关键词： children   drooling   long-term   neurological impairment   quality of life   surgery  

摘要： Objectives The best surgical option to treat drooling in neurodisabilities is still under debate. The aim of this study was to describe the technique of subtotal functional sialoadenectomy (SFS) (ie four-duct ligation (4-DL) together with bilateral sublingual gland excision) and its long-term outcomes, in comparison with 4-DL. Design Retrospective observational cohort study. Setting Unit of Pediatric Surgery of Bambino Gesu Children's Hospital (Rome). Participants Seventy-five patients surgically treated for drooling between 2002 and 2012, with at least five years of follow-up, divided into two groups: 4-DL group (19 patients) underwent four-duct ligation, and SFS group (56 patients) underwent subtotal functional sialoadenectomy. Main outcome measures Primary end points were the evaluation of drooling improvement after surgery (parameters: Drooling Severity and Frequency Scale, DSFS;no of bibs/day;no of shirts/day;no of pneumonia/year;use of antidrooling drugs) and the comparison between two different surgical techniques. Results Median age at surgery was 10 years (1-35). Long-term outcomes showed significant improvement in DSFS and in no of shirts/day in both groups. Significantly better results were found in the SFS group than in the 4-DL group as far as DSFS (Pvalue .045), no of bibs/day (Pvalue .041), no of shirts/day (Pvalue .032) are concerned. Reoperation rate for recurrence was 42% in the 4-DL group and 0% in the SFS group (Pvalue < .0001). Six patients (8%;2 in the 4-DL group and 4 in the SFS group) experienced perioperative complications, while 4 patients (5%;2 in the 4-DL group and 2 in the SFS group) recorded long-term complications, with no difference between groups neither need for surgical treatment. No surgery-related mortality was recorded. Conclusions In our experience, subtotal functional sialoadenectomy ensured significantly greater long-term effects than four-duct ligation for drooling treatment in neurologically impaired child, with equal compl

关键词： Bariatric surgery   Diabetic neuropathy   Diabetic nephropathy   Diabetic opthalmopathy  

摘要： Background Bariatric surgery has been shown to improve glycemic control in patients with type 2 diabetes. However, less is known whether it can also reduce diabetic renal, neurological, and ophthalmic complications. Methods This prospective multicenter cohort study compared renal, ophthalmic, and neurological complications between 49 patients with obesity/overweight receiving bariatric surgery and 338 patients receiving standard medical treatment after follow-up for 2 years. Patients received neurological examinations including toe tuning fork vibration test, ankle tendon reflex test, 10-g monofilament test, and ophthalmic examinations including visual acuity measurement and fundus examinations. Multiple regressions, propensity score weighting, and matching were employed to adjust for baseline differences. Results After 2 years of follow-up, patients with type 2 diabetes receiving bariatric surgery had greater reduction in BMI, HbA1c, and urine albumin-creatinine ratio, greater improvement in estimated glomerular filtration rate, and greater increase in tuning fork test score of right and left toes compared with the medical group. However, there is no improvement in 10 g-monofilament test, visual acuity, diabetic non-proliferative retinopathy, and proliferative retinopathy. Similar results were obtained using multiple regression adjustment, propensity-score weighting, or comparing age-, sex-, and BMI-matched subjects. Conclusions After 2-year follow-up, patients with obesity/overweight and type 2 diabetes receiving bariatric surgery have increased glomerular filtration rate, reduced albuminuria, and improved tuning folk vibration sensation.

关键词： Epilepsy   Depression   NDDI-E   Reliability   Validity   Persian  

摘要： Introduction: Major depression is common among people with epilepsy (PWE), but it is underdiagnosed. The aim of the present study was to assess the reliability and validity of the Persian version of the Neurological Disorders Depression Inventory for Epilepsy (P-NDDI-E) as a screening tool for major depression in patients with epilepsy. Method: A total of 210 patients suffering from epilepsy have been assessed using the NDDI-E and the Beck Depression Inventory-II (BDI-II) with no difficulty in understanding or answering the Persian version of the questionnaire. Patients identified as depressed under BDI-II underwent a psychiatric evaluation to confirm depression according to 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD) criteria. Result: According to the BDI-II and the ICD-10 criteria, major depression was diagnosed in 75 patients (32% men, 68% women). Cronbach's a coefficient was 0.826, suggesting a very good internal consistency. The receiver operating characteristic analysis showed an area under the curve of 0.90 (95% confidence interval [CI] = 0.86-0.94, standard error [SE]: 0.02, p < 0.001). A cutoff of >= 14 resulted in an 83% sensitivity, an 80% specificity, a 70.1% positive predictive value, and an 88.6% negative predictive value. A significant and positive correlation between the P-NDDI-E and the BDI-II was shown (Spearman's p = 0.604, p < 0.001). Discussion: The P-NDDI-E could be used as a reliable and valid instrument in detecting major depression in PWE. (C) 2020 Elsevier Inc. All rights reserved.

关键词： early neurological improvement   endovascular thrombectomy   ischaemic stroke   nomogram  

摘要： Background and purpose Early neurological improvement (ENI) after endovascular thrombectomy (EVT) has been associated with favorable outcomes. This study aimed to identify the optimal definition of ENI and develop a nomogram for predicting ENI after EVT in acute ischaemic stroke. Methods Patients with EVT were enrolled from a multicenter registry as the training cohort. The receiver operating characteristic curve was used to estimate the optimal threshold for ENI at 24 h of EVT. Logistic regression analysis was utilized to generate the best-fit nomogram for predicting ENI. The discrimination of the nomogram was assessed using the area under the receiver operating characteristic curve (AUC). An additional 447 patients from two stroke centers were prospectively recruited as the test cohort for validating the nomogram. Results A total of 612 patients with EVT were included in the training cohort. The optimal threshold for predicting 3-month favorable outcome (modified Rankin Scale 0-2) was an improvement of the National Institutes of Health Stroke Scale (NIHSS) score by >= 6 points (AUC 0.875;sensitivity 79.5%;specificity 90.7%). Age, blood glucose, recanalization, symptomatic intracranial hemorrhage (sICH) and baseline Alberta Stroke Program Early Computed Tomography Score (ASPECTS) were independently associated with ENI, and were incorporated in the nomogram. The AUC of the nomogram was 0.795 in the training cohort and 0.752 in the test cohort. Conclusions A reduction of NIHSS score >= 6 appeared to be the optimal definition of ENI. The nomogram composed of age, blood glucose, recanalization, sICH and baseline ASPECTS may predict the probability of ENI in ischaemic stroke patients treated with EVT.

关键词： Adverse outcomes   congenital Zika syndrome   infant   pregnancy   Zika virus  

摘要： Objective To define the prevalence of adverse outcomes of maternal infection in a large cohort of ZIKV-infected Brazilian women and their infants. Design Prospective population-based cohort study. Setting Ribeirao Preto's region's private and public health facilities. Population Symptomatic ZIKV-infected mothers and their infants. Methods Prenatal/early neonatal data were obtained for all mother-child pairs. A subgroup of infants had cranial ultrasonography, eye fundoscopy, hearing and neurological examinations and Bayley III screening tests within 3 months of age. Main outcome measures Prevalence of pregnancy losses and anomalies detected at birth or within 3 months according to the gestational age of infection. Results Overall, 511 ZIKV-infected women were identified from a total of 1116 symptomatic women;as there were two twins, there were a total of 513 fetuses included. Of these, 13 (2.5%;95% CI 1.5-4.3) presented with major signs of congenital Zika syndrome (CZS). Of the 511 women, there were 489 livebirths and 24 (4.7%) pregnancy losses (20 miscarriages and four stillbirths). ZIKV-related anomalies occurred in the offspring of 42/511 (8.2%) mothers. Microcephaly or other CNS malformations were diagnosed in 1/4 (25.0%) stillbirths and in 19/489 (3.9%;95% CI 2.5-5.9) of the liveborn infants. Fetal abnormalities were 14.0 (95% CI 7.6-26.0) times more likely with gestational infection occurring in <= 11 weeks. On follow up of 280 asymptomatic infants, 2/155 (1.3%) had eye abnormalities, 1/207 (0.5%) had CNS imaging findings and 16/199 (8%) presented neurological alert signs. Conclusions This prospective population-based study represents the largest Brazilian cohort study of ZIKV in pregnancy. Congenital anomalies potentially associated with CZS are less frequent than previously thought. There is a strong association between the gestational age of infection (<= 11 weeks) and a poorer early infant prognosis. A notable proportion of apparently asymptomatic newborns

关键词： communication   conversion disorder   education   functional neurological disorder   psychoeducation   psychogenic  

摘要： Background and purpose Functional neurological disorder (FND) is a common diagnosis within neurology. Effective communication of the diagnosis is known to be an important part of treatment and can result in reduction or cessation of symptoms, as well as decreased healthcare utilisation. A single group education session, facilitated by professionals commonly involved in the care of patients with FND, was developed to further enhance patients' and relatives' understanding and acceptance of diagnosis. Methods Patients and relatives attending a single education session were asked to complete self-report ratings of understanding of diagnosis, acceptance of diagnosis, belief in treatability and hopefulness regarding recovery, at the beginning and end of the session. Satisfaction data were also collected. Results Data were obtained from 193 patients and 153 relatives. Patients had experienced a median duration of symptoms of 4 years, and more than 80% of patients reported more than one functional neurological symptom. There were significant increases in terms of understanding, acceptance, belief in treatability and hopefulness for patients and relatives. Effect sizes ranged from large for improved understanding of FND to small-to-medium for increased agreement with FND diagnosis. High levels of satisfaction were also reported. Conclusions A multidisciplinary, single group education session is an effective and acceptable method of increasing understanding and acceptance of an FND diagnosis, even for patients with a long duration of symptoms and high symptom burden. It could help improve readiness for further treatment.

关键词： Congenital heart disease   Hypoplastic left heart syndrome   Immaturity   Brain injury   Surgery  

摘要： Congenital heart defects (CHD) is one of the most common types of birth defects. Thanks to advances in surgical techniques and intensive care, the majority of children with severe forms of CHD survive into adulthood. However, this increase in survival comes with a cost. CHD survivors have neurological functioning at the bottom of the normal range. A large spectrum of central nervous system dysmaturation leads to the deficits seen in critical CHD. The heart develops early during gestation, and CHD has a profound effect on fetal brain development for the remainder of gestation. Term infants with critical CHD are born with an immature brain, which is highly susceptible to hypoxic-ischemic injuries. Perioperative blood flow disturbances due to the CHD and the use of cardiopulmonary bypass or circulatory arrest during surgery cause additional neurological injuries. Innate patient factors, such as genetic syndromes and preterm birth, and postoperative complications play a larger role in neurological injury than perioperative factors. Strategies to reduce the disability burden in critical CHD survivors are urgently needed.

关键词： neurocognitive complications   phenylalanine hydroxylase deficiency   phenylketonuria   psychological complications (PKU or PAH deficiency)  

摘要： Purpose The purpose of this article is to review literature for neurocognitive, neuropsychiatric, neurological complications associated with phenylalanine hydroxylase (PAH) deficiency. The goal is to familiarize nurse practitioners with treatment and monitoring guidelines for persons living with the disorder. Conclusions Appropriate treatment can maximize neurocognitive and neuropsychiatric outcomes. Practice Implications Nurse practitioners can help persons with PAH deficiency through education and providing appropriate referrals and by supporting disease-specific treatment.

关键词： Adeno-associated virus   Alzheimer's disease   Blood-brain barrier   Gene therapy   Parkinson's disease  

摘要： Magnetic resonance-guided focused ultrasound (MRgFUS) is a cutting-edge technology that is changing the practice of movement disorders surgery. Given the noninvasive and innovative nature of this technology, there is great interest in expanding the use of MRgFUS to additional diseases and applications. Current approved applications target the motor thalamus to treat tremor, but clinical trials are exploring or plan to study noninvasive lesions with MRgFUS to ablate tumor cells in the brain as well as novel targets for movement disorders and brain regions associated with pain and epilepsy. Although there are additional potential indications for lesioning, the ability to improve function by destroying parts of the brain is still limited. However, MRgFUS can also be applied to a brain target after intravenous delivery of microbubbles to create cavitations and focally open the blood-brain barrier (BBB). This has already proven to be safe and technically feasible in human patients with Alzheimer's disease, and this action alone has potential to clear extracellular pathology associated with this and other neurodegenerative disorders. This also provides a foundation for noninvasive intravenous delivery of therapeutic molecules to precise brain targets after transient disruption of the BBB. Certain chemotherapies for brain tumors, immunotherapies, gene, and cell therapies are all examples of therapeutic or even restorative agents that normally will not enter the brain without direct infusion but which have been shown in preclinical studies to effectively traverse the BBB after transient disruption with MRgFUS. Here we will review these novel applications of MRgFUS to provide an overview of the extraordinary potential of this technology to expand future neurosurgical treatments of brain diseases.