Published in Prog Neurobiol on July 29, 2013
Creutzfeldt-Jakob disease associated with a V203I homozygous mutation in the prion protein gene. Prion (2014) 0.80
Prion Diseases. Continuum (Minneap Minn) (2015) 0.78
Prions. Proc Natl Acad Sci U S A (1998) 27.80
Novel proteinaceous infectious particles cause scrapie. Science (1982) 24.09
A new variant of Creutzfeldt-Jakob disease in the UK. Lancet (1996) 20.15
Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature (1997) 15.73
Mice devoid of PrP are resistant to scrapie. Cell (1993) 11.46
Common variants at MS4A4/MS4A6E, CD2AP, CD33 and EPHA1 are associated with late-onset Alzheimer's disease. Nat Genet (2011) 10.07
A cellular gene encodes scrapie PrP 27-30 protein. Cell (1985) 8.86
Synthetic mammalian prions. Science (2004) 7.35
Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol (1999) 6.98
Unconventional viruses and the origin and disappearance of kuru. Science (1977) 6.38
Self-replication and scrapie. Nature (1967) 6.01
Kuru in the 21st century--an acquired human prion disease with very long incubation periods. Lancet (2006) 5.42
Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol (1994) 5.20
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain (2009) 5.03
Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature (1989) 4.69
Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet (2004) 4.59
Experimental transmission of a Kuru-like syndrome to chimpanzees. Nature (1966) 4.29
Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science (1990) 4.19
Does the agent of scrapie replicate without nucleic acid? Nature (1967) 4.14
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet (1999) 4.02
Bovine spongiform encephalopathy: epidemiological studies on the origin. Vet Rec (1991) 3.99
Guidelines for environmental infection control in health-care facilities. Recommendations of CDC and the Healthcare Infection Control Practices Advisory Committee (HICPAC). MMWR Recomm Rep (2003) 3.96
Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion. Nat Med (2011) 3.95
Bovine spongiform encephalopathy: epidemiological studies. Vet Rec (1988) 3.55
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 3.43
Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Natl Acad Sci U S A (2001) 3.39
Cell biology. A unifying role for prions in neurodegenerative diseases. Science (2012) 3.34
Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology (2000) 3.23
Keeping pace with the times--the Genetic Information Nondiscrimination Act of 2008. N Engl J Med (2008) 3.22
Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease. Arch Neurol (2003) 3.20
Recombinant prion protein induces a new transmissible prion disease in wild-type animals. Acta Neuropathol (2010) 3.19
Birth of a normal girl after in vitro fertilization and preimplantation diagnostic testing for cystic fibrosis. N Engl J Med (1992) 3.12
Degenerative disease of the central nervous system in New Guinea; the endemic occurrence of kuru in the native population. N Engl J Med (1957) 3.07
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome. Exp Neurol (1989) 3.00
Rapidly progressive dementia. Ann Neurol (2008) 3.00
The exceptionally small size of the scrapie agent. Biochem Biophys Res Commun (1966) 2.83
Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol (2008) 2.80
Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology (2004) 2.76
Chronic wasting disease. Vet Pathol (2005) 2.74
A comparison of tau and 14-3-3 protein in the diagnosis of Creutzfeldt-Jakob disease. Neurology (2012) 2.66
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60
Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron (2007) 2.58
Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem (2010) 2.57
Hashimoto's encephalitis as a differential diagnosis of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry (1999) 2.55
Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease. Brain (2006) 2.54
Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol (2000) 2.42
Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology (2011) 2.42
Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial. Lancet Neurol (2009) 2.41
Detection of CWD prions in urine and saliva of deer by transgenic mouse bioassay. PLoS One (2009) 2.34
Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature (2003) 2.31
The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review. J Neurol Neurosurg Psychiatry (2010) 2.29
A systematic review of prion therapeutics in experimental models. Brain (2006) 2.25
Transmission and detection of prions in feces. J Infect Dis (2008) 2.20
CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease. Neurology (2006) 2.19
A worldwide assessment of the frequency of suicide, suicide attempts, or psychiatric hospitalization after predictive testing for Huntington disease. Am J Hum Genet (1999) 2.12
De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A (2008) 2.10
Clinical and pathological features and laboratory confirmation of Creutzfeldt-Jakob disease in a recipient of pituitary-derived human growth hormone. N Engl J Med (1985) 2.09
Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease. Ann Neurol (2010) 2.09
Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol (1986) 2.06
Prion disease genetics. Eur J Hum Genet (2006) 2.02
International Huntington Association and the World Federation of Neurology Research Group on Huntington's Chorea. Guidelines for the molecular genetics predictive test in Huntington's disease. J Med Genet (1994) 2.00
Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models. J Virol (2004) 1.97
Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain (2004) 1.96
Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism. Possible relation to the administration of cadaveric human growth hormone. N Engl J Med (1985) 1.92
Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology (2005) 1.88
Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol (2011) 1.86
De novo generation of infectious prions in vitro produces a new disease phenotype. PLoS Pathog (2009) 1.83
Misleading results with the 14-3-3 assay for the diagnosis of Creutzfeldt-Jakob disease. Neurology (2000) 1.82
Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol (2004) 1.74
Total tau protein in cerebrospinal fluid and diffusion-weighted MRI as an early diagnostic marker for Creutzfeldt-Jakob disease. Dement Geriatr Cogn Disord (2007) 1.72
The possible nature of the transmissible agent of scrapie. Vet Rec (1967) 1.68
Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol (2010) 1.66
Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol (1996) 1.66
Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK. Eur J Neurol (2008) 1.63
Molecular analysis of ovine prion protein identifies similarities between BSE and an experimental isolate of natural scrapie, CH1641. J Gen Virol (1999) 1.62
Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology (2006) 1.61
Creutzfeldt-Jakob disease after administration of human growth hormone. Lancet (1985) 1.61
Rapidly progressive dementia. Neurol Clin (2007) 1.58
New variant Creutzfeldt-Jakob disease: psychiatric features. Lancet (1997) 1.55
Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice. Neuron (2009) 1.49
The expanding universe of prion diseases. PLoS Pathog (2006) 1.45
Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology (2004) 1.45
Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia (2010) 1.45
Inactivation of prions by acidic sodium dodecyl sulfate. J Virol (2006) 1.43
Law & psychiatry: Genetic discrimination in mental disorders: the impact of the genetic information nondiscrimination act. Psychiatr Serv (2010) 1.43
Purified scrapie prions resist inactivation by UV irradiation. J Virol (1987) 1.43
Acquired prion disease: iatrogenic CJD, variant CJD, kuru. Br Med Bull (2003) 1.41
Prion hypothesis: the end of the controversy? Trends Biochem Sci (2010) 1.40
RESISTANCE OF THE SCRAPIE AGENT TO FORMALIN. J Comp Pathol (1965) 1.40
Inherited prion diseases and transmission to rodents. Brain Pathol (1995) 1.39
Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry (2001) 1.39
Epidemiological and genetical differences between classical and atypical scrapie cases. Vet Res (2006) 1.38
Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD. Ann Neurol (1998) 1.37
The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementia. J Neurosurg (2007) 1.37
A natural case of chronic wasting disease in a free-ranging moose (Alces alces shirasi). J Wildl Dis (2007) 1.34
Scrapie infections initiated at varying doses: an analysis of 117 titration experiments. Philos Trans R Soc Lond B Biol Sci (2000) 1.34
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
Impact of genetic testing for Huntington disease on the family system. Am J Med Genet (2000) 1.26
PrP knock-out and PrP transgenic mice in prion research. Br Med Bull (2003) 1.26
AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol (2009) 4.49
Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol (2005) 3.11
Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol (2006) 2.27
Diagnosis of human prion disease. Proc Natl Acad Sci U S A (2005) 2.13
Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. Arch Neurol (2003) 2.12
Adult-onset drug-refractory seizure disorder associated with anti-voltage-gated potassium-channel antibody. Epilepsia (2009) 1.88
Clinicopathological correlations in corticobasal degeneration. Ann Neurol (2011) 1.86
Seizures and epileptiform activity in the early stages of Alzheimer disease. JAMA Neurol (2013) 1.68
Exome sequencing identifies ACSF3 as a cause of combined malonic and methylmalonic aciduria. Nat Genet (2011) 1.48
Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease. Lab Invest (2009) 1.48
Evidence for a role of the rare p.A152T variant in MAPT in increasing the risk for FTD-spectrum and Alzheimer's diseases. Hum Mol Genet (2012) 1.46
The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementia. J Neurosurg (2007) 1.37
Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects. Cell Rep (2012) 1.16
Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet J Rare Dis (2013) 1.15
Rapidly progressive dementia: prion diseases and other rapid dementias. Continuum (Minneap Minn) (2010) 1.15
A randomized, placebo-controlled trial of latrepirdine in Huntington disease. Arch Neurol (2010) 1.10
Anti-GAD antibody cerebellar ataxia mimicking Creutzfeldt-Jakob disease. Clin Neurol Neurosurg (2006) 1.03
A 54-year-old man with slowness of movement and confusion. Neurology (2007) 1.01
Prion diseases. Semin Neurol (2013) 1.01
Rapidly Progressive Dementia. Continuum (Minneap Minn) (2016) 0.99
Differential diagnosis of Jakob-Creutzfeldt disease. Arch Neurol (2012) 0.98
Thorough work-up and new diagnostic criteria needed for CJD. Nat Rev Neurol (2011) 0.93
Human prions and plasma lipoproteins. Proc Natl Acad Sci U S A (2006) 0.93
Clinical trials for prion disease: difficult challenges, but hope for the future. Lancet Neurol (2009) 0.90
Neurodegenerative disease phenotypes in carriers of MAPT p.A152T, a risk factor for frontotemporal dementia spectrum disorders and Alzheimer disease. Alzheimer Dis Assoc Disord (2013) 0.90
Familial Creutzfeldt-Jakob disease with V180I mutation. J Korean Med Sci (2010) 0.90
Creutzfeldt-Jakob disease in recipients of corneal transplants. Cornea (2008) 0.84
Clinical update of Jakob-Creutzfeldt disease. Curr Opin Neurol (2015) 0.84
Executive functions in premanifest Huntington's disease. Mov Disord (2013) 0.84
Diagnosis and treatment of rapidly progressive dementias. Neurol Clin Pract (2012) 0.83
Immunologically mediated dementias. Curr Neurol Neurosci Rep (2009) 0.83
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease. J Neuropathol Exp Neurol (2010) 0.80
Clinical overlap between Jakob-Creutzfeldt disease and Lewy body disease. Can J Neurol Sci (2012) 0.79
Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia. JAMA Neurol (2013) 0.79
Neuroimaging in dementia. Semin Neurol (2008) 0.78
Prion Diseases. Continuum (Minneap Minn) (2015) 0.78
Human growth hormone-related iatrogenic Creutzfeldt-Jakob disease with abnormal imaging. Arch Neurol (2006) 0.78
De novo prions. F1000 Biol Rep (2010) 0.78
When sporadic disease is not sporadic: the potential for genetic etiology. Arch Neurol (2004) 0.75
Psychiatric and neuroimaging findings in Creutzfeldt-Jakob disease. Curr Psychiatry Rep (2003) 0.75