Chapter 20 Differential diagnosis with other rapid progressive dementias in human prion diseases

Publication date: 2018 Source:Handbook of Clinical Neurology, Volume 153 Author(s): Michael D. Geschwind, Katy Murray Prion diseases are unique in medicine as in humans they occur in sporadic, genetic, and acquired forms. The most common human prion disease is sporadic Creutzfeldt–Jakob disease (CJD), which commonly presents as a rapidly progressive dementia (RPD) with behavioral, cerebellar, extrapyramidal, and some pyramidal features, with the median survival from symptom onset to death of just a few months. Because human prion diseases, as well as other RPDs, are relatively rare, they can be difficult to diagnose, as most clinicians have seen few, if any, cases. Not only can prion diseases mimic many other conditions that present as RPD, but some of those conditions can present similarly to prion disease. In this article, the authors discuss the different etiologic categories of conditions that often present as RPD and also present RPDs that had been misdiagnosed clinically as CJD. Etiologic categories of conditions are presented in order of the mnemonic used for remembering the various categories of RPDs: VITAMINS-D, for vascular, infectious, toxic-metabolic, autoimmune, mitochondrial/metastases, iatrogenic, neurodegenerative, system/seizures/sarcoid, and demyelinating. When relevant, clinical, imaging, or other features of an RPD that overlap with those of CJD are presented.
Source: Handbook of Clinical Neurology - Category: Neurology Source Type: research

Related Links:

Anti‑osteopontin autoantibodies in various types of cancer. Oncol Rep. 2018 Oct 05;: Authors: Alsarkhi LK, Weber GF Abstract The aberrant processing of biomolecules by cancer cells may give rise to autoimmune phenomena. The metastasis gene osteopontin is alternatively spliced only in transformed cells, and the variants promote tumor progression. They may also serve as tumor‑associated antigens, and their neutralization by autoantibodies could favorably affect prognosis. A competitive solid‑phase ELISA format was applied to determine reactivity toward recombinant osteopontin in human serum or plas...
Source: Oncology Reports - Category: Cancer & Oncology Tags: Oncol Rep Source Type: research
Authors: Qiu L, Zhou Y, Yu Q, Yu J, Li Q, Sun R Abstract Early stratification of the severity of acute pancreatitis (AP) is clinically important. Regulatory B cells have been found to be associated with disease activity of autoimmune diseases. However, the role of Regulatory B cells in AP remains unknown. We investigate the dynamic longitudinal changes in circulating IL-10-producing B cells (B10) and memory CD19+CD24hiCD27hi cells in patients with AP to evaluate their prediction utility for AP severity. B10, CD19+CD24hiCD27hi cells, inflammatory markers and cytokines were detected in patients with AP immediately af...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
AbstractThe distinction that in areas where helminthic infections are common, autoimmune diseases are less prevalent, led to the investigation of immune modulatory properties of helminths and their derivatives. Such are phosphorylcholine (PC) moieties which are a component of secreted products of helminths. PC has been broadly studied for its attenuating effects on the human immune system. In an attempt to develop a novel therapeutic small molecule for the treatment of autoimmune conditions, we have conjugated PC with tuftsin, a natural immunomodulatory tetrapeptide, to create TPC. Herein, we review our findings regarding ...
Source: Immunologic Research - Category: Allergy & Immunology Source Type: research
AbstractEBV (Epstein-Barr Virus) and other human DNA viruses are associated with autoimmune syndromes in epidemiologic studies. In this work, immunoglobulin G response to EBV-encoded proteins which share regions with human immune response proteins from the human host including ZEBRA (BZLF-1 encoded protein), BALF-2 recombinase expressed primarily during the viral lytic replication cycle, and EBNA-1 (Epstein-Barr Virus Nuclear Antigen) expressed during the viral latency cycle respectively were characterized using a laser-printed micro-array (PEPperprint.com). IgG response to conserved “A/T hooks” in EBV-encoded ...
Source: Immunologic Research - Category: Allergy & Immunology Source Type: research
Publication date: Available online 13 December 2018Source: The Journal of Steroid Biochemistry and Molecular BiologyAuthor(s): Martin Kaufmann, Corine Martineau, Alice Arabian, Mary Traynor, René St-Arnaud, Glenville JonesAbstractCalcitroic acid, the excretory form of vitamin D, is the terminal product of a 5-step pathway catalyzed by CYP24A1, commencing with C24-hydroxylation of 1,25-dihydroxyvitamin D3 (1,25-(OH)2D3). Catabolism of 25-hydroxyvitamin D3 (25-OH-D3) proceeds via analogous steps culminating in calcioic acid; however this C23-truncated acid has not been reported in the circulation. It has recently been...
Source: The Journal of Steroid Biochemistry and Molecular Biology - Category: Biochemistry Source Type: research
In conclusion, one year or longer supplementation with a large daily, weekly or monthly dose of vitamin D2 /D3 did not significantly increase a risk of total adverse events or kidney stones, although there was a trend towards increased hypercalcemia, and possibly for hypercalciuria.Graphical abstract
Source: The Journal of Steroid Biochemistry and Molecular Biology - Category: Biochemistry Source Type: research
Authors: Bohn T Abstract Carotenoids are C-30, C-40 or C-50 terpenoids produced by a number of bacteria, fungi, and plants. In addition to acting as vitamin A precursors such as β-carotene, their dietary intake and blood plasma/serum and tissue levels have been associated in several epidemiological studies to the reduced incidence of chronic diseases, including the reduction of type 2 diabetes and other cardiometabolic diseases, as well as some types of cancer. Lutein and zeaxanthin also appear to play a role in the amelioration of age-related macular degeneration (AMD), the main cause of blindness in the elde...
Source: International Journal for Vitamin and Nutrition Research - Category: Nutrition Tags: Int J Vitam Nutr Res Source Type: research
Authors: Schadock I, Freitas BG, Moreira IL, Rincon JA, Nunes Correa M, Zanella R, Sampaio Silva E, Carvalho Araujo R, Buchweitz MRD, Helbig E, Del Vecchio FB, Schneider A, Castilho Barros C Abstract β-hydroxy-β-methyl butyrate (HMB) is a bioactive metabolite derived from the amino acid leucine, usually applied for muscle mass increase during physical training, as well as for muscle mass maintenance in debilitating chronic diseases. The hypothesis of the present study is that HMB is a safe supplement for muscle mass gain by strength training. Based on this, the objective was to measure changes in body com...
Source: International Journal for Vitamin and Nutrition Research - Category: Nutrition Tags: Int J Vitam Nutr Res Source Type: research
Dr. Sharon K. Inouye, a Milken Institute Center for the Future of Aging Longevity Innovator, talks about delirium and dementia and the aging brain.
Source: Forbes.com Healthcare News - Category: Pharmaceuticals Authors: Source Type: news
Women with depression, posttraumatic stress disorder, or traumatic brain injury are 50% to 80% more likely to develop dementia, suggests a new study of more than 100,000 US female veterans.Medscape Medical News
Source: Medscape Medical News Headlines - Category: Consumer Health News Tags: Neurology & Neurosurgery News Source Type: news
More News: Autoimmune Disease | Brain | Dementia | Genetics | Mitochondrial Disease | Neurology | Sarcoidosis | Toxicology | Vitamins