关键词： Goat   Poisoning   Ipomoea trifida   Ipomoea cornea   Calystegines   Nicaragua  

摘要： A disease characterized by ataxia, tremors and nystagmus had been observed in goats in Nicaragua. The main histologic lesions were loss and neuronal vacuolation of Purkinje cells and Wallerian-like degeneration mainly in the cerebellum, suggesting a glycoprotein storage disease. Ipomoea cornea and Ipomoea trifida found in the paddocks were negative for swainsonine, but contained calystegines at 0.02% and 0.06% suggesting that the disease was caused by these substances, which are competitive inhibitors of beta-glucosidase and alpha-galactosidase activities.

关键词： Smoking   Pregnancy   Follow up   Long term   Neurological morbidity   Hospitalization  

摘要： Objective: To evaluate the long-term pediatric neurological morbidity of children born to mothers who reported smoking during pregnancy as compared with children born to non-smoking mothers. Study design: A population-based cohort analysis was performed comparing all deliveries of mothers who reported smoking during pregnancy and non-smoking mothers between 1991 and 2014 at a single tertiary medical center. Hospitalizations of the offspring up to the age of 18 years involving neurological morbidities were evaluated according to a predefined set of ICD-9 codes, including autism, development and movement disorders, cerebral palsy and more. A Kaplan-Meier survival curve was used to compare cumulative hospitalization rate in exposed and unexposed offspring and a Cox proportional hazards model was used to control for confounders. Results: During the study period, 242,342 deliveries met the inclusion criteria. Of them, 2861 (1.2%) were children of smoking mothers. Neurological-related hospitalizations were significantly higher in children born to smoking mothers, as compared with the non-smoking group (5.3% vs. 3.1%, p < 0.01). Specifically, these children had higher rates of movement, eating and developmental disorders as well as attention deficit hyperactive disorder. The Kaplan-Meier survival curve demonstrated a significant higher cumulative incidence of neurological-related hospitalizations in the smoking group (log rank p < 0.01). Using a Cox proportional hazards model, controlling for potential confounders, maternal tobacco use was found to be independently associated with long-term neurological morbidity of the offspring (adjusted HR = 1.58, CI 1.33-1.89, p value < 0.01). Conclusion: Maternal smoking during pregnancy is an independent risk factor for long-term neurological morbidity of the offspring.

关键词： best practice   clinical practice guideline   mixed methods   multiple sclerosis   online survey   palliative care   patient and public involvement  

摘要： Background and purpose: Patient and public involvement in clinical practice guideline development is recommended to increase guideline trustworthiness and relevance. The aim was to engage multiple sclerosis (MS) patients and caregivers in the definition of the key questions to be answered in the European Academy of Neurology guideline on palliative care of people with severe MS. Methods: A mixed methods approach was used: an international online survey launched by the national MS societies of eight countries, after pilot testing/debriefing on 20 MS patients and 18 caregivers, focus group meetings of Italian and German MS patients and caregivers. Results: Of 1199 participants, 951 (79%) completed the whole online survey and 934 from seven countries were analysed: 751 (80%) were MS patients (74% women, mean age 46.1) and 183 (20%) were caregivers (36% spouses/partners, 72% women, mean age 47.4). Participants agreed/strongly agreed on inclusion of the nine pre-specified topics (from 89% for 'advance care planning' to 98% for 'multidisciplinary rehabilitation'), and <5% replied 'I prefer not to answer' to any topic. There were 569 free comments: 182 (32%) on the pre-specified topics, 227 (40%) on additional topics (16 guideline-pertinent) and 160 (28%) on outcomes. Five focus group meetings (three of MS patients, two of caregivers, and overall 35 participants) corroborated the survey findings. In addition, they allowed an explanation of the guideline production process and the exploration of patient-important outcomes and of taxing issues. Conclusions: Multiple sclerosis patient and caregiver involvement was resource and time intensive, but rewarding. It was the key for the formulation of the 10 guideline questions and for the identification of patient-important outcomes.

关键词： HEPATOLENTICULAR degeneration   MENTAL disabilities   COPPER metabolism  

摘要： Maternal epilepsy has been associated with adverse short-term perinatal outcome of the offspring. The aim of the present study was to study the possible association between maternal epilepsy and the risk for long-term neurological morbidity of the offspring.

关键词： acute subdural hemorrhage   anoxic depolarization   secondary injury   spontaneous cortical spreading depression   tissue impedance  

摘要： Acute subdural hemorrhage (ASDH) is common and associated with severe morbidity and mortality. To date, the role of spontaneous cortical spreading depression (sCSD) in exaggerating secondary injury after ASDH, is poorly understood. The present study contains two experimental groups: First, we investigated and characterized the occurrence of sCSD after subdural blood infusion (300L) via tissue impedance (IMP) measurement in a rat model. Second, we compared the occurrence and influence of sCSD on lesion growth and neurological deficit in the presence and absence of whole blood constituents. In the first experimental group, three IMP traits could be distinguished after ASDH: no sCSD, recurrent sCSD, and constant elevated IMP (anoxic depolarization [AD]). In the second experimental group, sCSD occurred more often after autologous blood, compared with paraffin oil infusion. Lesion volume 7 days post-ASDH was 27.3 +/- 6.8mm(3) after blood and 3.4 +/- 2.1mm(3) after paraffin oil infusion. Subgroup analysis showed larger lesion size in animals with sCSD, than in those without. Further, occurrence of sCSD led to worse neurological outcomes in both groups. sCSD occurs early after ASDH and does not depend on the presence of whole blood constituents. However, numbers and degree of sCSD are more frequent and severe after autologous blood infusion, compared with an inert volume substance. The occurrence of sCSD leads to lesion growth and worse neurological outcome. Thus, our data advocate close monitoring and targeted treatment of sCSD after ASDH evacuation.

关键词： Diagnosis   disease   encephalitis   nervous   physiology   typhoid  

摘要： Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of unchecked activation of the immune system leading to phagocytosis of blood cells and proliferation of histiocytes in solid organs. HLH can be primary or secondary to infective, autoimmune and malignant conditions. Scrub typhus is an infective illness caused by Orientia tsutsugamushi, transmitted by mite. The illness ranges from mild fever with rash to severe multisystem illness. Scrub typhus has rarely been associated with secondary HLH. We report an infant with scrub typhus who progressed to develop HLH with central nervous system involvement with fatal outcome.

摘要： This commentary is on the original article by Paz Levy et al. on pages 91-97 of this issue

摘要： Initiated in the 1990s by a small group of researchers commissioned by the World Bank, the Global Burden of Diseases, Injuries, and Risk Factors (GBD) Study now provides an independent, systematic, comparative quantification of health loss from hundreds of diseases and injuries in 195 *** GBD analyses that forecast scenarios for life-expectancy over the next two decades predict slower but further increases (by more than four years) for both men and women, with more than 60 countries, including China—the most populous country in the world—having a forecast life expectancy of over 80 years in *** of population growth and ageing, non-communicable diseases will then account for almost 70% of years of life *** bridge the gap between life expectancy and healthy life expectancy, health-care systems and research funders should focus on reducing the burden of neurological diseases, whose extent is being assessed regularly by the GBD Study.

摘要： A study1 reports the construction of an integrated network of genomic, transcriptomic, proteomic, and histopathological data from three independent cohorts of patients with Alzheimer's disease via next generation sequencing of post-mortem brain tissue.1 These data showed increased nucleic acids and proteins from human herpesvirus 6A and 7 in four brain regions of patients with Alzheimer's disease compared with healthy controls.[...]acute bacterial meningitis still carries a high mortality and long-term morbidity due to delayed presentation, delayed treatment initiation, and a small number of diagnostic facilities.[...]to improve the course of acute bacterial meningitis, early adjunctive therapeutic strategies, such as neurocritical care and treatment of brain oedema with intravenous dexamethasone, were implemented.[...]the possibility of autoimmune-mediated encephalitis after HSE might carry important therapeutic implications, because immune suppression and modulation might improve prognosis.4 The best therapeutic regimen should now be explored in clinical trials.