SUBACUTE SPONGIFORM ENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE): THE NATURE AND PROGRESSION OF SPONGIFORM CHANGE

Prions

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Prion Protein Biology

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Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease

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Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: Disease Phenotype Determined by a DNA Polymorphism

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Creutzfeldt‐Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering

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Sporadic and familial CJD: classification and characterisation

Gambetti, P. L., Kong, Q., Zou, W., Parchi, P., & Chen, S. G. (2003) · British Medical Bulletin · doi:10.1093/bmb/66.1.213 · Cited by 568 · see citing works

Prions: Protein Aggregation and Infectious Diseases

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Dementia lacking distinctive histologie features

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Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism.

Monari, L., Chen, S. G., Brown, P., Parchi, P., Petersen, R. B., Mikol, J., Gray, F., Cortelli, P., Montagna, P., & Ghetti, B. F. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.7.2839 · Cited by 332 · see citing works

Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft

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Prion diseases of humans and animals

Prusiner, S. B., Telling, G. C., Cohen, F. E., & DeArmond, S. J. (1996) · Seminars in Virology · doi:10.1006/smvy.1996.0021 · Cited by 241 · see citing works

Role of microglia in neuronal cell death in prion disease

Giese, A., Brown, D. R., Groschup, M. H., Feldmann, C., Haist, I., & Kretzschmar, H. A. (1998) · Brain Pathology · doi:10.1111/j.1750-3639.1998.tb00167.x · Cited by 239 · see citing works

Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes

Legname, G., Nguyen, H.-O. B., Peretz, D., Cohen, F. E., DeArmond, S. J., & Prusiner, S. B. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0608970103 · Cited by 219 · see citing works

Etiology and pathogenesis of prion diseases.

DeArmond, S. J., & Prusiner, S. B. (1995) · Europe PMC (PubMed Central) · Cited by 219 · see citing works

A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-79. I: Clinical features.

Will, R. G., & Matthews, W. B. (1984) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.47.2.134 · Cited by 212 · see citing works

Parietal and Occipital Lobe Epilepsy: A Review

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Prion Protein of 106 Residues Creates an Artificial Transmission Barrier for Prion Replication in Transgenic Mice

Supattapone, S., Bosque, P. J., Muramoto, T., Wille, H., Aagaard, C., Peretz, D., Nguyen, H.-O. B., Heinrich, C., Torchia, M., Safar, J., et al. (1999) · Cell · doi:10.1016/s0092-8674(00)80596-6 · Cited by 205 · see citing works

PrP Deposition, Microglial Activation, and Neuronal Apoptosis in Murine Scrapie

Williams, A., Lucassen, P. J., Ritchie, D., & Bruce, M. (1997) · Experimental Neurology · doi:10.1006/exnr.1997.6424 · Cited by 204 · see citing works

Prions and Neurodegenerative Diseases

Epstein, F. H., & Prusiner, S. B. (1987) · New England Journal of Medicine · doi:10.1056/nejm198712173172505 · Cited by 192 · see citing works

Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95

van Duijn, C. M., Delasnerie-Lauprêtre, N., Masullo, C., Zerr, I., de Silva, R., Wientjens, D., Jp, B., Weber, T., Bonavita, V., Zeidler, M., et al. (1998) · The Lancet · doi:10.1016/s0140-6736(97)09468-3 · Cited by 184 · see citing works

MRI of Creutzfeldt–Jakob Disease: Imaging Features and Recommended MRI Protocol

Collie, D. A., Sellar, R. J., Zeidler, M., Colchester, A. C. F., Knight, R., & Will, R. G. (2001) · Clinical Radiology · doi:10.1053/crad.2001.0771 · Cited by 179 · see citing works

Creutzfeldt–Jakob Disease in a Young Adult with Idiopathic Hypopituitarism

Koch, T., Berg, B. O., Armond, S. J. D., & Gravina, R. F. (1985) · New England Journal of Medicine · doi:10.1056/nejm198509193131206 · Cited by 172 · see citing works

Correlation of Diffusion-Weighted Magnetic Resonance Imaging With Neuropathology in Creutzfeldt-Jakob Disease

Mittal, S., Farmer, P., Kalina, P., Kingsley, P. B., & HALPERIN, J. J. (2002) · Archives of Neurology · doi:10.1001/archneur.59.1.128 · Cited by 170 · see citing works

Genetic and Infectious Prion Diseases

Prusiner, S. B. (1993) · Archives of Neurology · doi:10.1001/archneur.1993.00540110011002 · Cited by 167 · see citing works

Neuronal Cell Death in Scrapie‐Infected Mice Is Due to Apoptosis

Giese, A., Groschup, M. H., Heß, B., & Kretzschmar, H. A. (1995) · Brain Pathology · doi:10.1111/j.1750-3639.1995.tb00597.x · Cited by 163 · see citing works

Progressive aphasia without dementia: Two cases with focal spongiform degeneration

Kirshner, H. S., Tanrıdağ, O., Thurman, L., & Whetsell, W. O. (1987) · Annals of Neurology · doi:10.1002/ana.410220413 · Cited by 156 · see citing works

Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future

Parchi, P., Strammiello, R., Giese, A., & Kretzschmar, H. A. (2010) · Acta Neuropathologica · doi:10.1007/s00401-010-0779-6 · Cited by 155 · see citing works

Prion Protein Fragment PrP-(106–126) Induces Apoptosis via Mitochondrial Disruption in Human Neuronal SH-SY5Y Cells

O'Donovan, C. N., Tobin, D., & Cotter, T. G. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m103894200 · Cited by 152 · see citing works

Creutzfeldt‐Jakob disease of long duration: Clinicopathological characteristics, transmissibility, and differential diagnosis

Brown, P., Rodgers‐Johnson, P., Cathala, F., Gibbs, C. J., & Gajdusek, D. C. (1984) · Annals of Neurology · doi:10.1002/ana.410160305 · Cited by 148 · see citing works

Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt‐Jakob disease

Salazar, A. Μ., Masters, C. L., Gajdusek, D. C., & Gibbs, C. J. (1983) · Annals of Neurology · doi:10.1002/ana.410140104 · Cited by 138 · see citing works

Hereditary dysphasic dementia and the Pick‐Alzheimer spectrum

Morris, J. C., Cole, M., Banker, B. Q., & Wright, D. (1984) · Annals of Neurology · doi:10.1002/ana.410160407 · Cited by 137 · see citing works

Discovery of 2-Aminothiazoles as Potent Antiprion Compounds

Ghaemmaghami, S., May, B. C. H., Renslo, A. R., & Prusiner, S. B. (2009) · Journal of Virology · doi:10.1128/jvi.02145-09 · Cited by 136 · see citing works

Abnormal Diffusion-Weighted Magnetic Resonance Images in Creutzfeldt-Jakob Disease

Bahn, M. M., & Parchi, P. (1999) · Archives of Neurology · doi:10.1001/archneur.56.5.577 · Cited by 133 · see citing works

Classical sheep transmissible spongiform encephalopathies: pathogenesis, pathological phenotypes and clinical disease

Jeffrey, M., & González, L. (2007) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2007.00868.x · Cited by 128 · see citing works

Diffusion-Weighted Magnetic Resonance Imaging in Probable Creutzfeldt-Jakob Disease

Na, D. L., Suh, C. K., Choi, S. H., Moon, H., Seo, D., Kim, S. E., Na, D. G., & Adair, J. C. (1999) · Archives of Neurology · doi:10.1001/archneur.56.8.951

The cellular prion protein modulates phagocytosis and inflammatory response

de Almeida, C. J. G., Chiarini, L. B., da Silva, J. P., Silva, P., Martins, M. A., & Linden, R. (2004) · Journal of Leukocyte Biology · doi:10.1189/jlb.1103531 · Cited by 116 · see citing works

Evidence for an alternative direct route of access for the scrapie agent to the brain bypassing the spinal cord.

Baldauf, E., Diringer, H., & Beekes, M. (1997) · Journal of General Virology · doi:10.1099/0022-1317-78-5-1187 · Cited by 116 · see citing works

Bovine Spongiform Encephalopathy: A Neuropathological Perspective

Wells, G. A. H., Wilesmith, J. W., & McGill, I. S. (1991) · Brain Pathology · doi:10.1111/j.1750-3639.1991.tb00642.x · Cited by 112 · see citing works

Neuroimaging findings in human prion disease

Macfarlane, R., Wroe, S. J., Collinge, J., Yousry, T. A., & Jäger, H. R. (2006) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2006.094821 · Cited by 111 · see citing works

Panencephalopathic type of Creutzfeldt-Jakob disease: primary involvement of the cerebral white matter

Mizutani, T., Okumura, A., Oda, M., & SHIRAKI, H. (1981) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.44.2.103 · Cited by 111 · see citing works

Iatrogenic transmission of Creutzfeldt-Jakob disease

Weller, R. O. (1989) · Psychological Medicine · doi:10.1017/s0033291700010965 · Cited by 105 · see citing works

Microglia and the pathogenesis of spongiform encephalopathies

Rezaie, P. (2001) · Brain Research Reviews · doi:10.1016/s0165-0173(01)00042-x · Cited by 99 · see citing works

Pathological Effect of Homeostatic Synaptic Scaling on Network Dynamics in Diseases of the Cortex

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Bovine Spongiform Encephalopathy

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Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease

Manners, D. N., Parchi, P., Tonon, C., Capellari, S., Strammiello, R., Testa, C. M., Tani, G., Malucelli, E., Spagnolo, C., Cortelli, P., et al. (2009) · Neurology · doi:10.1212/wnl.0b013e3181a18846 · Cited by 91 · see citing works

Neuronal Apoptosis in Fatal Familial Insomnia

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Brain Magnetic Resonance Diffusion Abnormalities in Creutzfeldt-Jakob Disease

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Early and Rapid Engraftment of Bone Marrow-Derived Microglia in Scrapie

Priller, J., Prinz, M., Heikenwälder, M. F., Zeller, N., Schwarz, P., Heppner, F. L., & Aguzzi, A. (2006) · Journal of Neuroscience · doi:10.1523/jneurosci.2275-06.2006 · Cited by 89 · see citing works

Creutzfeldt-Jakob Disease: Serial Changes on Diffusion-Weighted MRI

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Classification of Dystonia

di Biase, L., Santo, A. D., Caminiti, M. L., Pecoraro, P. M., & Lazzaro, V. D. (2022) · Life · doi:10.3390/life12020206 · Cited by 85 · see citing works

Temporal dynamics of hippocampal neurogenesis in chronic neurodegeneration

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Familial Creutzfeldt-Jakob disease

Haltia, M., Kovanen, J., van Crevel, H., Bots, G. T. A. M., & STEFANKO, S. Z. (1979) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(79)90171-0 · Cited by 81 · see citing works

The Nosology of Creutzfeldt‐Jakob Disease and Conditions Related to the Accumulation of PrPCJD in the Nervous System

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Prion Diseases of Humans and Animals

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Immunohistochemical study of microglia in the Creutzfeldt-Jakob diseased brain

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Immunohistochemical confirmation of Creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies.

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Phenotyping of Protein-Prion (PrPsc)-accumulating Cells in Lymphoid and Neural Tissues of Naturally Scrapie-affected Sheep by Double-labeling Immunohistochemistry

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Prions and related neurological diseases

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Movement disorders and Creutzfeldt-Jakob disease: A review

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Prion-Induced Neuronal Damage — The Mechanisms of Neuronal Destruction in the Subacute Spongiform Encephalopathies

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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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Prion Protein Misfolding, Strains, and Neurotoxicity: An Update from Studies on Mammalian Prions

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Retinal correlates of neurological disorders

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Quantification of cortical atrophy in a case of progressive fluent aphasia

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Pathology of the transmissible spongiform encephalopathies with special emphasis on ultrastructure

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Early unsuspected neuron and axon terminal loss in scra pie‐infected mice revealed by morphometry and immunocytochemistry

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Correlating DWI MRI With Pathologic and Other Features of Jakob-Creutzfeldt Disease

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Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease)

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Phenotypic variability in human prion diseases

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PATHOGENESIS OF MOUSE SCRAPIE: DYNAMICS OF VACUOLATION IN BRAIN AND SPINAL CORD AFTER INTRAPERITONEAL INFECTION

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Experimental transmission of human subacute spongiform encephalopathy to small rodents

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Prions in the peripheral nerves of bovine spongiform encephalopathy-affected cattle

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Amyotrophy in Prion Diseases

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Prion protein immunocytochemistry: reliable protocols for the investigation of Creutzfeldt–Jakob disease

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Vacuolar Change in Alzheimer's Disease

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Familial myoclonic dementia masquerading as Creutzfeldt‐Jakob disease

Little, B. W., Brown, P., Rodgers‐Johnson, P., Perl, D. P., & Gajdusek, D. C. (1986) · Annals of Neurology · doi:10.1002/ana.410200209 · Cited by 51 · see citing works

Vibrator (vb): a spinocerebellar system degeneration with autosomal recessive inheritance in mice

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Descriptive Epidemiology of Creutzfeldt-Jakob Disease in Chile

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Orally Administered Prion Protein Is Incorporated by M Cells and Spreads into Lymphoid Tissues with Macrophages in Prion Protein Knockout Mice

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Inherited prion disease caused by the V210I mutation

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Neuropathologic characteristics of brainstem lesions in sporadic Creutzfeldt-Jakob disease

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The Neurochemistry of Prion Diseases

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Emerging patterns of diffusion-weighted MR imaging in Creutzfeldt-Jakob disease: case report and review of the literature.

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Prions and blood products

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The Pathogenesis of Rabies

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Clinical and pathological study of three Tunisian siblings with L-2-hydroxyglutaric aciduria

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Subakute spongiforme Encephalopathie mit multiformer Plaquebildung

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Creutzfeldt-Jakob disease with extensive degeneration of white matter

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Anti-PrP Mab 6D11 suppresses PrPSc replication in prion infected myeloid precursor line FDC-P1/22L and in the lymphoreticular system in vivo

Sadowski, M. J., Pankiewicz, J. E., Prelli, F. C., Scholtzova, H., Spinner, D. S., Kascsak, R. B., Kascsak, R. J., & Wısnıewskı, T. (2009) · Neurobiology of Disease · doi:10.1016/j.nbd.2009.01.013 · Cited by 45 · see citing works

Identification of the End Stage of Scrapie Using Infected Neural Grafts

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Conflicting MRI signals from gliosis and neuronal vacuolation in prion diseases

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Bax deletion does not protect neurons from BSE-induced death

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Cell death in prion disease

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