Results 1 to 10 of about 21,599 (190)

Three cases of Creutzfeldt–Jakob disease presenting with a predominant dysexecutive syndrome

Journal of Neurology, 2022
Creutzfeldt-Jakob disease (CJD) is a rare, uniformly fatal prion disease. Although CJD commonly presents with rapidly progressive dementia, ataxia, and myoclonus, substantial clinicopathological heterogeneity is observed in clinical practice. Unusual and predominantly cognitive clinical manifestations of CJD mimicking common dementia syndromes are ...
Nick Corriveau-Lecavalier, Wentao Li, Vijay K. Ramanan, Daniel A. Drubach, Gregory S. Day, David T. Jones   +5 more
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Creutzfeldt-Jakob syndrome presenting as epilepsia partialis continua

Journal of Clinical Neuroscience, 2001
Creutzfeldt-Jakob disease (CJD) is an uncommon rapidly progressive neurological disorder which can have protean clinical presentations. We report an autopsy-proven case of CJD presenting initially as epilepsia partialis continua but then developing the typical clinical features of CJD over several weeks.
J, Parry, P, Tuch, W, Knezevic, V, Fabian   +3 more
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Creutzfeldt-Jakob–Like Syndrome due to Hypercalcemic Encephalopathy

Clinical EEG and Neuroscience, 2014
Hypercalcemia can cause a subacute syndrome of progressive dementia and marked changes in the electroencephalogram (EEG). We report a case of iatrogenic hypercalcemia with a close correlation between the clinical course and the EEG changes. A 73-year-old woman presented with a subacute syndrome of progressive dementia and bursts of 1.5 to 2 Hz ...
Johannes, Rösche, Catharina, Sieveking, Christina, Kampf, Reiner, Benecke   +3 more
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MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

The Cerebellum, 2009
Creutzfeldt-Jakob Disease (CJD) is characterized by bilateral basal ganglia hyperintensities on T2W and diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) scans, consistent with its extrapyramidal neurological manifestations. MRI is diagnostically uninformative about the cerebellar symptoms, equally prominent in CJD.
Oren S, Cohen, Chen, Hoffmann, Hedok, Lee, Joab, Chapman, Robert K, Fulbright, Isak, Prohovnik   +5 more
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Presentation of Creutzfeldt–Jakob disease as acute corticobasal degeneration syndrome

Movement Disorders, 2004
AbstractWe provide a brief report with videotape documentation of a possible case of Creutzfeldt–Jakob disease.
Galit, Kleiner-Fisman, Catherine, Bergeron, Anthony E, Lang   +2 more
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Transmission of Creutzfeldt–Jakob disease with scrapie-like syndromes to mice

Nature, 1978
THE transmission of Creutzfeldt–Jakob disease of man, one of the subacute spongiform virus encephalopathies1, to guinea pigs2,3 and to hamsters4 has been reported from this laboratory; Brownell et al. have also claimed transmission of Creutzfeldt–Jakob disease to mice5,6.
E E, Manuelidis, E J, Gorgacz, L, Manuelidis   +2 more
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Creutzfeldt-Jakob disease presenting as Wernicke-Korsakoff syndrome

Journal of the Neurological Sciences, 1992
A 47-year-old man began to suffer from progressive truncal ataxia and mental alterations typical of Wernicke-Korsakoff syndrome. He showed confusional state, hallucinations, delirium of jealousy and a serious impairment of recent memory. The symptomatology lasted 13 months, but only in the last weeks was it complicated by myoclonias.
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[A note on the epidemiology of Creutzfeldt-Jakob syndrome].

Revue neurologique, 1998
The annual incidence of sporadic Jakob-Creuzfeldt disease has been stable for the last 30 years. The new variant affecting young adults which appeared in the United Kingdom (20 cases) and France (1 case) in 1994 is due to the same infectious agent which causes bovine spongiform encephalopathy.
A, Alpérovitch, N, Delasnerie-Lauprêtre, J P, Brandel   +2 more
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Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome

Experimental Neurology, 1989
A host protein encoded by the gene specifying the scrapie amyloid precursor affects pathogenesis of the transmissible spongiform encephalopathies: Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker's syndrome (GSS), and kuru in man, and scrapie in animals.
D, Goldgaber, L G, Goldfarb, P, Brown, D M, Asher, W T, Brown, S, Lin, J W, Teener, S M, Feinstone, R, Rubenstein, R J, Kascsak   +9 more
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Characterising the uncommon corticobasal syndrome presentation of sporadic Creutzfeldt-Jakob disease

Parkinsonism & Related Disorders, 2013
Corticobasal syndrome (CBS), which encompasses cortical sensory loss, alien limb, bradykinesia, rigidity, limb apraxia and dystonia, is the classic presentation of corticobasal degeneration (CBD). It may occur in other neurodegenerative disorders including sporadic Creutzfeldt-Jakob disease (sCJD).
Lee, W, Simpson, M, Ling, H, Mclean, C, Collins, S, Williams, DR   +5 more
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