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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 Clinical Medicine Research Mar 2018Tonometry has been identified as a common method for measuring the intraocular pressure in patients. The direct contact between the tonometer and the eye may contribute... (Review)
Review
Tonometry has been identified as a common method for measuring the intraocular pressure in patients. The direct contact between the tonometer and the eye may contribute to the risk of cross infection, especially of viral particles, from one patient to another. A systematic review was undertaken to address the likelihood of human immunodeficiency virus (HIV), hepatitis B virus, hepatitis C virus, and prion diseases transmission through the use of tonometers. Additionally, a comparison of the current tonometer disinfection methods is provided to assist with identifying which technique effectively reduces the risk of disease transmission. An electronic literature search was conducted using the following databases: Web of Science, EMBASE, CINAHL, SCOPUS, Biosis Previews, Cochrane Library, PubMed, and Google Scholar. Dissertation indexes were also searched, and these included: Dissertations and Abstracts, and Dissertations and Abstracts - UK/Ireland. Additionally, the Clinicaltrials.gov trial registry was searched to identify any other relevant literature. Two independent reviewers critically appraised the articles retrieved through the literature search. In total, 11 unique studies were deemed relevant for this systematic review. The available evidence demonstrated that the use of tonometers contributes to the transmission of these infectious diseases . The results also demonstrated variability in determining the most effective tonometer sterilization technique against these infectious diseases . There was limited evidence available regarding the transmission of HIV, hepatitis B, hepatitis C, and prion diseases through the use of tonometers. Additionally, due to the variability regarding the most effective sterilization techniques, it is difficult to identify which sterilization technique is most effective or adequately effective against these infectious diseases. Future research studies regarding infectious disease transmission through tonometry and sterilization techniques should be completed to more adequately inform infectious disease control guidelines.
PubMed: 29416571
DOI: 10.14740/jocmr3294w -
Euro Surveillance : Bulletin Europeen... Oct 2017Sporadic Creutzfeldt-Jakob disease (sCJD) is potentially transmissible to humans. (Review)
Review
BACKGROUND
Sporadic Creutzfeldt-Jakob disease (sCJD) is potentially transmissible to humans.
OBJECTIVE
This study aimed to summarise and rate the quality of the evidence of the association between surgery and sCJD.
DESIGN AND METHODS
Firstly, we conducted systematic reviews and meta-analyses of case-control studies with major surgical procedures as exposures under study. To assess quality of evidence, we used the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. Secondly, we conducted a systematic review of sCJD case reports after sharing neurosurgical instruments.
RESULTS
Thirteen case-control studies met the inclusion criteria for the systematic review of case-control studies. sCJD was positively associated with heart surgery, heart and vascular surgery and eye surgery, negatively associated with tonsillectomy and appendectomy, and not associated with neurosurgery or unspecified major surgery. The overall quality of evidence was rated as very low. A single case-control study with a low risk of bias found a strong association between surgery conducted more than 20 years before disease onset and sCJD. Seven cases were described as potentially transmitted by reused neurosurgical instruments.
CONCLUSION
The association between surgery and sCJD remains uncertain. Measures currently recommended for preventing sCJD transmission should be strongly maintained. Future studies should focus on the potential association between sCJD and surgery undergone a long time previously.
Topics: Creutzfeldt-Jakob Syndrome; Female; Humans; Male; Middle Aged; Neurosurgical Procedures; Prion Diseases; Surgical Instruments; Surgical Procedures, Operative
PubMed: 29090678
DOI: 10.2807/1560-7917.ES.2017.22.43.16-00806 -
Movement Disorders : Official Journal... Jul 2017Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. (Review)
Review
BACKGROUND
Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes.
OBJECTIVE
To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP.
METHODS
We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in this cohort.
RESULTS
Of 4166 articles identified by the database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the following 4 functional domains: ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity.
CONCLUSIONS
Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. The features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP. © 2017 International Parkinson and Movement Disorder Society.
Topics: Humans; Supranuclear Palsy, Progressive
PubMed: 28500752
DOI: 10.1002/mds.27034 -
BMC Veterinary Research Aug 2016Chronic wasting disease (CWD) is a contagious, fatal prion disease affecting cervids in a growing number of regions across North America. Projected deer population... (Review)
Review
BACKGROUND
Chronic wasting disease (CWD) is a contagious, fatal prion disease affecting cervids in a growing number of regions across North America. Projected deer population declines and concern about potential spread of CWD to other species warrant strategies to manage this disease. Control efforts to date have been largely unsuccessful, resulting in continuing spread and increasing prevalence. This systematic review summarizes peer-reviewed published reports describing field-applicable CWD control strategies in wild deer populations in North America using systematic review methods. Ten databases were searched for peer-reviewed literature. Following deduplication, relevance screening, full-text appraisal, subject matter expert review and qualitative data extraction, nine references were included describing four distinct management strategies.
RESULTS
Six of the nine studies used predictive modeling to evaluate control strategies. All six demonstrated one or more interventions to be effective but results were dependant on parameters and assumptions used in the model. Three found preferential removal of CWD infected deer to be effective in reducing CWD prevalence; one model evaluated a test and slaughter strategy, the other selective removal of infected deer by predators and the third evaluated increased harvest of the sex with highest prevalence (males). Three models evaluated non-selective harvest of deer. There were only three reports that examined primary data collected as part of observational studies. Two of these studies supported the effectiveness of intensive non-selective culling; the third study did not find a difference between areas that were subjected to culling and those that were not. Seven of the nine studies were conducted in the United States.
CONCLUSIONS
This review highlights the paucity of evaluated, field-applicable control strategies for CWD in wild deer populations. Knowledge gaps in the complex epidemiology of CWD and the intricacies inherent to prion diseases currently pose significant challenges to effective control of this disease in wild deer in North America.
Topics: Animals; Deer; Disease Management; North America; Periodicals as Topic; Research; Wasting Disease, Chronic
PubMed: 27549119
DOI: 10.1186/s12917-016-0804-7