Mad cow-related death in Texas

Lab tests confirm that a patient who recently died had Variant CJD, a fatal brain disorder linked to mad cow disease, health officials say.
Source: CNN.com - Health - Category: Consumer Health News Source Type: news

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AbstractHuman prion diseases (HPDs) are progressive and fatal neurodegenerative diseases caused by abnormal prion protein (PrPSc). They can be sporadic, genetic, or acquired. Sporadic HPDs include sporadic Creutzfeldt –Jakob disease (CJD) and sporadic fatal insomnia. Genetic HPDs include genetic CJD, Gerstman–Sträussler–Scheinker disease and fatal familial insomnia. Acquired HPDs include Kuru, variant CJD, and iatrogenic CJD. The World Health Organization clinical diagnostic criteria for HPDs include clini cal findings, cerebrospinal fluid (CSF) protein markers, and electroencephalography, and the UK...
Source: Clinical and Experimental Neuroimmunology - Category: Neurology Authors: Tags: INVITED REVIEW Source Type: research
Research advances in recent years have shown that some individuals with vegetative state or minimally conscious state can emerge to higher states of consciousness even years after injury. A minority of behaviorally unresponsive patients with vegetative state have also been shown to follow commands, or even communicate, using neuroimaging or electrophysiological techniques. These advances raise ethical questions that have important implications for clinical care. In this article, the authors argue that adopting a neuropalliative care approach can help clinicians provide ethical, compassionate care to these patients and their caregivers.
Source: Neurologic Clinics - Category: Neurology Authors: Source Type: research
Conclusions The incidence of CJD, in SWSLHD, exceeds the national average of 1/million. Cost-effective, adequate diagnostic and screening tools, implementab le over a large population, will become increasingly essential.  
Source: Neuroepidemiology - Category: Epidemiology Source Type: research
Tauopathies like Alzheimer's disease are characterized by the spread of tau aggregates through the brain. Tau is one of the few molecules in the body that can become altered in a way that encourages other copies of the same molecule to also alter, causing aggregates to form. These aggregates and their surrounding biochemistry are disruptive to cell function and toxic to cells. A number of neurodegnerative conditions are associated with protein aggregates of amyloid-β, α-synuclein, and tau. The mechanisms by which this spread occurs are debated, but researchers strongly suspect a role for glial cells in this proc...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs
Journal of Palliative Medicine, Ahead of Print.
Source: Journal of Palliative Medicine - Category: Palliative Care Authors: Source Type: research
Source: Acta Neurologica Belgica - Category: Neurology Source Type: research
AbstractHuman prion diseases are fatal neurodegenerative disorders that include sporadic, infectious and genetic forms. Inherited Creutzfeldt-Jakob disease due to the E200K mutation of the prion protein-coding gene is the most common form of genetic prion disease. The phenotype resembles that of sporadic Creutzfeldt-Jakob disease at both the clinical and pathological levels, with a median disease duration of 4 months. To date, there is no available treatment for delaying the occurrence or slowing the progression of human prion diseases. Existingin vivo models do not allow high-throughput approaches that may facilitate the ...
Source: Brain - Category: Neurology Source Type: research
This report describes the patient’s insidious clinical evolution and the paraclinical examinations and pathology reports gathered at different time points of disease progression. Unfortunately, the absence of typical clinical and paraclinical features of classic sporadic Creutzfeldt–Jakob disease made the brain biopsy surgery necessary. This case report illustrates the diagnostic difficulties posed by the phenotypic heterogeneity of sporadic Creutzfeldt–Jakob disease and urges clinicians to consider this diagnosis even in patients who do not fulfil the typical clinical disease criteria. Furthermore, it hi...
Source: Viruses - Category: Virology Authors: Tags: Case Report Source Type: research
We report the case of a 56-year-old woman with the rapidly progressive cognitive impairment, motor dysfunctions and the fulminant neurological deterioration to akinetic mutism during the five weeks' hospitalisation. The probable diagnosis of sCJD was based on medical history and characteristic findings in MRI. The positive result of the real-time quaking-induced conversion (RT-QuIC) test and presence of protein 14-3-3 were obtained post-mortem and definite diagnosis was confirmed by neuropathological examination. In this paper we would like to emphasize the difficulties in reaching the diagnosis and the need for a series o...
Source: Folia Neuropathologica - Category: Pathology Authors: Source Type: research
Asian J Surg. 2021 Oct 7:S1015-9584(21)00565-0. doi: 10.1016/j.asjsur.2021.08.062. Online ahead of print.NO ABSTRACTPMID:34629264 | DOI:10.1016/j.asjsur.2021.08.062
Source: Asian Journal of Surgery - Category: Surgery Authors: Source Type: research
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