Miracle-dz/UniFAR-FTUs huggingface dataset:
Unified Huntington's disease rating scale for advanced patients: Validation and follow‐up study

The Unified Huntington's Disease Rating Scale (UHDRS) adequately measures decline in patients at early and moderate stages of Huntington's disease (HD). In advanced patients, floor effects hamper the evaluation, thus calling for an adjusted scale. We designed the UHDRS‐For Advanced Patients (UHDRS‐FAP), in order to improve longitudinal assessment of patients at advanced disease stage. Sixty‐nine patients with a Total Functional Capacity (TFC) ≤ 5 were recruited in France and in the Netherlands. Among them, 45 patients were followed longitudinally (mean 1.6 ± 1.2 years) with the UHDRS‐FAP; 30 were also assessed with the UHDRS. Cross‐sectional analyses evaluated psychometric properties and interrater reliability of the scale. Longitudinal analyses evaluated the sensitivity to decline compared to the UHDRS. Internal consistency was higher for motor and cognitive scores than for somatic and behavioral scores (0.84, 0.91, 0.70, and 0.49, respectively). Interrater reliability was ≥ 0.88 in all scores. The somatic score, specific to the UHDRS‐FAP, declined over time, as well as motor and cognitive performance with both scales. Although performance with the 2 scales correlated, the UHDRS‐FAP appeared more sensitive to change and was the only scale that detected decline in patients with a TFC ≤ 1. Neither scale detected a significant decline in behavioral scores. The UHDRS‐FAP is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains. It offers items relevant for daily care. Behavioral scores tended to decline but this may reflect the decline in the communicative abilities of the patients. © 2013 International Parkinson and Movement Disorder Society
REPARATIVE PROCESSES IN THE MEMBRANA TYMPANI: SOME INTERESTING MANIFESTATIONS

EPIDERMAL ESCALATION The ability of the membrana tympani to reconstruct itself is exceeded only by its failure to do so in some instances. In the same hour I have observed a minute perforation of several years' duration, and a "new growth" membrane which had formed after a radical operation on the mastoid, the membrane extending from the anterior wall of the canal to the posterior wall of the mastoid cavity, completely partitioning the tympanum, with a surface area about equal to that of a 5 cent piece. This membrane was perfectly transparent, apparently consisting only of the epidermal layer, growing posteriorly from a vestige of the anterior border, as proliferation proceeds only in this direction. How so frail and poorly nourished a reconstruction was possible when the minute perforation consisting of all the layers with an abundant blood supply failed to close can be explained on the basis of
Tracking motor impairments in the progression of Huntington's disease

The Unified Huntington's Disease Rating Scale is used to characterize motor impairments and establish motor diagnosis. Little is known about the timing of diagnostic confidence level categories and the trajectory of motor impairments during the prodromal phase. Goals of this study were to estimate the timing of categories, model the prodromal trajectory of motor impairments, estimate the rate of motor impairment change by category, and provide required sample size estimates for a test of efficacy in clinical trials. In total, 1010 gene‐expanded participants from the Neurobiological Predictors of Huntington's Disease (PREDICT‐HD) trial were analyzed. Accelerated failure time models were used to predict the timing of categories. Linear mixed effects regression was used to model the longitudinal motor trajectories. Age and length of gene expansion were incorporated into all models. The timing of categories varied significantly by gene expansion, with faster progression associated with greater expansion. For the median expansion, the third diagnostic confidence level category was estimated to have a first occurrence 1.5 years before diagnosis, and the second and first categories were estimated to occur 6.75 years and 19.75 years before diagnosis, respectively. Motor impairments displayed a nonlinear prodromal course. The motor impairment rate of change increased as the diagnostic confidence level increased, with added acceleration for higher progression scores. Motor items can detect changes in motor impairments before diagnosis. Given a sufficiently high progression score, there is evidence that the diagnostic confidence level can be used for prodromal staging. Implications for Huntington's disease research and the planning of clinical trials of efficacy are discussed. © 2013 International Parkinson and Movement Disorder Society
The unified huntington's disease rating scale for advanced patients: Validation and follow‐up study

The Unified Huntington's Disease Rating Scale (UHDRS) adequately measures decline in patients at early and moderate stages of Huntington's disease (HD). In patients with advanced HD, floor effects hamper the evaluation, thus calling for an adjusted scale. We designed the UHDRS‐For Advanced Patients (UHDRS‐FAP) to improve longitudinal assessment of patients at the advanced disease stage. Sixty‐nine patients with a Total Functional Capacity score ≤ 5 were recruited in France and the Netherlands. Among them, 45 patients were followed longitudinally (mean ± standard deviation, 1.6 ± 1.2 years) with the UHDRS‐FAP; 30 patients also were assessed with the UHDRS. In cross‐sectional analyses, the psychometric properties and inter‐rater reliability of the scale were evaluated. Longitudinal analyses were used to evaluate the sensitivity to decline of the UHDRS‐FAP compared with the UHDRS. Internal consistency was higher for motor (0.84) and cognitive (0.91) scores than for somatic (0.70) and behavioral (0.49) scores. Inter‐rater reliability was ≥ 0.88 for all scores. The somatic score, which was specific to the UHDRS‐FAP, declined over time along with motor and cognitive performance on both scales. Although performance with the two scales was correlated, the UHDRS‐FAP appeared to be more sensitive to change and was the only scale that detected decline in patients with a Total Functional Capacity score ≤ 1. Neither scale detected a significant decline in behavioral scores. The results indicate that the UHDRS‐FAP is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains. It offers items that are relevant for daily care. Behavioral scores tended to decline, but this may reflect the decline in patients' communicative abilities. © 2013 International Parkinson and Movement Disorder Society
Prevalence, diversity, and interaction patterns of avian haemosporidians in a four-year study of blackcaps in a migratory divide

SUMMARY Migratory birds contribute to the movement of avian parasites between distant locations, thereby influencing parasite distribution and ecology. Here we analyse the prevalence, diversity and interaction patterns of Haemosporida parasites infecting Blackcap (Sylvia atricapilla) populations in a recently established migratory divide of southwestern Germany across 4 years. We hypothesize that the temporal and spatial isolation provided by 2 sympatric Blackcap breeding populations (migratory divide) might modify ecological interactions and thus create differences in the structure of the parasite community according to migratory route. We used a fragment of the mitochondrial DNA cytochrome b gene to determine haemosporidian haplotypes. We detected an overall infection prevalence of 70·3% (348 out of 495 blackcaps sampled from 2006 to 2009), and prevalence rates were significantly different among years and seasons. We observed a total of 27 parasite haplotypes infecting blackcaps, from them 6 new rare Haemoproteus haplotypes were found in 2 mixed infections. H. parabelopolskyi haplotypes SYAT01 (35·7%) and SYAT02 (20·8%) comprised most of the infections. An association analysis suggests that SYAT01 and SYAT02 are interacting negatively, implying that they are either competing directly for host resources, or indirectly by eliciting a cross-immune response. Molecular data show no clear difference between the parasite communities infecting blackcaps with different migratory routes, despite some temporal and spatial isolation between the two sympatric blackcap populations.
Do molecules matter more than morphology? Promises and pitfalls in parasites

SUMMARY Systematics involves resolving both the taxonomy and phylogenetic placement of organisms. We review the advantages and disadvantages of the two kinds of information commonly used for such inferences – morphological and molecular data – as applied to the systematics of metazoan parasites generally, with special attention to the malaria parasites. The problems that potentially confound the use of morphology in parasites include challenges to consistent specimen preservation, plasticity of features depending on hosts or other environmental factors, and morphological convergence. Molecular characters such as DNA sequences present an alternative data source and are particularly useful when not all the parasite's life stages are present or when parasitaemia is low. Nonetheless, molecular data can bring challenges that include troublesome DNA isolation, paralogous gene copies, difficulty in developing molecular markers, and preferential amplification in mixed species infections. Given the differential benefits and shortcomings of both molecular and morphological characters, both should be implemented in parasite taxonomy and phylogenetics.
Effects of Anticoagulants in Patients With Cirrhosis and Portal Vein Thrombosis: A Systematic Review and Meta-analysis.

BACKGROUND & AIMS Liver cirrhosis is complicated by bleeding from portal hypertension but also by portal vein thrombosis (PVT). PVT occurs in approximately 20% to 50% of patients with cirrhosis, and is a warning sign for poor outcome. It is a challenge to treat patients with cirrhosis using anticoagulants, because of the perception that the coexistent coagulopathy could promote bleeding. We performed a systematic review and meta-analysis to determine the effects of anticoagulant therapy in patients with cirrhosis and PVT. METHODS We searched the PubMed, ISI Web of Science, SCOPUS, and Cochrane databases through February 14, 2017, for studies that assessed the effect of anticoagulant therapy vs no treatment in patients with cirrhosis and PVT. We performed a meta-analysis to estimate the effect of anticoagulant treatment vs no therapy on recanalization and progression of PVT in patients with cirrhosis. We also assessed variceal and nonvariceal bleeding. RESULTS We analyzed data from 8 studies, comprising 353 patients, that assessed the effects of anticoagulant therapy (low-weight heparin or warfarin vs no therapy) in patients with cirrhosis and PVT; these studies reported rates of complete and partial recanalization. A significantly higher proportion of patients treated with anticoagulants underwent PVT recanalization than patients who did not receive anticoagulants (71% vs 42%, respectively; P < .0001). From 6 studies (comprising 217 patients), 53% of patients treated with anticoagulants vs 33% of patients who did not receive anticoagulants had complete PVT recanalization (P = .002). From 6 studies (comprising 225 patients), PVT progressed in 9% of patients treated with anticoagulants vs 33% of patients who did not receive these drugs (P < .0001). Six studies (257 patients) reported rates of any bleeding; there was no difference in the proportions of patients with major or minor bleeding between groups that did vs did not receive anticoagulants (11% for both groups). Four studies (comprising 158 patients) reported rates of spontaneous variceal bleeding, which occurred in a significantly lower proportion of patients who received anticoagulants vs those who did not (P = .04). CONCLUSIONS Based on a systematic review and meta-analysis, patients with cirrhosis and PVT who receive anticoagulant therapy have increased recanalization and reduced progression of thrombosis, compared with patients who do not receive anticoagulants, with no excess of major and minor bleedings and less incidence of variceal bleeding.
Apolipoprotein E &egr;4 and short-term recovery from predominantly mild brain injury

ObjectiveTo determine whether APOE genotype explained variability in short-term recovery from predominantly mild traumatic brain injury (TBI). MethodsA total of 87 adult patients presenting with mild or moderate TBI to a shock trauma center were enrolled prospectively. A battery of 13 neuropsychological tests was administered twice, at approximately 3 and 6 weeks after injury. Eighty of 87 patients were successfully genotyped for APOE using a buccal swab technique. ResultsNinety percent of study patients had mild TBI (Glasgow Coma Scale score of 13 to 15); 18 (22.5%) had one APOE &egr;4 and none had two &egr;4 alleles. After adjusting for potential confounders, patients positive for the APOE &egr;4 allele had lower mean scores on 12 of 13 neuropsychological outcomes at visit 1 compared with APOE &egr;4-negative patients. Two of the differences were significant (grooved pegboard test, p = 0.005; paced auditory serial addition task 2.8-second trial, p = 0.004). At visit 2, APOE &egr;4-positive patients had lower adjusted mean scores on 11 of the 13 neuropsychological outcomes. None of the differences was significant. ConclusionsAPOE genotype may influence the severity of the acute injury. However, with no consistent pattern to the recovery curves, it is not clear if APOE genotype influences the rate of recovery.
Interval Colorectal Cancer Incidence Among Subjects Undergoing Multiple Rounds of Fecal Immunochemical Testing.

BACKGROUND & AIMS Among subjects screened for colorectal cancer (CRC) by the guaiac fecal occult blood test, interval cancers develop in 48% to 55% of the subjects. Data are limited on how many persons screened by fecal immunochemical tests (FIT), over multiple rounds, develop interval cancers. In the Netherlands, a pilot FIT-based biennial CRC screening program was conducted between 2006 and 2014. We collected and analyzed data from the program on CRCs detected during screening (SD-CRC) and CRCs not detected within the screening program (non-SD-CRC; such as FIT interval cancers, colonoscopy interval cancers, and cancer in nonparticipants). METHODS Screenees with a negative FIT result received a letter explaining that no blood had been detected in the stool sample and were re-invited, if eligible, for screening biennially. Screenees with a positive FIT result (hemoglobin concentration of 10 μg Hb/g feces) were invited for consultation and scheduled for colonoscopy; results were collected. After the fourth round of FIT screening, the cohort was linked to the Netherlands Cancer Registry, through March 31, 2015; participant characteristics, data on tumor stage, location (at time of resection), and survival status were collected for all identified CRC cases. A reference group comprised all persons with CRC diagnosed in the Netherlands general population during the same period, in the same age range (50-76 years), who had not been offered CRC screening. The median time between invitations (2.37 years) was used as a cutoff to categorize participants within the FIT interval cancer category. We compared participant characteristics, tumor characteristics, and mortality among subjects with SD-CRC and with non-SD-CRC. RESULTS A total of 27,304 eligible individuals were invited for FIT screening, of whom 18,716 (69%) participated at least once. Of these, 3005 (16%) had a positive result from the FIT in 1 of the 4 screening rounds. In total, CRC was detected in 261 participants: 116 SD-CRCs and 145 non-SD-CRCs (27 FIT interval cancers, 9 colonoscopy interval cancers, and 109 CRCs in nonparticipants). The FIT interval cancer proportion after 3 completed screening rounds was 23%. Participants with SD-CRC had more early-stage tumors than participants with non-SD-CRCs (P < .001). Of persons with SD-CRC and FIT interval cancers, significantly higher proportions survived (89% and 81%, respectively) compared with persons with colonoscopy interval cancers (44% survival) and nonparticipants with CRC (60% survival) (P < .001). CONCLUSIONS In an analysis of data from a pilot FIT-based biennial screening program, we found that among persons screened by FIT, 23% developed FIT interval cancer. FIT therefore detects CRC with 77% sensitivity. The proportion of FIT interval cancers in FIT screening appears to be lower than that with guaiac fecal occult blood testing. Clinical trial registry: yes, www.trialregister. nl, trial number: NTR5385.
Initiating levodopa therapy for Parkinson's disease

We thank Dr. Rajput for his kind comments on our recent article and are well aware of his early recognition of the importance of low-dose levodopa (L-dopa). Since that time, several community-based studies have recognized the relationship between the risk of developing dyskinesia and L-dopa dose, but these were not prospective or double-blind. The Earlier Versus Later Levodopa (ELLDOPA) trial made a similar observation in a prospective dosing study, but that trial was of relatively short duration (9 months). Our study was prospective, double-blind, relatively long in duration (up to 4 years), and emphasized that L-dopa dose was related to the development of both dyskinesia and wearing off. In our study, participants were permitted to increase the dose in order to obtain optimal symptom control, and it is not possible to say with certainty that disease severity was not a contributing factor. However, the Unified Parkinson’s Disease Rating Scale (UPDRS) part III (motor section) score was not a risk factor for dyskinesia, and several analyses, including the C-statistic, suggested that L-dopa dose was an independent risk factor for both wearing off and dyskinesia. It is not intuitive that higher doses of Ldopa should be associated with a greater risk of wearing off. It would be ideal to perform a long-term, double-blind dosing study, but it is not at all certain that such a study will ever be performed. For the present, we believe our data offer the best information available for informing physicians on the risks associated with the development of motor complications and the potential role of L-dopa dose. C. Warren Olanow, MD*
Prospective study of obesity, hypertension, high cholesterol, and risk of restless legs syndrome

Because previous cross‐sectional studies suggest an association between metabolic disorders and restless legs syndrome (RLS), we prospectively evaluated whether obesity, hypercholesterolemia, and hypertension were associated with increased risk of RLS. Our study consisted of 42,728 female participants from the Nurses' Health Study II and 12,812 male participants from the Health Professionals Follow‐up Study, free of RLS at baseline (2002 for men and 2005 for women), and free of diabetes and arthritis through follow‐up (2002‐2008 for men and 2005‐2009 for women). RLS symptoms were assessed using the International RLS Study Group's standardized questionnaire. We considered RLS symptoms a “case” if the symptoms occurred ≥5 times/month and met International RLS Study Group criteria. We found that obesity was associated with an increased risk RLS among both men and women (P difference for sex >0.5). The pooled multivariate‐adjusted odds ratio (OR) for RLS was 1.57 (95% confidence interval [CI]: 1.33‐1.85; P trend <0.0001) for body mass index >30 versus ≤23 kg/m2 and 1.56 (95% CI: 1.29‐1.89; P trend = 0.0001) comparing two extreme waist circumference quintiles, adjusting for age, ethnicity, smoking, physical activity, use of antidepressant, and other covariates. A similar significant association was found for high cholesterol; the pooled adjusted OR for total serum cholesterol >240 versus <159 mg/dL was 1.33 (95% CI: 1.11‐1.60; P trend = 0.002). There was no significant association between hypertension and RLS risk (adjusted OR: 0.90; 95% CI: 0.79‐1.02). In this large, prospective study, we found that obesity and high cholesterol, but not high blood pressure, were significantly associated with an increased risk of developing RLS. © 2014 International Parkinson and Movement Disorder Society
FOOD ADVERTISING ON IRANIAN CHILDREN’S TELEVISION: A CONTENT ANALYSIS AND AN EXPERIMENTAL STUDY WITH JUNIOR HIGH SCHOOL STUDENTS KEYWORDS

Nature of food advertising during children’s television in Iran was examined in two parts. Part 1 - a content analysis study, monitored 13 hours of children’s TV programs broadcast on Channel 1, for 1 week (November 4–10, 2005) to analyze whether the messages delivered in food advertisements were consistent with dietary recommendation. Results showed food products comprised 25% in number and 21% in duration of advertising for the monitored period. “Ashimashi’s” puffed cereals comprised the largest category (36%) of advertised foods. The message most frequently used to promote the sale of a product was “taste” (56%). The most frequent appeal of food advertisements was “attributed quality” (67%). Half of nutritional messages of the advertisements were scientifically untrue or misleading. Part 2- simultaneously performed with Part 1, was an investigation with 398 (198 girls; 198 boys) junior high school students from six state and six private junior high schools in a middle class district of Tehran. The students were interviewed to determine which food advertisement they remembered mostly. “Ashimashi’s” puffed cereals was the most recalled advertisement by the students. These findings show that food advertisements directed at children are not based on sound nutritional principles and therefore are misleading.
Thin‐slice T2 MRI imaging predicts vascular pathology in hemifacial spasm: A case‐control study

Hemifacial spasm (HFS) is a condition that may severely reduce patients' quality of life. We sought to determine the sensitivity and specificity of thin‐slice T2 magnetic resonance imaging (MRI) for detecting vascular compression in HFS patients. Prospective information was collected on 28 patients with HFS who presented to our center between March 2011 and March 2012 with thin‐slice T2 MR imaging. The sensitivity and specificity for differentiating patients from controls were calculated. Sensitivities were 78.6% and 92.9% for the blinded radiologists and 75% for the partially blinded neurosurgeon. Specificities were 42.9% and 28.6% for the blinded radiologists and 75% for the partially blinded neurosurgeon. Magnetic resonance imaging of the facial nerve can guide clinicians in selecting patients who are good surgical candidates. Thin‐slice T2 MRI should be viewed as supportive rather than diagnostic. © 2014 International Parkinson and Movement Disorder Society
Long-Term Outcome of Liver Transplantation in HIV-1-Positive Patients: 15-Year Follow-Up

Liver transplantation (LT) for patients with human immunodeficiency virus type-1 (HIV-1) infection has been associated with poor outcome. However, after the introduction of the highly active antiretroviral therapy, short-term patient survival after LT has improved significantly. We examined the long-term outcome of HIV-1-positive patients who underwent LT. Medical records were analysed in nine HIV-1-positive LT patients who underwent LT from August 1998 to May 2012. Eight were known to be HIV-1 positive at the time of listing for LT and had end-stage liver disease (ESLD) due to hepatitis C. One patient had primary biliary cirrhosis, and primary HIV-1 infection was found at the date of LT. Seven of the nine patients remain alive to date. So far, three have survived more than 12 years after LT. The overall patient survival rate for both five and 10 years is 77.8%. Four patients experienced acute rejection and six acquired biopsy-confirmed HCV recurrence. HIV-1 replication was effectively blocked during follow-up in all patients. We conclude that long-term survival of HIV-1-positive patients after LT can be achieved. Our study suggests that LT can offer an effective treatment option in selected HIV-1 infected patients with ESLD.