence of lesion severity. Neurosci Lett (2004) 1.33
Analysis of 18F-FET PET for grading of recurrent gliomas: is evaluation of uptake kinetics superior to standard methods? J Nucl Med (2006) 1.33
O-methylguanine-DNA methyltransferase (MGMT) mRNA expression predicts outcome in malignant glioma independent of MGMT promoter methylation. PLoS One (2011) 1.32
Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol (2009) 1.32
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype. Neurobiol Aging (2012) 1.30
Antitumor effects of a combined 5-aza-2'deoxycytidine and valproic acid treatment on rhabdomyosarcoma and medulloblastoma in Ptch mutant mice. Cancer Res (2009) 1.30
Early detection of abnormal prion protein in genetic human prion diseases now possible using real-time QUIC assay. PLoS One (2013) 1.30
Low values of 5-hydroxymethylcytosine (5hmC), the "sixth base," are associated with anaplasia in human brain tumors. Int J Cancer (2012) 1.30
Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol (2012) 1.29
Deletion of cellular prion protein results in reduced Akt activation, enhanced postischemic caspase-3 activation, and exacerbation of ischemic brain injury. Stroke (2006) 1.29
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Arch Neurol (2008) 1.28
Expression of integrin alphavbeta3 in gliomas correlates with tumor grade and is not restricted to tumor vasculature. Brain Pathol (2008) 1.28
Imaging of integrin alpha(v)beta(3) expression in patients with malignant glioma by [18F] Galacto-RGD positron emission tomography. Neuro Oncol (2009) 1.27
Assessment of beta-amyloid deposits in human brain: a study of the BrainNet Europe Consortium. Acta Neuropathol (2009) 1.27
Age-dependent cognitive decline and amygdala pathology in alpha-synuclein transgenic mice. Neurobiol Aging (2006) 1.26
Distinct pathological subtypes of FTLD-FUS. Acta Neuropathol (2010) 1.26
The role of the octarepeat region in neuroprotective function of the cellular prion protein. Brain Pathol (2007) 1.25
Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years. Brain (2012) 1.24
Expression of FoxM1 is required for the proliferation of medulloblastoma cells and indicates worse survival of patients. Clin Cancer Res (2011) 1.23
Preclinical deposition of pathological prion protein PrPSc in muscles of hamsters orally exposed to scrapie. J Clin Invest (2004) 1.21
Cerebrospinal fluid biomarkers in human genetic transmissible spongiform encephalopathies. J Neurol (2009) 1.21
Pathological properties of the Parkinson's disease-associated protein DJ-1 in alpha-synucleinopathies and tauopathies: relevance for multiple system atrophy and Pick's disease. Acta Neuropathol (2004) 1.19
Generation of genuine prion infectivity by serial PMCA. Vet Microbiol (2007) 1.19
Characteristic CSF prion seeding efficiency in humans with prion diseases. Mol Neurobiol (2014) 1.19
Rapidly progressive Alzheimer disease. Arch Neurol (2011) 1.19
MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain (2005) 1.18
Conversion efficiency of bank vole prion protein in vitro is determined by residues 155 and 170, but does not correlate with the high susceptibility of bank voles to sheep scrapie in vivo. J Biol Chem (2006) 1.17
Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease. Arch Neurol (2006) 1.15
Quantitative analysis of transthyretin, tau and amyloid-beta in patients with dementia. J Alzheimers Dis (2008) 1.14
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration. Acta Neuropathol (2007) 1.14
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet (2011) 1.12
A refined method for molecular typing reveals that co-occurrence of PrP(Sc) types in Creutzfeldt-Jakob disease is not the rule. Lab Invest (2007) 1.12
Influence of timing on CSF tests value for Creutzfeldt-Jakob disease diagnosis. J Neurol (2007) 1.12
Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein. Proc Natl Acad Sci U S A (2009) 1.10
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis. Acta Neuropathol (2014) 1.10
Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests. APMIS (2002) 1.10
Novel molecular stereotactic biopsy procedures reveal intratumoral homogeneity of loss of heterozygosity of 1p/19q and TP53 mutations in World Health Organization grade II gliomas. J Neuropathol Exp Neurol (2009) 1.09
Creutzfeldt-Jakob disease in a patient with an R208H mutation of the prion protein gene (PRNP) and a 17-kDa prion protein fragment. Acta Neuropathol (2005) 1.09
Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases. J Biol Chem (2001) 1.08
Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie. PLoS Pathog (2007) 1.08
Transcriptome analysis reveals altered cholesterol metabolism during the neurodegeneration in mouse scrapie model. J Neurochem (2007) 1.07
Proteome analysis of the thalamus and cerebrospinal fluid reveals glycolysis dysfunction and potential biomarkers candidates for schizophrenia. J Psychiatr Res (2010) 1.06
Brain-derived proteins in the CSF: do they correlate with brain pathology in CJD? BMC Neurol (2006) 1.06
Cell-free formation of misfolded prion protein with authentic prion infectivity. Proc Natl Acad Sci U S A (2006) 1.05
Sonic hedgehog-associated medulloblastoma arising from the cochlear nuclei of the brainstem. Acta Neuropathol (2012) 1.05
Progressive loss of a glial potassium channel (KCNJ10) in the spinal cord of the SOD1 (G93A) transgenic mouse model of amyotrophic lateral sclerosis. J Neurochem (2006) 1.04
Amyloid precursor protein intracellular domain modulates cellular calcium homeostasis and ATP content. J Neurochem (2007) 1.04
Identification of anti-prion compounds as efficient inhibitors of polyglutamine protein aggregation in a zebrafish model. J Biol Chem (2006) 1.03
Cerebrospinal fluid-optimized two-dimensional difference gel electrophoresis (2-D DIGE) facilitates the differential diagnosis of Creutzfeldt-Jakob disease. Proteomics (2008) 1.03
Time-point and dosage of gene inactivation determine the tumor spectrum in conditional Ptch knockouts. Carcinogenesis (2009) 1.03