关键词： Digital footprint   Google   Neurosurgery   Online healthcare information   Online presence   Physician rating sites  

摘要： BACKGROUND: Patients are increasingly turning to online resources to inquire about individual physicians and to gather health information. However, little research exists studying the online presence of neurosurgeons across the country. This study aimed to characterize these online profiles and assess the scope of neurosurgeons' digital identities. METHODS: Medicare-participating neurologic surgeons from the United States and Puerto Rico were identified using the Centers for Medicare and Medicaid Services (CMS) Physician Comparable Downloadable File. Each physician was characterized by his or her medical education, graduation year, city of practice, gender, and affiliation with an academic institution. Using a Google-based custom search tool, the top 10 search results for each physician were extracted and categorized as 1 of the following: 1) physician, hospital, or healthcare system controlled, 2) third-party or government controlled, 3) social media-based, 4) primary journal article, or 5) other. RESULTS: Among the physicians within the CMS database, 4751 self-identified as being neurosurgeons, yielding a total of 45,875 uniform resource locator search results pertinent to these physicians. Of the 4751 neurosurgeons, 2317 (48.8%) and 2434 (51.2%) were classified as academic and nonacademic neurosurgeons, respectively. At least 1 search result was obtained for every physician. Hospital, healthcare system, or physician-controlled websites (18,206;39.7%) and third-party websites (17,122;37.3%) were the 2 most commonly observed domain types. Websites belonging to social media platforms accounted for 4843 (10.6%) search results, and websites belonging to peer-reviewed academic journals accounted for 1888 (4.1%) search results. The frequency with which a third-party domain appeared as the first search result was higher for nonacademic neurosurgeons than for academic neurosurgeons. CONCLUSIONS: In general, neurosurgeons lacked a controllable online presence within their

关键词： Farber disease   disseminated lipogranulomatosis   drug development   natural history   orphan disease  

摘要： Purpose: Farber disease (OMIM 22800) is an ultrarare progressive multisystemic neurodevelopmental storage disorder caused by a deficiency of the lysosomal enzyme acid ceramidase (AC). Hard clinical end points for future clinical trials remain to be defined. Methods: We quantitatively analyzed published cases with Farber disease (N = 96). The main outcome variables were survival and diagnostic delay. As a potential predictor of survival, the influence of residual AC enzyme activity was investigated. The analysis was performed in compliance with STROBE criteria. Results: The median survival period of the study population was 3 years. The median age at disease onset was 3 months, and the median age at diagnosis was 17 months. The median diagnostic delay was 13.75 months. Patients with residual AC activity in fibroblasts at more than 5.1% of the normal level survived significantly longer than patients with residual AC activity below this threshold. In addition, higher residual AC activity was associated with a later onset of symptoms. Conclusion: Farber disease onset is in infancy. Diagnostic delay is typically substantial. Our data suggest a phenotype-biomarker association with implications for future clinical and therapeutic trials. In the absence of a prospective multicenter natural-history study protocol, we believe that our modeling approach, based on published case descriptions, is the best and most timely approximation for generalizability.

关键词： COLOR vision   DIFFERENTIAL diagnosis   NEUROLOGICAL disorders -- Diagnosis   PARKINSON'S disease diagnosis   ESSENTIAL tremor   DIAGNOSIS  

关键词： Falls risk assessment   neurological rehabilitation   specificity   sensitivity  

摘要： Objective: To calculate scale performance of the newly developed Hessisch Oldendorf Fall Risk Scale (HOSS) for classifying fallers and non-fallers in comparison with the Risk of Falling Scale by Huhn (FSH), a frequently used assessment tool. Design: A prospective observational trail was conducted. Setting: The study was performed in a large specialized neurological rehabilitation facility. Subjects: The study population (n=690) included neurological and neurosurgery patients during neurological rehabilitation with varying levels of disability. Around the half of the study patients were independent and dependent in the activities of daily living (ADL), respectively. Interventions: Fall risk of each patient was assessed by HOSS and FSH within the first seven days after admission. Main measures: Event of fall during rehabilitation was compared with HOSS and FSH scores as well as the according fall risk. Scale performance including sensitivity and specificity was calculated for both scales. Results: A total of 107 (15.5%) patients experienced at least one fall. In general, fallers were characterized by an older age, a prolonged length of stay, and a lower Barthel Index (higher dependence in the ADL) on admission than non-fallers. The verification of fall prediction for both scales showed a sensitivity of 83% and a specificity of 64% for the HOSS scale, and a sensitivity of 98% with a specificity of 12% for the FSH scale, respectively. Conclusion: The HOSS shows an adequate sensitivity, a higher specificity and therefore a better scale performance than the FSH. Thus, the HOSS might be superior to existing assessments.

关键词： Neurologically normal   Nissen fundoplication   Toupet fundoplication  

摘要： Introduction: We compared laparoscopic Toupet fundoplication (LTF) and laparoscopic Nissen fundoplication (LNF) in neurologically normal children. Methods: Forty neurologically normal children who were followed up for more than 3 years after LTF (n = 22) or LNF (n = 18) were reviewed retrospectively. LTF and LNF were performed between 2006 and 2012. Results: There were no significant differences in gender (LTF, 15 male and 7 female patients;LNF:, 12 male and 6 female patients), mean age at surgery (LTF vs LNF: 2.5 vs 2.3 years), mean weight at surgery (LTF vs LNF: 9.6 vs 8.9 kg), preoperative symptoms, preoperative pH monitoring (pH < 4) (LTF vs LNF: 26.7% vs 21.8%), mean operative time (LTF vs LNF: 117 vs 126 min), postoperative recommencement of enteral feeding (LTF vs LNF: 3.7 vs 3.8 days), or duration of hospitalization (LTF vs LNF: 5.5 vs 6.3 days). Intraoperative complications were esophageal trauma (LTF;n = 1;4.5%) and liver trauma (LNF;n = 1;5.6%) (P = 0.70). Post-LTF complications were wrap stenosis (n = 1;4.5%), and post-LNF complications were wrap stenosis (n = 1;5.5%) and gastric outlet obstruction (n = 1;5.5%) (P = 0.43);all were managed conservatively. No case required conversion to open repair. There was no recurrence after LTF, but there were three cases (16.7%) after LNF (P = 0.08). Reoperation was performed at 4, 11, and 13 months, respectively. Conclusion: Despite LTF and LNF appearing to be equally effective, three nLNF cases required reoperation.

关键词： echinacoside   preparation   pharmacokinetics   Parkinson's disease   Alzheimer's disease  

摘要： Echinacoside (ECH), a natural phenylethanoid glycoside, was first isolated from Echinacea angustifolia DC. (Compositae) sixty years ago. It was found to possess numerous pharmacologically beneficial activities for human health, especially the neuroprotective and cardiovascular effects. Although ECH showed promising potential for treatment of Parkinson's and Alzheimer's diseases, some important issues arose. These included the identification of active metabolites as having poor bioavailability in prototype form, the definite molecular signal pathways or targets of ECH with the above effects, and limited reliable clinical trials. Thus, it remains unresolved as to whether scientific research can reasonably make use of this natural compound. A systematic summary and knowledge of future prospects are necessary to facilitate further studies for this natural product. The present review generalizes and analyzes the current knowledge on ECH, including its broad distribution, different preparation technologies, poor pharmacokinetics and kinds of therapeutic uses, and the future perspectives of its potential application.

关键词： focused ultrasound   MRgFUS   essential tremor   thalamotomy   DBS  

摘要： Background: Magnetic resonance imaging-guided focused ultrasound thalamotomy is approved by the U.S. Food and Drug Administration for treatment of essential tremor. Although this incisionless technology creates an ablative lesion, it potentially avoids serious complications of open stereotactic surgery. Objective: To determine the safety profile of magnetic resonance imaging-guided focused ultrasound unilateral thalamotomy for essential tremor, including frequency, and severity of adverse events, including serious adverse events. Methods: Analysis of safety data for magnetic resonance imaging-guided focused ultrasound thalamotomy (186 patients, five studies). Results: Procedure-related serious adverse events were very infrequent (1.6%), without intracerebral hemorrhages or infections. Adverse events were usually transient and were commonly rated as mild (79%) and rarely severe (1%). As previously reported, abnormalities in sensation and balance were the commonest thalamotomy-related adverse events. Conclusion: The overall safety profile of magnetic resonance imaging-guided focused ultrasound thalamotomy supports its role as a new option for patients with medically refractory essential tremor. (c) 2018 International Parkinson and Movement Disorder Society

关键词： acute disseminated encephalomyelitis   chikungunya   complications   congenital infections   encephalitis   Guillain-Barre syndrome   myelitis   neonatal infection   neurological   optic neuritis   retinitis   uveitis  

摘要： We performed a systematic review on the neurological complications of chikungunya virus. Such complications are being reported increasingly, owing primarily to the scale of recent epidemics but also to a growing understanding of the virus' neurovirulence. We performed a thorough literature search using PubMed and Scopus databases, summating the data on all published reports of neurological disease associated with chikungunya virus. We appraised the data for each major condition in adults, children, and neonates, as well as evaluating the latest evidence on disease pathogenesis and management strategies. The review provides a comprehensive summary for clinicians, public health officials, and researchers tackling the challenges associated with this important emerging pathogen.

关键词： FLUENCY (Language learning)   SPEECH disorders   NEUROLOGICAL disorders   STUTTERING   DIAGNOSIS   BRAIN injuries   SPEECH therapists   SPEECH therapy  

摘要： Purpose: The aim of this study is to investigate how speech fluency in typical and atypical speech is perceptually assessed by speech-language pathologists (SLPs). Our research questions were as follows: (a) How do SLPs rate fluency in speakers with and without neurological communication disorders? (b) Do they differentiate the speaker groups? and (c) What features do they hear impairing speech fluency? Method: Ten SLPs specialized in neurological communication disorders volunteered as expert judges to rate 90 narrative speech samples on a Visual Analogue Scale (see Kempster, Gerratt, Verdolini Abbott, Barkmeier-Kraemer, & Hillman, 2009;p. 127). The samples-randomly mixed-were from 70 neurologically healthy speakers (the control group) and 20 speakers with traumatic brain injury, 10 of whom had neurogenic stuttering (designated as Clinical Groups A and B). Results: The fluency rates were higher for typical speakers than for speakers with traumatic brain injury;however, the agreement among the judges was higher for atypical fluency. Auditory-perceptual assessment of fluency was significantly impaired by the features of stuttering and something else but not by speech rate. Stuttering was also perceived in speakers not diagnosed as stutterers. A borderline between typical and atypical fluency was found. Conclusions: Speech fluency is a multifaceted phenomenon, and on the basis of this study, we suggest a more general approach to fluency and its deviations that will take into account, in addition to the motor and linguistic aspects of fluency, the metalinguistic component of expression as well. The results of this study indicate a need for further studies on the precise nature of borderline fluency and its different disfluencies.

关键词： Diagnosis   Biological Markers   Nervous System Diseases   Metabolic Diseases   Inborn errors of metabolism   Metabolomics  

摘要： Conventional workup of rare neurological disease is frequently hampered by diagnostic delay or lack of diagnosis. While biomarkers have been established for many neurometabolic disorders, improved methods are required for diagnosis of previously unidentified or underreported causes of rare neurological disease. This would result in a higher diagnostic yield and increased patient numbers required for interventional studies. Recent studies using next-generation sequencing and metabolomics have led to identification of novel disease-causing genes and biomarkers. This combined approach can assist in overcoming challenges associated with analyzing and interpreting the large amount of data obtained from each technique. In particular, metabolomics can support the pathogenicity of sequence variants in genes encoding enzymes or transporters involved in metabolic pathways. Moreover, metabolomics can show the broader perturbation caused by inborn errors of metabolism and identify a metabolic fingerprint of metabolic disorders. As such, using "omics" has great potential to meet the current needs for improved diagnosis and elucidation of rare neurological disease.