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Familial Creutzfeldt-Jakob disease
Journal of the Neurological Sciences, 1979A Finnish family is described with 9 cases of presenile dementia in 3 generations. The mean age at onset was 52 years (range 46--62 years). Progressive dementia, upper motor neuron signs, muscular rigidity, and twitching, irregular tremors were consistent features in the 6 clinically investigated patients and were associated with spongiform change in ...
M, Haltia +4 more
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UNDERSTANDING Creutzfeldt-Jakob DISEASE
Journal of Gerontological Nursing, 1997ABSTRACT The "mad cow disease" that decimated cattle in England has brought widespread attention to a similar disease in humans, Creutzfeldt-Jakob disease (CJD). This has led to concerns about the transmissibility and contagiousness of the infectious agent from possible OD patients to health care workers and family members.
S T, Nguyen, L S, Rickman
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Iatrogenic Creutzfeldt-Jakob disease
Alzheimer Disease & Associated Disorders, 1987Over the past 2 years, Creutzfeldt-Jakob disease (CJD) has affected several patients who received cadaver pituitary-derived growth hormone (pit-hGH) and one patient who received a cadaveric dura mater graft. The risk of iatrogenic transmission of CJD has long been recognized, but until recently, the low prevalence of the disorder and minimal use of ...
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Unilateral Creutzfeldt‐Jakob disease
Neurology, 1986A 73-year-old woman had progressive right hemiparesis, aphasia, and focal motor seizures. EEG showed periodic discharges on the left. She died 8 weeks after onset. At autopsy, there was marked spongiform change, neuronal loss, and severe proliferation of astrocytes predominantly on the left and most prominently in the insular and centroparietal cortex.
H, Yamanouchi, H, Budka, K, Vass
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Variant Creutzfeldt–Jakob disease
2018Variant CJD (vCJD) was described first in the United Kingdom in 1996. It is a zoonotic form of human prion disease, originating from dietary contamination of human food with material from bovine spongiform encephalopathy (BSE)-affected cattle. It has important epidemiologic, clinical, and neuropathogic differences from other forms of human prion ...
Jean-Philippe, Brandel, Richard, Knight
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Variant Creutzfeldt-Jakob disease
The Lancet, 1999It is clear that the prion strain causing bovine spongiform encephalopathy (BSE) in cattle has infected human beings, manifesting itself as a novel human prion disease, variant Creutzfeldt-Jakob disease (CjD). Studies of the incubation periods seen in previous epidemics of human prion disease and of the effect of transmission barriers limiting spread ...
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Iatrogenic Creutzfeldt–Jakob disease
2018Iatrogenic transmission of Creutzfeldt-Jakob disease (CJD) has occurred through particular medical procedures. Among them, dura mater grafts and pituitary-derived growth hormone obtained from human cadavers undiagnosed as CJD are the most frequent sources of infection. Recent advances in our knowledge about dura mater graft- and human pituitary-derived
Atsushi, Kobayashi +2 more
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Genetic Creutzfeldt–Jakob disease
2018Genetic Creutzfeldt-Jakob disease (CJD) is associated with mutations in the human PrP gene (PRNP) on chromosome 20p12-pter. Pathogenic mutations have been identified in 10-15% of all CJD patients, who often have a family history of autosomal-dominant pattern of inheritance and variable penetrance.
Anna, Ladogana, Gabor G, Kovacs
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Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease
Journal of Neurology, Neurosurgery & Psychiatry, 2009The “pulvinar sign” on MRI brain scan is defined as hyperintensity of the posterior thalamus relative to the signal intensity of the anterior putamen.1 In the appropriate clinical context, the pulvinar sign and hyperintensity in the dorsomedial thalamic nuclei (the hockey stick sign) are sensitive and specific features of variant Creutzfeldt–Jakob ...
G G Kovacs +7 more
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