Mad Cow Disease in California

Mad Cow Disease in California
Source: eMedicineHealth.com - Category: Journals (General) Source Type: news

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We describe a patient who presented with visual snow, illusory visual motion, photopsia, and reduced night vision. He subsequently developed progressive cognitive impairment, myoclonus and ataxia. A diagnosis of sporadic Creutzfeldt-Jakob Disease was confirmed on post-mortem examination more than 49 months after symptom onset. The visual snow syndrome is typically benign, but our patient illustrates that occasionally it is the first manifestation of a serious brain disease. Careful application of the diagnostic criteria for the visual snow syndrome is important, particularly with the use of neuroimaging to exclude pathol...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Publication date: Available online 22 March 2019Source: Journal of Clinical NeuroscienceAuthor(s): Yoon-Jung Kang, Kyung-Hye Kim, Sung-Hwan Jang, Gha-Hyun Lee, Yoon-Jung Lee, Yong-Sun Kim, Eun-Joo KimAbstractThe familial Creutzfeldt-Jakob disease (fCJD) usually has similar clinical and neuroimaging features as sporadic CJD (sCJD). A 57-year-old man presented with a four-month history of rapidly progressive dementia (RPD). Laboratory tests for RPD were all normal. Brain MRI demonstrated diffuse cortical atrophy and no abnormal cortical or striatal hyperintensities on fluid-attenuated inversion recovery (FLAIR)/diffusion wei...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
CONCLUSIONS: The indistinguishable clinical features of P102L GSS patients with sCJD, especially in the early stage, support the importance of PRNP testing for diagnosing GSS. PMID: 30877692 [PubMed - as supplied by publisher]
Source: Journal of Clinical Neurology - Category: Neurology Tags: J Clin Neurol Source Type: research
enbaum Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology, and molecular genetics. We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt–Jakob disease, as well as the ...
Source: Viruses - Category: Virology Authors: Tags: Review Source Type: research
g J Abstract PMID: 30840102 [PubMed - as supplied by publisher]
Source: Der Nervenarzt - Category: Neurology Authors: Tags: Nervenarzt Source Type: research
Source: Neuroscience Bulletin - Category: Neuroscience Source Type: research
CONCLUSIONS: The surveillance of CJD in ES (2004-2016) showed an increased incidence of CJD in ES, reaching the incidence rate of the whole country, with a permanent proportion of 70% gCJD cases based on the E200K mutation. Clinical, electrophysiological and MR features of sCJD and gCJD cases were in conformity with already published data. Epidemiological analysis of CJD in ES shows increasing detection of CJD but also suggests that current routine surveillance systems for CJD may underestimate the true burden of disease, especially sporadic cases in Slovakia. PMID: 30817871 [PubMed - in process]
Source: Central European Journal of Public Health - Category: International Medicine & Public Health Tags: Cent Eur J Public Health Source Type: research
Officials are worried that a prion disease spreading among deer in the U.S. may someday threaten the health of humans. Chronic wasting disease (CWD), which is nicknamed “zombie deer disease,” gets its name from a range of symptoms. For deer, elk and moose, these include stumbling, listlessness, drooling and rapid weight loss. The fatal disease degrades the brains, spines and bodies of the animals it infects, and there are no known treatments or vaccines. No cases of CWD have been recorded in humans. It has not even been definitely proven that humans can be infected by CWD, though some research has suggested it&...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized healthytime public health Source Type: news
A 71-year-old man was referred to Neuro-Ophthalmology for evaluation of reading problems associated to pituitary adenoma. Perimetry showed a right temporal crescent syndrome ( “half moon” syndrome) suggesting left occipital disease. Brain MRI, electroencephalogram and CSF examination (14-3-3 protein) were consistent with a diagnosis of Creutzfeldt-Jakob disease. His neurologic condition rapidly deteriorated with memory problems, myoclonus and apathy followed by death, four months later. This case shows how visual problems may be the first manifestation of this rare prion disease (Heidehain variant of Creutzfeldt-Jakob disease).
Source: Survey of Ophthalmology - Category: Opthalmology Authors: Tags: Clinical challenges Source Type: research
The disease course of dementia with Lewy bodies (DLB) can be rapidly progressive, clinically resembling Creutzfeldt-Jakob's disease (CJD). To better understand factors contributing to this rapidly progressive disease course, we describe load and distribution of neuropathology, and the presence of possible disease-associated genetic defects in a post-mortem series of DLB cases clinically suspected of CJD.
Source: Parkinsonism and Related Disorders - Category: Neurology Authors: Source Type: research
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