Epidemiology of amyotrophic lateral sclerosis in Southern Germany

The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture–recapture rate of >80% and population-based case–control study in ALS patients in South Western Germany. 1163 incidents of ALS were registered. Clinical and neuropsychological data were prospectively collected from 699 cases. The mean age at onset was 66.6 (SD = 11.6) years in prospective cases (N = 699). The site of onset was more frequently bulbar (34.1%) than lumbosacral (30.7%), cervical (27.0%), or thoracic (3.1%). Cognitive deficits (ranging from 27.5 to 42.1%, depending on the screening instrument) and behavioral changes (29%) were frequently detected. The incidence rate dropped markedly after 79 years of age, and bulbar onset as well as cognitive impairment were more frequent in ALS cases >75 years. The mean survival time of ALS cases from first paresis was 31 months. The age-standardized incidence rate (ASR) of ALS in 2012/2013 was found to be 2.4 (95% CI 2.2–2.7) per 100,000 person-years (resulting in an ASR of 3.1/100,000 with 100% coverage). Based on the predicted age distribution of the German population, the incidence of ALS was estimated to be 4.5/100,000 for men and 3.3/100,000 for women in the year 2050. ALS prevalence will rise to about 9.2–9.8/100,000 person-years in Germany in 2050. An increased proportion of patients with bulbar onset and/or cognitive deficits can be used as basic epidemiologic data on ALS for future health care decisions.     

Exploring the heterogeneity of neural social indices for genetically distinct etiologies of autism

Background

Autism spectrum disorder (ASD) is a genetically and phenotypically heterogeneous disorder. Promising initiatives utilizing interdisciplinary characterization of ASD suggest phenotypic subtypes related to specific likely gene-disrupting mutations (LGDMs). However, the role of functionally associated LGDMs in the neural social phenotype is unknown.

Methods

In this study of 26 children with ASD (n?=?13 with an LGDM) and 13 control children, we characterized patterns of mu attenuation and habituation as children watched videos containing social and nonsocial motions during electroencephalography acquisition.

Results

Diagnostic comparisons were consistent with prior work suggesting aberrant mu attenuation in ASD within the upper mu band (10–12 Hz), but typical patterns within the lower mu band (8–10 Hz). Preliminary exploration indicated distinct social sensitization patterns (i.e., increasing mu attenuation for social motion) for children with an LGDM that is primarily expressed during embryonic development. In contrast, children with an LGDM primarily expressed post-embryonic development exhibited stable typical patterns of lower mu attenuation. Neural social indices were associated with social responsiveness, but not cognition.

Conclusions

These findings suggest unique neurophysiological profiles for certain genetic etiologies of ASD, further clarifying possible genetic functional subtypes of ASD and providing insight into mechanisms for targeted treatment approaches.

     

Acute blood pressure response in hypertensive elderly women immediately after water aerobics exercise: A crossover study

Water aerobics exercise is widely recommended for elderly people. However, little is known about the acute effects on hemodynamic variables. Thus, we assessed the effects of a water aerobic session on blood pressure in hypertensive elderly women. Fifty hypertensive elderly women aged 67.8 ± 4.1 years, 1.5 ± 0.6 m high and BMI 28.6 ± 3.9 kg/m², participated in a crossover clinical trial. The experiment consisted of a 45-minute water aerobics session (70%–75% HRmax adjusted for the aquatic environment) (ES) and a control session (no exercise for 45 minutes) (CS). Heart rate was monitored using a heart rate monitor and systolic blood pressure (SBP) and diastolic (DBP) measurements were taken using a semi-automatic monitor before and immediately after the sessions, and at 10, 20 and 30 minutes thereafter. It was using a generalized estimating equation (GEE) with Bonferroni’s post-hoc test (p < 0.05). At the end of the experimental session, ES showed a rise in SBP of 17.4 mmHg (14.3%, p < 0.001) and DBP of 5.4 mmHg (7.8%, p < 0.001) compared to CS. At 10 minutes after exercise, BP declined in ES by a greater magnitude than in CS (SBP 7.5 mmHg, 6.2%, p = 0.005 and DBP 3.8 mmHg, 5.5%, p = 0.013). At 20 minutes after exercise and thereafter, SBP and DBP were similar in both ES and CS. In conclusion, BP returned to control levels within 10–20 minutes remaining unchanged until 30 minutes after exercise, and post-exercise hypotension was not observed. Besides, BP changed after exercise was a safe rise of small magnitude for hypertensive people.     

Impact of eculizumab treatment on paroxysmal nocturnal hemoglobinuria: a treatment versus no‐treatment study

Intravascular hemolysis in Paroxysmal nocturnal hemoglobinuria (PNH) can effectively be controlled with eculizumab, a humanized monoclonal antibody that binds complement protein C5. We report here a retrospective comparison study between 123 patients treated with eculizumab in the recent period (>2005) and 191 historical controls (from the French registry). Overall survival (OS) at 6 years was 92% (95%CI, 87 to 98) in the eculizumab cohort versus 80% (95%CI 70 to 91) in historical controls diagnosed after 1985 (HR 0.38 [0.15 to 0.94], P = 0.037). There were significantly fewer thrombotic events (TEs) in the group of patients treated with eculizumab (4% [1–10]) as compared to the historical cohort (27% [20–34]). However, we found that TEs may still occur after the initiation of eculizumab treatment and that previous TEs still have a negative impact on survival. Evolutions to myelodysplastic syndrome or acute leukemia were similar in both cohorts. There was less evolution to aplastic anemia in the treatment group. In multivariate analysis, absence of a previous TE and treatment with eculizumab were associated with a better OS. Treatment with eculizumab improves overall survival in classic PNH patients without increasing the risk of clonal evolution. Am. J. Hematol. 91:366–370, 2016. © 2016 Wiley Periodicals, Inc.     

Trends and predictions for gastric cancer mortality in Brazil

AIM: To analyze the effect of age-period and birth cohort on gastric cancer mortality, in Brazil and across its five geographic regions, by sex, in the population over 20 years of age, as well as make projections for the period 2010-2029.METHODS: An ecological study is presented herein,which distributed gastric cancer-related deaths in Brazil and its geographic regions. The effects of ageperiod and birth cohort were calculated by the Poisson regression model and projections were made with the age-period-cohort model in the statistical program R. RESULTS: Progressive reduction of mortality rates was observed in the 1980's, and then higher and lower mortality rates were verified in the 2000's, for both sexes, in Brazil and for the South, Southeast and Midwest regions. A progressive decrease in mortality rates was observed for the Northeast(both sexes) and North(men only) regions within the period 1995-1999, followed by rising rates. CONCLUSION: Regional differences were demonstrated in the mortality rates for gastric cancer in Brazil, and the least developed regions of the country will present increases in projected mortality rates.     

Propionibacterium acnes infections after cranial neurosurgery

BACKGROUND: Propionibacterium acnes (P. acnes) is a relatively avirulent organism that is part of the normal skin flora. Most patient isolates are considered contaminants but, in a small subset of patients, particularly in the post-neurosurgery setting, the organism can cause significant infections. We reviewed our experience with the occurrence and management of P. acnes infections after cranial neurosurgical procedures over a five-year period. METHODS: Patients with positive cultures for P. acnes between 1996 and 2001 were identified by review of the Saskatoon Health Region microbiology laboratory database. Of the 141 positive cultures, a review of hospital records identified six patients with P. acnes infections after neurosurgical procedures. A review of the literature related to P. acnes associated CNS infections was conducted. RESULTS: All patients had undergone a craniotomy or burrhole placement, and one patient had received prior radiotherapy. There were no P. acnes-related ventriculoperitoneal shunt infections. All patients presented with scalp swelling and three had purulent discharge. Symptoms occurred more than two months after the initial surgery in five of six patients, while one patient developed symptoms three years post-operatively. Management for all patients included removal of the craniotomy flap and treatment with parenteral antibiotics, followed in most cases by oral antibiotics. A good response without relapse of infection was seen in five patients; one patient had recurrent infection after cranioplasty. CONCLUSIONS: P. acnes is a rare but important cause of infection after craniotomy. Wound debridement, removal of the bone flap and adequate antibiotic coverage result in cure in the majority of patients.     

Recovery from paralysis in adult rats using embryonic stem cells

OBJECTIVE: We explored the potential of embryonic stem cell-derived motor neurons to functionally replace those cells destroyed in paralyzed adult rats. METHODS: We administered a phosphodiesterase type 4 inhibitor and dibutyryl cyclic adenosine monophosphate to overcome myelin-mediated repulsion and provided glial cell-derived neurotrophic factor within the sciatic nerve to attract transplanted embryonic stem cell-derived axons toward skeletal muscle targets. RESULTS: We found that these strategies significantly increased the success of transplanted axons extending out of the spinal cord into ventral roots. Furthermore, transplant-derived axons reached muscle, formed neuromuscular junctions, were physiologically active, and mediated partial recovery from paralysis. INTERPRETATION: We conclude that restoration of functional motor units by embryonic stem cells is possible and represents a potential therapeutic strategy for patients with paralysis. To our knowledge, this is the first report of the anatomical and functional replacement of a motor neuron circuit within the adult mammalian host.     

Moral judgment competence of medical students: a transcultural study

The authors conducted a cross-sectional short-term study using Lind’s Moral Judgment Test (MJT) to compare moral judgment competence (C-score) among students from a medical school in the Northeast region of Brazil and a medical school in the Northern region of Portugal. This study compares the C-scores of groups in the first and eighth semesters of study within each medical school and groups from corresponding semesters between the two medical schools. This study also evaluates the influence of such factors as age and gender on moral competence. A regression of moral judgment competence among the students in their eighth semester versus the students in the first semester of Brazilian medical school (p < 0.001) and a stagnation of moral competence among students in their eighth semester versus the first semester students in the Portuguese medical school (p = 0.06) were observed. For both the first semester and eighth semester groups, the students in the Portuguese medical school had higher C-scores than the students in the Brazilian medical school. In the analysis of the students’ performances in terms of MJT dilemmas, the phenomenon of “moral segmentation” was observed in all of the groups, and the students performed better on the worker’s dilemma than on the doctor’s dilemma. Among students in the same semester of study, older students had lower C-scores. There was generally no significant difference between men’s and women’s C-scores.     

The effect of polymerization cycles on porosity of microwave-processed denture base resin

STATEMENT OF PROBLEM: Although most of the physical properties of denture base resin polymerized by microwave energy have been shown to be similar to resins polymerized by the conventional heat polymerization method, the presence of porosity is a problem. PURPOSE: This study evaluated the effect of different microwave polymerization cycles on the porosity of a denture base resin designed for microwave polymerization. MATERIAL AND METHODS: Thirty-two rectangular resin specimens (65 x 40 x 5 mm) were divided into 3 experimental groups (A, B, and C; Onda-Cryl, microwave-polymerized resin) and 1 control group (T; Clássico, heat-polymerized resin), according to the following polymerization cycles: (A) 500 W for 3 minutes, (B) 90 W for 13 minutes+500 W for 90 seconds, (C) 320 W for 3 minutes+0 W for 4 minutes+720 W for 3 minutes, and (T) 74 degrees C for 9 hours. Porosity was calculated by measurement of the specimen volume before and after its immersion in water. Data were analyzed using 1-way analysis of variance (alpha=.05). RESULTS: The mean values and SDs of the percent mean porosity were: A=1.05%+/-0.28%, B=0.91%+/-0.15%, C=0.88%+/-0.23%, T=0.93%+/-0.23%. No significant differences were found in mean porosity among the groups evaluated. CONCLUSION: Within the limitations of this study, a denture base resin specifically designed for microwave polymerization tested was not affected by different polymerization cycles. Porosity was similar to the conventional heat-polymerized denture base resin tested.