关键词： Fundoplication   Children   Gastroesophageal reflux disease   Boix-Ochoa   Partial fundoplication  

摘要： Background In 4-5% of cases of gastroesophageal reflux disease (GERD), surgical treatment is required. The aim of the study was to evaluate the success of Boix-Ochoa antireflux surgery, which is considered more physiologic with a higher failure rate (need for reoperation) than Nissen fundoplication, which is believed to be the gold standard operation. Method In the 13 years from 2005 to 2018, the medical records of all children who underwent Boix-Ochoa in a single institution by pediatric surgeons were reviewed retrospectively. Results A total of 133 fundoplications were performed, of which patients were divided into four groups: neurologically impaired, structurally impaired, neurologically and structurally impaired, and neurologically and structurally normal;there were 64, 8, 34, and 27 patients in each group, respectively. Structural impairments included hiatal hernia and esophagus atresia, having previously had a gastrostomy and esophageal stenosis. The most common short-term complication was distal esophageal stenosis (13%), which caused vomiting and dysphagia, and was treated by dilatations. There were six (4.5%) recurrences of GER, one in the neurologically and structurally impaired group with a hiatal hernia and five in the structurally impaired group (three esophagus atresias, two caustic esophageal strictures). The mean follow-up period was 5.27 +/- 3.43 years. Neurological impairment did not affect the success rate. Conclusion Although there has not any literature demonstrating significant benefits of one procedure, we detected with this largest study in the pediatric literature about Boix-Ochoa fundoplication (more physiologic and easily performed) that it was successful (95%) in protecting reflux even in neurologically impaired patients (98%). We consider Boix-Ochoa (partial fundoplication) to be an alternative method to Nissen (complete fundoplication), and it can be done safely with a high success rate.

关键词： MAMs   ERMES   amyotrophic lateral sclerosis   Parkinson's disease   Alzheimer's disease   cerebral ischemia   Huntington's disease  

摘要： The endoplasmic reticulum (ER) and mitochondria are fundamental organelles highly interconnected with a specialized set of proteins in cells. ER-mitochondrial interconnections form specific microdomains, called mitochondria-associated ER membranes, that have been found to play important roles in calcium signaling and lipid homeostasis, and more recently in mitochondrial dynamics, inflammation, and autophagy. It is not surprising that perturbations in ER-mitochondria connections can result in the progression of disease, especially neurological disorders;hence, their architecture and regulation are crucial in determining the fate of cells and disease. The molecular identity of the specialized proteins regulating ER-mitochondrial crosstalk remains unclear. Our discussion here describes the physical and functional crosstalk between these two dynamic organelles and emphasizes the outcome of altered ER-mitochondrial interconnections in neurological disorders.

关键词： Central nervous system   Kidney transplantation   Neurologic complication   Peripheral nervous system   Renal failure   Renal transplantation   Tremor  

摘要： The aim of this systematic review and meta-analysis was to evaluate the prevalence of neurological complication after renal transplantation. The searches were conducted by two independent researchers in the international (PubMed, Web of Science, Scopus, and Google Scholar) and national databases (Magiran and SID) to find the relevant studies published in English and Persian languages since the creation of the databases until January 2019 (without time limitations). The keywords used in the search strategy were: neurologic complication, central nervous system, peripheral nervous system, tremor, CVA, encephalopathy, neurological complications, renal transplantation, renal failure, kidney transplantation, immunosuppression, neurotoxicity, opportunistic infections, CNS, cerebrovascular disease, chronic kidney disease, cognitive impairment, and end-stage renal disease, which were combined using the AND, OR, and NOT operators. Finally, a meta-analysis was conducted in STATA14 statistical software. Based on the random effect model, the total prevalence of neurologic complications in 4674 patients who had undergone the renal transplantation surgery was 7.9% (95% confidence interval [CI]:7.2%,8.7%, I-2 = 90.1%). The prevalence of infectious, non-infectious and treatment associated neurologic complications was 9.5% (95% CI -8.9, 10.2), 91.8% (95% CI -91.3, 92.4) and 97% (95% CI-95.7%,98.4%) of all neurologic complications in renal transplant patients, respectively. And according to the present subgroup analysis, peripheral neuropathy with a prevalence about 30% (29%) (95% CI -27.6%, 30.4%, I-2 = 99.4%) was the most common neurological disorder in renal transplant patients followed by tremor with a prevalence of 19.5% (CI -17.6%, 21.3%, I-2 = 97.1%), cerebrovascular events with a prevalence of 15.1% (95% CI -13.9%, 16.4%, I-2 = 96.5%), encephalopathy with the prevalence of 13% (95% CI -12%, 14%, I-2 = 99.3%), headache with a prevalence of 8.3% (95% CI -6.8%, 9.8%, I-2 = 97.3%)

关键词： Attention   Distraction   MMN   P3a   RON   Neurological disorders   Psychiatric disorders  

摘要： Involuntary attention allows for the detection and processing of novel and potentially relevant stimuli that lie outside of cognitive focus. These processes comprise change detection in sensory contexts, automatic orientation toward this change, and the selection of adaptive responses, including reorientation to the original goal in cases when the detected change is not relevant for task demands. These processes have been studied using the Event-Related Potential (ERP) technique and have been associated to the Mismatch Negativity (MMN), the P3a, and the Reorienting Negativity (RON) electrophysiological components, respectively. This has allowed for the objective evaluation of the impact of different neuropsychiatric pathologies on involuntary attention. Additionally, these ERP have been proposed as alternative measures for the early detection of disease and the tracking of its progression. The objective of this review was to integrate the results reported to date about MMN, P3a, and RON in different neurological and psychiatric disorders. We included experimental studies with clinical populations that reported at least two of these three components in the same experimental paradigm. Overall, involuntary attention seems to reflect the state of cognitive integrity in different pathologies in adults. However, if the main goal for these ERP is to consider them as biomarkers, more research about their pathophysiological specificity in each disorder is needed, as well as improvement in the general experimental conditions under which these components are elicited. Nevertheless, these ERP represent a valuable neurophysiological tool for early detection and follow-up of diverse clinical populations.

关键词： iron   iron deficiency   neonatal period   neurological development   newborn infant   maternal health  

摘要： Iron deficiency is the most common micronutrient deficiency worldwide. Iron is essential for the development of multiple organ systems, most especially the developing brain. Iron deficiency, particularly during sensitive periods of brain development, such as in early childhood, is associated with long-lasting adverse consequences for cognition, motor function and behaviour. Little consideration has been given to iron deficiency in newborn infants and its potential health consequences. Fetal iron accretion is compromised by pregnancy complications such as pre-term birth and gestational diabetes mellitus, and our work has identified an increased risk of low iron stores at birth from maternal lifestyle factors such as smoking and obesity. Early-life events, including Caesarean section delivery, further add to the cumulative risk of neonatal iron deficiency, which can persist throughout infancy into early childhood. While investigations into the long-term neurological consequences of neonatal iron deficiency are limited, there is evidence of poorer memory, motor function and language ability in children born iron deficient. Recently, we also identified significant behavioural consequences of neonatal deficiency persisting from 2 to 5 years of age, with effects particularly apparent in 'high-risk' children born to obese or smoking mothers or delivered by Caesarean section. Interventions targeting the fetal/neonatal period could therefore represent a key opportunity for the prevention of iron deficiency and its associated long-term health consequences. A dual approach is required, comprising public health strategies targeting prevention, to improve health in women of reproductive age, and the development of screening strategies for the early detection of iron deficiency in newborn infants.

摘要： Background: Non-adherence to medication is a common and serious problem in health care. To develop more effective interventions to improve adherence, there is a need for a better understanding of the individual types of non-adherence. Objective: To determine clusters of non-adherence in neurological patients using a complex adherence questionnaire. Methods: In this observational, monocentric study 500 neurological patients (consecutive sampling) were recruited in the Department of Neurology at the Jena University Hospital, Germany (outpatient clinic, wards) over a period of 5 months. Patients with severe dementia or delirium who were unable to complete the questionnaire were excluded. Due to missing adherence data, in total, 429 patients with common neurological disorders were analyzed. Different types and clusters of non-adherence using the German Stendal Adherence to Medication Score (SAMS) were determined. Results: For the 429 patients, the mean age was 63 years (SD = 16), 189 were female. According to the SAMS total score 74 (17.2%) were fully adherent, 252 (58.7%) showed moderate non-adherence and 103 (24%) showed clinically significant non-adherence. Principal component analysis with Varimax rotation revealed three independent factors explaining 60.5% of the SAMS variance. The bulk of non-adherence was attributed to modifications of medication (41.7%) and forgetting to take the medication (33.2%) followed by lack of knowledge 25.1% about reasons, dosages and time of administration for the medication. Conclusions: Intentional non-adherence was the primary self-reported behavior identified among non-adherent neurological participants. Many patients modified their prescribed medication due to various reasons, such as side effects or lacking effect. Different clusters require different interventions. While for the cluster 'forgetfulness' the reduction of poly-medication and a behavioral approach with reminders seems reasonable, patients in cluster 'missing knowled

关键词： Psychogenic nonepileptic seizures   Epileptic seizures   Medically unexplained physical symptoms  

摘要： Objectives: This study examined the demographic, social, and clinical (neurological and psychiatric) characteristics of people with psychogenic nonepileptic seizures (PNES) presenting to tertiary neurological services at Auckland District Health Board, New Zealand. Methods: Electronic notes and video-electroencephalography (video-EEG) data gathered from the telemetry unit based on synchronized acquisition (motor activity and brain electrophysiology) over a five-year period (2011 to 2015 inclusive) were retrospectively examined. Two groups were compared: people with PNES only or people with combined PNES and epileptic seizures (ES) (the group with PNES) and a control group with ES only, matched 1:1 by age and gender. Results: Sixty-six people in the group with PNES were matched with an equivalent number of ES controls. As a cohort, there was high psychiatric and medical comorbidity in both groups, but overall, those with PNES experienced higher rates than their ES counterparts. An older age of onset, female gender, and history of abuse were more frequently seen in those with PNES. Compared with controls, people with PNES more commonly had daily seizures (rather than monthly) but presented less frequently to neurology services. A high proportion of people with PNES experienced historical traumas, ongoing stressors, and disability. Almost half of the people with PNES were on antiepileptic drugs (AEDs) and received limited psychiatric or psychological input. In contrast, people with ES were more likely to be on psychotropic medication. Conclusion: Both PNES and ES are associated with high levels of psychiatric and medical comorbidity. Additionally, PNES were found to be associated with iatrogenic harm and disability. Though people with PNES and ES often have a range of associated needs, there is, however, limited access to appropriate services. The needs of these populations should be better met through enhanced integration across psychiatry, neurology, and multidiscipl

关键词： Delirium   Neurology   Risk factor   Neuropsychiatry  

摘要： Introduction Predisposing and precipitating factors for delirium are well known;however, their interaction and impact on delirium in neurological patients remains largely unknown. Therefore, those factors were evaluated in hospitalized patients with neurological disorders. Methods In this prospective cohort study, 1487 neurological patients were included, 356 patients with delirium and 1131 without delirium. Relevant neurological- and medical-related clusters were assessed with multiple regression analyses, prediction models, and cluster analysis evaluating their association with delirium. Results The 1-year incidence of delirium in this cohort was 23.9%. Delirium developed in 31% of patients with stroke, in 39.5% with epilepsy, and in 58.4% with ICH. The most relevant predisposing factors were substance-use disorders (OR 4.24, 2.28-7.78, p < 0.001), advanced age (OR 3.44, CI 2.40-4.92, p < 0.001), and neurodegenerative disorders (OR 2.58, CI 1.47-4.54, p = 0.001). The most relevant precipitating factors were meningitis (OR 21.52, CI 1.22-379.83, p = 0.036), acute renal failure (OR 10.01, CI 1.13-88.73, p = 0.039), and intracranial hemorrhage (OR 3.62, CI 2.08-6.30, p < 0.001). Delirious patients were hospitalized 6 days longer, had higher in-hospital mortality, and were discharged more often to nursing homes and rehabilitation. Best predictor for delirium was the coexistence of advanced age with epilepsy (58.3%, p < 0.001), while patients aged < 65 years without epilepsy and stroke rarely developed delirium (5.1%, p < 0.001). Conclusions Delirium is common in elder neurological patients and associated with worse outcome. Primary cerebral conditions most frequently precipitate delirium in neurology. Neurologists are advised to monitor symptoms of delirium in the presence of risk factors to enable both timely diagnostic work-up and management of delirium.

关键词： Woodhouse-Sakati syndrome   Dystonia   NBIA   Hypogonadism  

摘要： Background: Woodhouse-Sakati syndrome (WSS) is a rare autosomal recessive disease with characteristic neuroendocrine manifestations. WSS encompasses heterogeneous phenotypes and disease course. Objective: We aimed to characterize neurological involvement of the disease through subgrouping of core neurological manifestations. Methods: A single-institution retrospective analysis of patients with clinically and genetically confirmed diagnosis of WSS. Results: A total of 38 individuals belonging to 17 families were identified to have WSS. The mean age at enrollment was 30.1 years (range 16-53 years). Neurological involvement was noted in 31 patients (81.5%). Dystonia was the most common neurological manifestation (67%), followed by intellectual disability (45%) and sensorineural hearing loss (30%). Based on the Neurological Impairment Scale (NIS), the disease was recognized to have two distinct patterns. A disabling, rapidly progressive pattern (NIS of 3-4;Type 1) was noted in eighteen patients (12 males, 6 females;47.4%) with severe disability that occurs within a mean duration of 7.4 +/- 3.6 years. Type 2 WSS was identified in twenty patients (8 males, 12 females;52.6%), and showed either absent or mild neurological involvement with preserved activities of daily living (NIS of 0-1). The mean age of onset for neurological manifestations was earlier in type 1 (12.6 +/- 4.5 years) compared to type 2 (18.1 +/- 4.3 years). Type 1 WSS has a significantly higher rate of intellectual disability (p = < 0.001). Conclusions: In this pleiotropic syndrome, we identified two distinct phenotypes with variable prognosis. A high Interfamilial and intrafamilial phenotypic variability despite having a similar gene mutation suggests a possible role of genetic or environmental modifying factor.

关键词： Vitamin deficiency   B vitamin deficiency   Neurologic symptoms of vitamin deficiencies   Malabsorption vitamin deficiency   Nutritional deficiency  

摘要： Purpose of Review The goal of this chapter is to educate clinicians on the neurologic manifestations of certain nutritional deficiencies in order to promptly identify and appropriately treat these patients. Recent Findings Many vitamin and nutritional deficiencies have been described dating back to the early days of neurology and medicine. Some are very rare and thus, there are no randomized controlled studies to assess supplementation or dosage;however, there are reviews of case reports that can assist clinicians in choosing treatments. Summary While endemic vitamin and nutritional deficiencies may be rarely encountered in many countries, vulnerable populations continue to be at risk for developing neurologic complications. These populations include those with diseases causing malabsorption, the elderly, chronic alcohol users, as well as pregnant mothers with hyperemesis gravidarum to name a few. It is important to recognize syndromes associated with these nutritional deficiencies, as prompt identification and treatment may prevent permanent neurologic damage.