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BMJ (Clinical Research Ed.) Jul 2000To determine the strength of association between history of blood transfusion and development of Creutzfeldt-Jakob disease. (Review)
Review
OBJECTIVE
To determine the strength of association between history of blood transfusion and development of Creutzfeldt-Jakob disease.
DATA SOURCES
English and non-English language articles published from January 1966 to January 1999 were retrieved using a keyword search of Medline and Embase. These were supplemented by handsearching key journals and searching bibliographies of reviews.
STUDY SELECTION
Two independent reviewers selected the relevant abstracts and articles. Articles were chosen that reported the results of case-control studies trying to identify rates of prior blood transfusion in patients with Creutzfeldt-Jakob disease and in controls.
DATA EXTRACTION
Odds ratios and information on study quality were extracted from the selected articles by two independent reviewers.
DATA SYNTHESIS
Five studies containing data on 2479 patients were included. Three of the five studies used medical or neurological patients as controls, the other two used population controls. Odds ratios for developing Creutzfeldt-Jakob disease from blood transfusion ranged from 0.54 to 0.89. Four of the five studies had confidence intervals that crossed 1.0. The combined odds ratio was 0.70 (95% confidence interval 0.54 to 0.89).
CONCLUSIONS
Case-control studies do not suggest a risk of developing Creutzfeldt-Jakob disease from blood transfusion. Rather, a trend seems to exist towards a lower frequency of previous blood transfusion in patients with Creutzfeldt-Jakob disease than in controls. However, it is important to be aware of these studies' methodological limitations-primarily the choice of control population and reliability of recall of transfusion status.
Topics: Case-Control Studies; Creutzfeldt-Jakob Syndrome; Humans; Risk Factors; Transfusion Reaction
PubMed: 10875826
DOI: 10.1136/bmj.321.7252.17 -
Brain and Nerve = Shinkei Kenkyu No... Aug 2009Prion diseases are fatal infectious neurodegenerative disorders; examples include the Creutzfeldt-Jakob disease affecting humans and bovine spongiform encephalopathy in... (Review)
Review
Prion diseases are fatal infectious neurodegenerative disorders; examples include the Creutzfeldt-Jakob disease affecting humans and bovine spongiform encephalopathy in cattle. The causative agents of these diseases--the prions--are thought to consist of the pathogenic isoform of the prion protein PrP(Sc), which is produced by the conformational conversion of the normal isoform PrP(c). Many lines of evidence indicate that the constitutive conversion of PrP(c) to PrP(Sc), resulting in a marked accumulation of PrP(Sc) in the brain, is a central event in the pathogenesis of prion diseases. A large number of compounds have been identified as anti-prion agents and capable of reducing the PrP(Sc) levels in infected cells. Some of these compounds have been found to be partially effective in infected animals, thus resulting in the prolongation of the incubation or survival times and a few of these compounds were or are under clinical trials. However, none of these compounds have proven to be therapeutically effective against this group of diseases. This is probably because (1) these compounds fail to cross the blood-brain barrier and (2) their effectiveness is reduced because they are administered only to patients with clinically advanced disease owing to a lack of diagnostic indicators for presymptomatic individuals. In this communication, we systematically list these anti-prion compounds and summarize their effectiveness and possible mechanisms of action.
Topics: Aminopyridines; Animals; Antibodies, Monoclonal; Blood-Brain Barrier; Brain; Cattle; Clinical Trials as Topic; Drug Discovery; Gene Silencing; Humans; Pentosan Sulfuric Polyester; Polyelectrolytes; Polymers; PrPC Proteins; PrPSc Proteins; Prion Diseases; Protein Isoforms; Quinacrine; Species Specificity
PubMed: 19697882
DOI: No ID Found -
Tremor and Other Hyperkinetic Movements... 2019Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described.
BACKGROUND
Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described.
METHODS
We performed a systematic review of case reports and case series of pathologically- and genetically confirmed prionopathies. Timing of symptom and movement disorder onset were documented. Continuous variables were compared between two groups using the Wilcoxon rank sum test and between multiple groups using Kruskal-Wallis test. Categorical variables were compared using Fisher's exact test.
RESULTS
A total of 324 cases were included in this analysis. Movement disorders were a common feature at the onset of symptoms in most prionopathies. Gait ataxia was present in more than half of cases in all types of prionopathies. The prevalence of limb ataxia (20%) and myoclonus (24%) was lower in Gerstmann-Sträussler-Scheinker disease compared to other prionopathies (p ≤ 0.004). Myoclonus was common but often a later feature in sporadic Creutzfeldt-Jakob disease (2 months before death). Chorea was uncommon but disproportionately prevalent in variant Creutzfeldt-Jakob disease (30% of cases; p < 0.001). In genetic Creutzfeldt-Jakob disease, E200K carriers exhibited gait and limb ataxia more often when compared to other mutation carriers.
DISCUSSION
Movement disorders are differentially present in the course of the various prionopathies. The movement phenomenology and appearance are associated with the type of prion disease and the genotype and likely reflect the underlying pattern of neurodegeneration. Reliance on myoclonus as a diagnostic feature of sporadic Creutzfeldt-Jakob disease may delay its recognition given its relatively late appearance in the disease course.
Topics: Humans; Movement Disorders; Mutation; Myoclonus; Prion Diseases
PubMed: 31871824
DOI: 10.7916/tohm.v0.712 -
Journal of Neuroimaging : Official... 2015Diagnosis of Creutzfeldt-Jakob disease during life can be challenging since the huge variability of the symptoms which can be observed, especially in its early stages,... (Review)
Review
Diagnosis of Creutzfeldt-Jakob disease during life can be challenging since the huge variability of the symptoms which can be observed, especially in its early stages, may simulate other common forms of dementia. In latest years, noninvasive techniques such as magnetic resonance, positron emission tomography, and single-photon emission tomography have been evaluated to help clinical neurologists to provide a definite diagnosis. We here provide a systematic review of the current knowledge of neuroimaging in CJD in order to establish the actual state of the art.
Topics: Brain; Creutzfeldt-Jakob Syndrome; Evidence-Based Medicine; Humans; Magnetic Resonance Imaging; Neuroimaging; Reproducibility of Results; Sensitivity and Specificity; Tomography, Emission-Computed
PubMed: 24593302
DOI: 10.1111/jon.12098 -
Surgical Endoscopy Jun 2005Concern has long existed regarding the possible iatrogenic spread of variant Creutzfeldt-Jakob disease (v-CJD) through surgery. This had been fueled by recent reports of... (Review)
Review
BACKGROUND
Concern has long existed regarding the possible iatrogenic spread of variant Creutzfeldt-Jakob disease (v-CJD) through surgery. This had been fueled by recent reports of bovine spongiform encephalopathy in U.S. cattle and the first probable case of blood transmission of v-CJD in the UK.
METHODS
Systematic review of experimental and nonexperimental studies. Studies identified from searches of Medline, Embase, Cochrane Library, Science Citation Index medical databases, searching bibliographies of retrieved papers, and personal communication with international experts in the field.
RESULTS
Six articles satisfied our search criteria. Evidence stems from case reports, case series, and cross-sectional studies. There are no published cases of surgically transmitted v-CJD.
CONCLUSION
We found evidence of v-CJD prion agents in the spleen, appendix, rectum, and adrenal glands of affected patients and evidence of v-CJD prion in the appendix of patients in the preclinical stage of the disease. The risk of transmission of v-CJD prion during abdominal surgery is currently unquantifiable.
Topics: Abdomen; Creutzfeldt-Jakob Syndrome; Endoscopy; Equipment Contamination; Humans; Lymphoid Tissue; Mononuclear Phagocyte System; Risk Factors; Surgical Procedures, Operative
PubMed: 15868249
DOI: 10.1007/s00464-004-9205-2 -
The Journal of Trauma Jun 2006Both fresh frozen plasma (FFP) and platelets are heavily used in massive transfusion. Although FFP can partially correct abnormal coagulation, a recent systematic review... (Review)
Review
Both fresh frozen plasma (FFP) and platelets are heavily used in massive transfusion. Although FFP can partially correct abnormal coagulation, a recent systematic review revealed no randomized trials showing clinical benefit. Although the overall risks of FFP and platelets are low, they are the least safe blood components, due to immunologic reactions such as allergy/anaphylaxis, transfusion-related acute lung injury (TRALI) and hemolysis due to anti-A or anti-B if transfused across ABO groups. TRALI, an acute syndrome of dyspnoea, hypoxia and pulmonary 'white-out' is now a major cause of transfusion-related death. Since it is usually triggered by donor HLA antibodies, selecting non- immune donors for FFP production may be beneficial. For platelet components, risks may be reduced by platelet additive solution, allowing removal of 70% of plasma. Platelets have the additional hazard of bacterial contamination, with donor skin the predominant source. Improved arm cleansing, divert pouches for the first 30-50 mL blood and bacterial screening have been adopted internationally. Virus risks are now vanishingly low, although new agents e.g. West Nile virus can still appear. Pathogen reduction for FFP is now well established in Europe, with solvent detergent and methylene blue methods licensed, and the psoralen amotosalen in trial. Loss of clotting factors and natural anti-coagulants are recognized side effects. Amotosalen is also licensed for platelet concentrates, with the added benefit of bacterial killing. In the UK, concern regarding vCJD has led to importation of US FFP for children.
Topics: Bacterial Infections; Blood Component Transfusion; Creutzfeldt-Jakob Syndrome; Cytomegalovirus Infections; Hemolysis; Humans; Hypersensitivity; Respiratory Distress Syndrome; Wounds and Injuries
PubMed: 16763481
DOI: 10.1097/01.ta.0000199546.22925.31 -
International Journal of Molecular... Mar 2020Alzheimer's disease (AD) and Parkinson's disease (PD) are the most common neurodegenerative diseases (NDs), presenting a broad range of symptoms from motor dysfunctions...
Alzheimer's disease (AD) and Parkinson's disease (PD) are the most common neurodegenerative diseases (NDs), presenting a broad range of symptoms from motor dysfunctions to psychobehavioral manifestations. A common clinical course is the proteinopathy-induced neural dysfunction leading to anatomically corresponding neuropathies. However, current diagnostic criteria based on pathology and symptomatology are of little value for the sake of disease prevention and drug development. Overviewing the pathomechanism of NDs, this review incorporates systematic reviews on inflammatory cytokines and tryptophan metabolites kynurenines (KYNs) of human samples, to present an inferential method to explore potential links behind NDs. The results revealed increases of pro-inflammatory cytokines and neurotoxic KYNs in NDs, increases of anti-inflammatory cytokines in AD, PD, Huntington's disease (HD), Creutzfeldt-Jakob disease, and human immunodeficiency virus (HIV)-associated neurocognitive disorders, and decreases of neuromodulatory KYNs in AD, PD, and HD. The results reinforced a strong link between inflammation and neurotoxic KYNs, confirmed activation of adaptive immune response, and suggested a possible role in the decrease of neuromodulatory KYNs, all of which may contribute to the development of chronic low grade inflammation. Commonalities of multifactorial NDs were discussed to present a current limit of diagnostic criteria, a need for preclinical biomarkers, and an approach to search the initiation factors of NDs.
Topics: Alzheimer Disease; Anti-Inflammatory Agents; Biomarkers; Cytokines; Humans; Huntington Disease; Inflammation; Kynurenine; Neurodegenerative Diseases; Parkinson Disease; Reactive Oxygen Species; Tryptophan
PubMed: 32244523
DOI: 10.3390/ijms21072431 -
Cells Jan 2021Corticobasal syndrome (CBS) is an atypical parkinsonian presentation characterized by heterogeneous clinical features and different underlying neuropathology. Most CBS...
Corticobasal syndrome (CBS) is an atypical parkinsonian presentation characterized by heterogeneous clinical features and different underlying neuropathology. Most CBS cases are sporadic; nevertheless, reports of families and isolated individuals with genetically determined CBS have been reported. In this systematic review, we analyze the demographical, clinical, radiological, and anatomopathological features of genetically confirmed cases of CBS. A systematic search was performed using the PubMed, EMBASE, and Cochrane Library databases, included all publications in English from 1 January 1999 through 1 August 2020. We found forty publications with fifty-eight eligible cases. A second search for publications dealing with genetic risk factors for CBS led to the review of eight additional articles. was the most common gene involved in CBS, representing 28 out of 58 cases, followed by , and . A set of symptoms was shown to be significantly more common in -CBS patients, including visuospatial impairment, behavioral changes, aphasia, and language alterations. In addition, specific demographical, clinical, biochemical, and radiological features may suggest mutations in other genes. We suggest a diagnostic algorithm to help in identifying potential genetic cases of CBS in order to improve the diagnostic accuracy and to better understand the still poorly defined underlying pathogenetic process.
Topics: Age of Onset; Aged; Aphasia; C9orf72 Protein; Female; Genetic Association Studies; Genetic Predisposition to Disease; Humans; Language Disorders; Male; Mental Disorders; Middle Aged; Mutation; Neurodegenerative Diseases; Parkinsonian Disorders; Prion Proteins; Progranulins; Syndrome; Treatment Outcome; Vision Disorders; tau Proteins
PubMed: 33467748
DOI: 10.3390/cells10010171 -
The Journal of International Medical... May 2024To systematically review the reported cases of Creutzfeldt-Jakob disease (CJD) in Iran.
OBJECTIVE
To systematically review the reported cases of Creutzfeldt-Jakob disease (CJD) in Iran.
METHODS
A comprehensive literature review of CJD cases in Iran was undertaken using the PubMed®, Scopus® and Google Scholar databases. In addition, the Iranian database MagIran was searched for Persian language reports. Case selection used the following criteria: (i) patients of Iranian origin; (ii) publication in peer-reviewed journals or reputable medical databases; (iii) a definitive diagnosis of CJD based on established diagnostic criteria.
RESULTS
Thirteen cases from twelve reports were included in this systematic review. The majority of the cases were female (11 of 13; 84.6%). The mean ± SD age of patients at hospital admission was 59.38 ± 7.44 years. The findings of the case review suggested that the prevalence of CJD in Iran is not fully established. CJD may be misdiagnosed alongside other clinical signs. The most prevalent early indications of the disease were psychiatric and neurological in nature. A considerable delay in diagnosis was observed in some cases and there was a shortage of brain autopsy records.
CONCLUSION
Efforts to improve diagnostic capabilities, promote awareness and establish monitoring systems are necessary for managing the challenges of providing an early diagnosis of CJD in Iran.
Topics: Creutzfeldt-Jakob Syndrome; Humans; Iran; Female; Male; Middle Aged; Aged; Brain; Prevalence
PubMed: 38717041
DOI: 10.1177/03000605241247706 -
Connecticut Medicine 2007While the cause of sporadic Creutzfeldt-Jakob disease (sCJD) is unknown, case clustering could suggest infectious transmission in some instances. (Review)
Review
BACKGROUND
While the cause of sporadic Creutzfeldt-Jakob disease (sCJD) is unknown, case clustering could suggest infectious transmission in some instances.
METHODS
Publications of sCJD clusters were systematically identified and pooled to determine if their clinical, epidemiologic, and genetic features reflected potential infectious clustering mechanisms.
RESULTS
The search yielded five clusters involving 23 cases. Most notably, among 12 cases with reported residential histories, patients lived for a median of 30.0 years (25th percentile: 26.5 years; 75th percentile: 51.0 years) in their respective cluster regions. Of 19 cases that underwent genotyping for polymorphic codon 129, seventeen were methionine homozygotes (89.5%; 95% CI 67.4-98.3%); none were heterozygous.
CONCLUSIONS
Prolonged residency duration and Prnp codon 129 methionine homozygosity may be consistent with underlying disease-clustering mechanisms, although larger, controlled investigations might help to exclude the possibility that these findings reflect the normal distribution of these features among relevant comparison populations.
Topics: Cluster Analysis; Creutzfeldt-Jakob Syndrome; Genotype; Humans; Residence Characteristics; Risk Factors; Time Factors
PubMed: 17619468
DOI: No ID Found