A New Bibliometric Index: The Top 100 Most Disruptive and Developmental Publications in Colorectal Surgery Journals.

BACKGROUND: A new bibliometric index called the disruption score was recently proposed to identify innovative and paradigm-changing publications. OBJECTIVE: The goal was to apply the disruption score to the colorectal surgery literature to provide the community with a repository of important research articles. DESIGN: This study is a bibliometric analysis. SETTINGS: The 100 most disruptive and developmental publications in Diseases of the Colon & Rectum, Colorectal Disease, International Journal of Colorectal Disease, and Techniques in Coloproctology were identified from a validated data set of disruption scores and linked with the iCite National Institutes of Health tool to obtain citation counts. MAIN OUTCOME MEASURES: The primary outcomes measured were the disruption score and citation count. RESULTS: We identified 12,127 articles published in Diseases of the Colon & Rectum (n = 8109), International Journal of Colorectal Disease (n = 1912), Colorectal Disease (n = 1751), and Techniques in Coloproctology (n = 355) between 1954 and 2014. Diseases of the Colon & Rectum had the most articles in the top 100 most disruptive and developmental lists. The disruptive articles were in the top 1% of the disruption score distribution in PubMed and were cited between 1 and 671 times. Being highly cited was weakly correlated with high disruption scores (r = 0.09). Developmental articles had disruption scores that were more strongly correlated with citation count (r = 0.18). LIMITATIONS: This study is subject to the limitations of bibliometric indices, which change over time. DISCUSSION: The disruption score identified insightful and paradigm-changing studies in colorectal surgery. These studies include a wide range of topics and consistently identified editorials and case reports/case series as important research. This bibliometric analysis provides colorectal surgeons with a unique archive of research that can often be overlooked but that may have scholarly significance. See Video Abstract at http://links.lww.com/DCR/B639.UN NUEVO INDICE BIBLIOMÉTRICO: LAS 100 MAS IMPORTANTES PUBLICACIONES EN INNOVACIONES DESESTABILIZADORAS Y DE DESARROLLO EN LAS REVISTAS DE CIRUGÍA COLORRECTALANTECEDENTES:Un nuevo índice bibliométrico llamado innovación desestabilizadora y de desarrollo ha sido propuesto para identificar publicaciones de vanguardia y que pueden romper paradigmas.OBJETIVO:La meta fué aplicar el índice de desestabilización a la literature en cirugía colorectal para aportar a la comunidad con un acervo importante de artículos de investigación.DISEÑO:Un análisis bibliométrico.PARAMETROS:Las 100 publicaciones mas desestabilizadores y de desarrollo en las revistas: Diseases of the Colon and Rectum, Colorectal Disease, International Journal of Colorectal Disease, y Techniques in Coloproctology se recuperaron de una base de datos validada con puntuaciones de desestabilización y se ligaron con la herramienta iCite NIH para obtener la cuantificación de citas.PRINCIPAL MEDIDA DE RESULTADO:El índice desestabilizador y la cuantificación de citas.RESULTADOS:Se identificaron 12,127 articulos publicados en Diseases of the Colon and Rectum (n = 8,109), International Journal of Colorectal Disease (n = 1,912), Colorectal Disease (n = 1,751), y Techniques in Coloproctology (n = 355) de 1954-2014. Diseases of the Colon and Rectum representó la mayoría de las publicaciones dentro de la lista de los 100 mas desestabilizadores y de desarrollo. Esta literatura desestabilizadora se encuentra en el principal 1% de la distribución de la puntuacón desestabilizadora en PubMed y se citaron de 1 a 671 veces. El ser citado con frecuencia se relacionó vagamente con las puntuaciones de desastibilización (r = 0.09). Los artículos de desarrollo tuvieron puntuaciones de desestabilización que estuvieron muy correlacionados con la cuantificación de las citas (r = 0.18).LIMITACIONES:Las sujetas a las limitaciones de los índices bibliométricos, que se modifican en el tiempo.DISCUSION:La putuación de desestabilicación identificó trabajos perspicaces, pragmáticos y modificadores de paradigmas en cirugía colorrectal. Es de interés identificar que se incluyeron una gran variedad de temas y en forma consistente editoriales, reportes de casos y series de casos que representaron una investigación importante. Este análisis bibliométrico aporta a los cirujanos colorrectales de un acervo de investigación único que puede con frecuencia pasarse por alto, y sin embargo tener una gran importancia académica. Consulte Video Resumen en http://links.lww.com/DCR/B639. (Traducción- Dr. Miguel Esquivel-Herrera).

Quantifying the scientific interest in surgical training and education: numerical evidence of a PubMed analysis.

The scientific interest (SI) for a given field can be ascertained by quantifying the volume of published research. We quantified the SI in surgical education to clarify the extent of worldwide efforts on this crucial factor required to improve health-care systems. A set of Medical Subject Headings (MeSH) was defined for the PubMed search. The number of Pubmed Indexed Papers (nPIP) relevant to the SI was extracted from database conception to December 2016 and their distribution and evolution by country were analyzed at 10-year intervals. Population Adjusted Index (PAI) and Medical School Adjusted Index (MSAI) analyses were performed for countries with the nPIP > 30. We identified 51,713 articles written in 33 different languages related to surgical education; 87.6% of these were written in English. General surgery was the leading surgical specialty. The overall nPIP doubled every 10 years from 1987 (from 6009 to 13,501, to 26,272) but stabilized at 3707, 3800 and 3433 in the past 3 years, respectively. The PAI and MSAI analyses showed that the USA, United Kingdom, New Zealand, Canada, Australia and Ireland are top producers of published research in surgical education, constituting a combined 62.88% of the nPIP. Our quantification of the change in SI in surgical education and training gives a clear picture of evolution, efforts and leadership worldwide over time. This picture mirrors an international academic society that should encourage all those involved in surgical education to improve efforts in educational research.

Current trends in cancer immunotherapy: a literature-mining analysis.

Cancer immunotherapy is a rapidly growing field that is completely transforming oncology care. Mining this knowledge base for biomedically important information is becoming increasingly challenging, due to the expanding number of scientific publications, and the dynamic evolution of this subject with time. In this study, we have employed a literature-mining approach that was used to analyze the cancer immunotherapy-related publications listed in PubMed and quantify emerging trends. A total of 93,033 publications published in 5055 journals have been retrieved, and 141 meaningful topics have been identified, which were further classified into eight distinct categories. Statistical analysis indicates a mean annual increase in the number of published papers of approximately 8% in the last 20 years. The research topics that exhibited the highest trends included "immune checkpoint inhibitors," "tumor microenvironment," "HPV vaccination," "CAR T-cells," and "gene mutations/tumor profiling." The top identified cancer types included "lung," "colorectal," and "breast cancer," and a shift in popularity from hematological to solid tumors was observed. As regards clinical research, a transition from early phase clinical trials to randomized control trials was recorded, indicating that the field is entering a more advanced phase of development. Overall, this mining approach provided an unbiased analysis of the cancer immunotherapy literature in a time-conserving and scale-efficient manner.

Bibliometric Analysis of Pediatric Liver Transplantation Research in PubMed from 2014 to 2018.

BACKGROUND Pediatric liver transplantation is used to treat children with end-stage liver disease. This study explored the research hotspots and bibliometric characteristics of pediatric liver transplantation through a variety of bibliometric analysis software. We conducted hotspot analysis to help determine important directions for future scientific research. MATERIAL AND METHODS The study samples were articles related to pediatric liver transplantation published in PubMed in the past 5 years. The high-frequency keywords are extracted by BICOMB software, and then a binary matrix and a common word matrix were constructed. Gcluto software was used to perform double-clustering and visual analysis on high-frequency words, and then we obtained hot area classification. Strategic coordinates are constructed using Excel. Citespace and VOSviewer software are used for further analysis and bibliometric data visualization. RESULTS A total of 36 high-frequency words were found in the 4118 studies. A peak map was drawn through double-cluster analysis. Biclustering analysis was used to calculate the concentricity and density of each hotspot. We obtained the top 10 countries/regions engaged in pediatric liver transplantation research. VOSviewer was used to visualize the co-author map. CONCLUSIONS We found 5 clusters and 7 aspects for pediatric liver transplantation. Additionally, calculation results showed that post-transplant lymphoproliferative disorder in pediatric patients and outcomes of multivisceral transplantation seem very promising. This conclusion is of great value for future exploratory research.

Surgical publication activity in the English literature over a 10-year interval.

Background: Surgical publication activity in the English literature over a 10-year interval may have changed. This study sought to identify which countries make the most contributions and whether significant shifts have occurred in this time. Methods: Screening of 17 international journals in PubMed was performed for the time periods 2006-2007 and 2016-2017, for papers published by a first author belonging to a general surgical department. Data were collected by country regarding the total number of publications, cumulative impact factors (IFs), publications per inhabitant, IFs per inhabitant, and number of RCTs, meta-analyses and systematic reviews per country in both periods. Results: A total of 2247 and 3029 papers were found for 2006-2007 and 2016-2017 respectively. In 2006-2007, most papers (605, 26·9 per cent; 2697·3 IFs) came from the USA, followed by Japan (284, 12·6 per cent; 1042·1 IFs) and the UK (197, 8·8 per cent; 923·1 IFs). In 2016-2017, the USA led again with 898 papers (29·6 per cent; 4575·3 IFs), followed by Japan with 414 papers (13·7 per cent; 1556·6 IFs) and the Netherlands with 167 (5·5 per cent; 885·2 IFs). From the top 15 countries, Sweden, the Netherlands and Switzerland contributed the most articles per inhabitant during both time periods. During both periods, the UK published the most RCTs, meta-analyses and systematic reviews. Conclusion: Surgeons from the USA were the most productive in total number of publications during both time periods. However, smaller European countries were more active than the USA in relation to their population size.

Quantifying Sex Bias in Clinical Studies at Scale With Automated Data Extraction.

Importance: Analyses of female representation in clinical studies have been limited in scope and scale. Objective: To perform a large-scale analysis of global enrollment sex bias in clinical studies. Design, Setting, and Participants: In this cross-sectional study, clinical studies from published articles from PubMed from 1966 to 2018 and records from Aggregate Analysis of ClinicalTrials.gov from 1999 to 2018 were identified. Global disease prevalence was determined for male and female patients in 11 disease categories from the Global Burden of Disease database: cardiovascular, diabetes, digestive, hepatitis (types A, B, C, and E), HIV/AIDS, kidney (chronic), mental, musculoskeletal, neoplasms, neurological, and respiratory (chronic). Machine reading algorithms were developed that extracted sex data from tables in articles and records on December 31, 2018, at an artificial intelligence research institute. Male and female participants in 43 135 articles (792 004 915 participants) and 13 165 records (12 977 103 participants) were included. Main Outcomes and Measures: Sex bias was defined as the difference between the fraction of female participants in study participants minus prevalence fraction of female participants for each disease category. A total of 1000 bootstrap estimates of sex bias were computed by resampling individual studies with replacement. Sex bias was reported as mean and 95% bootstrap confidence intervals from articles and records in each disease category over time (before or during 1993 to 2018), with studies or participants as the measurement unit. Results: There were 792 004 915 participants, including 390 470 834 female participants (49%), in articles and 12 977 103 participants, including 6 351 619 female participants (49%), in records. With studies as measurement unit, substantial female underrepresentation (sex bias ≤ -0.05) was observed in 7 of 11 disease categories, especially HIV/AIDS (mean for articles, -0.17 [95% CI, -0.18 to -0.16]), chronic kidney diseases (mean, -0.17 [95% CI, -0.17 to -0.16]), and cardiovascular diseases (mean, -0.14 [95% CI, -0.14 to -0.13]). Sex bias in articles for all categories combined was unchanged over time with studies as measurement unit (range, -0.15 [95% CI, -0.16 to -0.13] to -0.10 [95% CI, -0.14 to -0.06]), but improved from before or during 1993 (mean, -0.11 [95% CI, -0.16 to -0.05]) to 2014 to 2018 (mean, -0.05 [95% CI, -0.09 to -0.02]) with participants as the measurement unit. Larger study size was associated with greater female representation. Conclusions and Relevance: Automated extraction of the number of participants in clinical reports provides an effective alternative to manual analysis of demographic bias. Despite legal and policy initiatives to increase female representation, sex bias against female participants in clinical studies persists. Studies with more participants have greater female representation. Differences between sex bias estimates with studies vs participants as measurement unit, and between articles vs records, suggest that sex bias with both measures and data sources should be reported.

Sturge-Weber Syndrome Coexisting With Moyamoya Disease in the Fifth Decade: A Case Report and Literature Review.

Sturge-Weber syndrome (SWS) is a sporadically occurring neurocutaneous syndrome characterized by port-wine stain over the face, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angiomas. It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or stroke-like episodes. Here, we report a 46-year-old male patient, having SWS coexisting with moyamoya disease, attending our hospital due to sudden loss of consciousness. We also searched PubMed (from its earliest date to August 2014) for case reports mentioning that SWS presents in adulthood. We identified 31 patients. The common clinical manifestation are seizures, stroke-like episodes, and migraine-like headaches.On the basis of our findings in this patient, we would recommend that patients with a port-wine nevus of the face should be given further investigation to rule out an intracranial vascular malformation, especially if seizures, stroke-like episodes, or migraine-like headaches are present.

Objective Measures to Characterize the Physiological Effects of Spinal Cord Stimulation in Neuropathic Pain: A Literature Review.

OBJECTIVE: The physiological mechanisms behind the therapeutic effects of spinal cord stimulation (SCS) are only partially understood. Our aim was to perform a literature review of studies that used objective measures to characterize mechanisms of action of SCS in neuropathic pain patients. MATERIALS AND METHODS: We searched the PubMed data base to identify clinical studies that used objective measures to assess the effects of SCS in neuropathic pain. We extracted the study factors (e.g., type of measure, diagnoses, painful area[s], and SCS parameters) and outcomes from the included studies. RESULTS: We included 67 studies. Of these, 24 studies used neurophysiological measures, 14 studies used functional neuroimaging techniques, three studies used a combination of neurophysiological and functional neuroimaging techniques, 14 studies used quantitative sensory testing, and 12 studies used proteomic, vascular, and/or pedometric measures. Our findings suggest that SCS largely inhibits somatosensory processing and/or spinal nociceptive activity. Our findings also suggest that SCS modulates activity across specific regions of the central nervous system that play a prominent role in the sensory and emotional functions of pain. CONCLUSIONS: SCS appears to modulate pain via spinal and/or supraspinal mechanisms of action (e.g., pain gating, descending pain inhibition). However, to better understand the mechanisms of action of SCS, we believe that it is necessary to carry out systematic, controlled, and well-powered studies using objective patient measures. To optimize the clinical effectiveness of SCS for neuropathic pain, we also believe that it is necessary to develop and implement patient-specific approaches.

The aetiologies of the unilateral oculomotor nerve palsy: a review of the literature.

Oculomotor nerve palsy (ONP) is an important and common clinical diagnosis. Its main features are diplopia and ptosis. Its aetiologies are various and complex. A number of different conditions have been reported to cause ONP, such as diabetes mellitus, aneurysm, tumours, painful ophthalmoplegia, pituitary lesions, cavernous sinus lesions, central nervous system infections, and subarachnoid haemorrhage. A patients needs to undergo several tests in order to establish the correct underlying pathology. In this review, we have summarized the aetiologies of the unilateral ONP, and discussed their relative clinical features, pathogenesis, diagnostic criteria, treatment options, and prognosis. We searched PubMed for papers related to ONP and its aetiologies, and selected the publications, which seemed appropriate.

Dentatorubral-pallidoluysian Atrophy: An Update.

Background: Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare, autosomal dominantly inherited disorder characterized by myoclonus, epilepsy, ataxia, and dementia. Diagnosis is challenging due to the heterogeneous presentation and symptomatic overlap with other spinocerebellar ataxias. Symptoms vary according to age of onset, with a mean age at onset of 31 years. A CAG repeat expansion in the ATN1 gene results in neuronal intranuclear inclusions, variable neuronal loss, and astrocytosis in the globus pallidus, dentate and red nuclei. No disease-modifying or curative treatments are currently available. Methods: We performed an online literature search using PubMed for all articles published in an English Language format on the topics of DRPLA or ATN1 over the last 10 years. Where these articles cited other research as support for findings, or statements, these articles were also reviewed. Contemporary articles from related research fields (e.g., Huntington's Disease) were also included to support statements. Results: Forty-seven articles were identified, 10 were unobtainable and 10 provided no relevant information. The remaining 27 articles were then used for the review template: seven case reports, seven case series, six model system articles (one review article), four population clinical and genetic studies (one review article), two general review articles, and one human gene expression study. Other cited articles or research from related fields gave a further 42 articles, producing a total of 69 articles cited: 15 case series (including eight family studies), 14 model systems (one review article), 14 population clinical and genetic studies (two review articles), 10 case reports, eight clinical trials/guidelines, four genetic methodology articles, three general review articles, and one human gene expression study. Discussion: DRPLA remains an intractable, progressive, neurodegenerative disorder without effective treatment. Early recognition of the disorder may improve patient understanding, and access to services and treatments. Large-scale studies are lacking, but are required to characterize the full allelic architecture of the disorder in all populations and the heterogeneous phenotypic spectrum, including neuroimaging findings, possible biomarkers, and responses to treatment.

Trends and topics in occupational diseases over the last 60 years from PubMed.

Objectives The purpose of this study was to provide an analysis of scientific production on occupational diseases (OD) during the period 1945-2015 in order to describe publication trends on that topic and identify the major diseases as well as the predominant actors (journals, countries) involved in this field. Methods A PubMed search was carried out to extract articles related to occupational diseases during the period 1 January 1945 to 31 December 2015 using a specific query. Data were downloaded from PubMed in Extensible Markup Language (XML) and processed through a dedicated parser. Results A total of 160 025 articles were retrieved from 7127 journals. One third of these articles were published in 39 journals: the core journals according to Bradford's law. Following exponential growth, OD publications reached a plateau in 2007. The overall dynamics of the OD field are heterogeneous with differences between subfields: psychological diseases emerged in the 1990s while "traditional" OD are less studied nowadays. Despite a sharp decrease in the proportion of publications, the most productive country remains the USA with 14.5% of the OD publications over the period but Scandinavian countries are, proportionally, the most active in research and publication on OD. Conclusions The proportion of publications on OD is decreasing in Medline, except for specific subfields of OD. This is discrepant with the global burden of occupational diseases.

Producción científica de los hospitales públicos de la Ciudad de Buenos Aires, 2017 / Scientific production from public hospitals of the City of Buenos Aires, 2017

El número de publicaciones en la literatura científica provenientes de una institución es indicador de su producción científica. La producción científica de los hospitales del Gobierno de la Ciudad de Buenos Aires (GCBA) ha sido valorada previamente, pero sin discriminar cuánto de dicha producción correspondía a otras instituciones académicas asentadas en ellos (Universidad de Buenos Aires, UBA, Consejo Nacional de Investigaciones Científicas y Técnicas, CONICET). Nuestro objetivo fue evaluar las publicaciones incluidas en PubMed que corresponden a hospitales del GCBA, describir sus principales características, y discriminar la contribución de otras instituciones académicas (UBA y CONICET). Es un estudio transversal por búsqueda en PubMed incorporando el nombre de los 34 hospitales del GCBA, UBA y CONICET. Se identificaron 2727 publicaciones de hospitales del GCBA (4.6% de las publicaciones de Argentina); 73.9% en inglés, 78.9% referidas a humanos, 37.2% de los últimos 5 años; 6.4% con alto nivel de evidencia (ensayos clínicos y metaanálisis), 28.4% incluían niños. En comparación con el total nacional, las publicaciones del GCBA incluyen menos trabajos en inglés, más investigaciones en humanos, más ensayos clínicos y más investigación en niños. De las publicaciones correspondientes a hospitales del GCBA, el 90.4% no compartían la afiliación con el CONICET ni con la UBA. Concluyendo, los hospitales del GCBA aportaron 4.6% del total de publicaciones de Argentina en PubMed; el 90% no es compartida con UBA ni CONICET. Las publicaciones de instituciones del GCBA incluyen más ensayos clínicos e investigación en niños.

The number of publications in the scientific literature coming from an institution is an indicator of its scientific production. The scientific production of the hospitals of the Government of the City of Buenos Aires (GCBA) has been evaluated previously, but without discriminating how much of that production corresponded to other academic institutions settled there (University of Buenos Aires, UBA, National Council of Scientific Research and Techniques, CONICET). Our objective was to evaluate the publications included in PubMed that correspond to hospitals of the GCBA, describe their main characteristics, and discriminate the contribution of other academic institutions (UBA and CONICET). It is a cross-sectional study based on a PubMed search, using the name of each of the 34 GCBA hospitals, CONICET and UBA in the "affiliation" field. In total, 2727 publications from GCBA hospitals were identified (4.6% of Argentine publications); 73.9% in English, 78.9% in relation to humans, 37.2% in the last 5 years; 6.4% with high level of evidence (clinical trials and meta-analysis), and 28.4% including children. Compared to the national total, the GCBA publications include fewer works in English, more research in humans, more clinical trials and more research in children. Of the publications corresponding to hospitals of the GCBA, 90.4% did not share the affiliation with CONICET or with UBA.In conclusion, the GCBA hospitals generated 4.6% of the total Argentine publications in PubMed; and 90% of these was not shared with UBA or CONICET. Publications from GCBA institutions include more clinical trials and research in children.

KCa3.1 Channels and Glioblastoma: In Vitro Studies.

BACKGROUND: Several tumor entities including brain tumors aberrantly overexpress intermediate conductance Ca2+ activated KCa3.1 K+ channels. These channels contribute significantly to the transformed phenotype of the tumor cells. METHOD: PubMed was searched in order to summarize our current knowledge on the molecular signaling upstream and downstream and the effector functions of KCa3.1 channel activity in tumor cells in general and in glioblastoma cells in particular. In addition, KCa3.1 expression and function for repair of DNA double strand breaks was determined experimentally in primary glioblastoma cultures in dependence on the abundance of proneural and mesenchymal stem cell markers. RESULTS: By modulating membrane potential, cell volume, Ca2+ signals and the respiratory chain, KCa3.1 channels in both, plasma and inner mitochondrial membrane, have been demonstrated to regulate many cellular processes such as migration and tissue invasion, metastasis, cell cycle progression, oxygen consumption and metabolism, DNA damage response and cell death of cancer cells. Moreover, KCa3.1 channels have been shown to crucially contribute to resistance against radiotherapy. Futhermore, the original in vitro data on KCa3.1 channel expression in subtypes of glioblastoma stem(-like) cells propose KCa3.1 as marker for the mesenchymal subgroup of cancer stem cells and suggest that KCa3.1 contributes to the therapy resistance of mesenchymal glioblastoma stem cells. CONCLUSION: The data suggest KCa3.1 channel targeting in combination with radiotherapy as promising new tool to eradicate therapy-resistant mesenchymal glioblastoma stem cells.

Adult Pilocytic Astrocytoma: An Institutional Series and Systematic Literature Review for Extent of Resection and Recurrence.

INTRODUCTION: Pilocytic astrocytoma is a classically benign tumor that most often affects pediatric patients. Rarely, it occurs during adulthood. We present a case series and systematic literature review of adult pilocytic astrocytoma (APA) to examine the clinical presentation, extent of resection, and recurrence rate associated with this tumor in this population. MATERIALS AND METHODS: Our institutional records were retrospectively reviewed for cases of pilocytic astrocytoma in adults. A PubMed search identified English-language studies of pathology-proven APA. A meta-analysis was performed to determine the relationship between extent of tumor resection and recurrence. RESULTS: Forty-six patients with APA were diagnosed at our institution (mean age 33.6 ± 13.3; 24 [52%] female). Twenty-four patients (52%) underwent gross total resection, 11 (24%) subtotal resection, 4 (9%) near total resection, 4 (9%) observation after biopsy, and 3 (6%) radiotherapy alone. Tumors recurred or progressed in 6 (13%) patients, of whom 4 were treated by STR and 2 were treated by radiotherapy alone. Thirty-nine (95%) patients were still alive at last follow-up. A systematic literature review identified 415 patients with APA in 38 studies. Including our case series, 7 studies reported extent of resection, follow-up, and recurrence. Of 254 patients with a weighted mean follow-up of 77.7 ± 49.6 (31-250) months, 129 (51%) were treated with gross total resection, and 125 (49%) underwent subtotal resection. Tumor recurred in 79 (31%) patients, 22 (27%) after gross total resection and 57 (73%) after subtotal resection (P < 0.001). CONCLUSIONS: Pilocytic astrocytoma rarely presents during adulthood. Overall, prognosis is favorable and survival rates are high. APA recurrence is more likely after STR, and the goal of surgery should always be GTR when feasible.

MRI-Guided stereotactic laser ablation for epilepsy surgery: Promising preliminary results for cognitive outcome.

Cognitive outcome data are reviewed with respect to the use of magnetic-resonance guided stereotactic laser ablation (SLA) as an epilepsy surgical procedure, with comparisons drawn to traditional open resection procedures. Cognitive outcome with stereotactic laser amygdalohippocampotomy (SLAH) appears better than open resection for several functions dependent on extra-mesial temporal lobe (TL) structures, including category-related naming, verbal fluency, and object/familiar person recognition. Preliminary data suggests episodic, declarative verbal memory can decline following SLAH in the language dominant hemisphere, although early findings suggest comparable or even superior outcomes compared with open resection. The hippocampus has long been considered a central structure supporting episodic, declarative memory, with epilepsy surgical teams attempting to spare it whenever possible. However, ample data from animal and human neuroscience research suggests declarative memory deficits are greater following broader mesial TL lesions that include parahippocampal gyrus and lateral TL inputs. Therefore, employing a neurosurgical technique that restricts the surgical lesion zone holds promise for achieving a better cognitive outcome. Focal SLA lesions outside of the amygdalohippocampal complex may impair select cognitive functions, although few data have been published in such patients to date. SLA is being effectively employed with adults and children with TL or lesional epilepsies across several U.S. epilepsy centers, which may simultaneously optimize cognitive outcome while providing a curative treatment for seizures.

Management of Tiny Unruptured Intracranial Aneurysms: A Comparative Effectiveness Analysis.

Importance: Unruptured intracranial aneurysms (UIAs) are relatively common in the general population and are being increasingly diagnosed; a significant proportion are tiny (≤3 mm) aneurysms. There is significant heterogeneity in practice and lack of clear guidelines on the management of incidental, tiny UIAs. It is important to quantify the implications of different management strategies in terms of health benefits to patients. Objective: To evaluate the effectiveness of routine treatment (aneurysm coiling) vs 3 strategies for imaging surveillance compared with no preventive treatment or routine follow-up of tiny UIAs. Design, Setting, and Participants: A decision-analytic model-based comparative effectiveness analysis was conducted from May 1 to June 30, 2017, using inputs from the medical literature. PubMed searches were performed to identify relevant literature for all key model inputs, each of which was derived from the clinical study with the most robust data and greatest applicability. Analysis included 10 000 iterations simulating adult patients with incidental detections of UIAs 3 mm or smaller and no history of subarachnoid hemorrhage. Interventions: The following 5 management strategies for tiny UIAs were evaluated: annual magnetic resonance angiography (MRA) screening, biennial MRA screening, MRA screening every 5 years, aneurysm coiling and follow-up, and no treatment or preventive follow-up. Main Outcomes and Measures: A Markov decision model for lifetime rupture was constructed from a societal perspective per 10 000 patients with incidental, tiny UIAs. Outcomes were assessed in terms of quality-adjusted life-years. Probabilistic, 1-way, and 2-way sensitivity analyses were performed. Results: In this analysis of 10 000 iterations simulating adult patients with a mean age of 50 years, the base-case calculation shows that the management strategy of no treatment or preventive follow-up has the highest health benefit (mean [SD] quality-adjusted life-years, 19.40 [0.31]). Among the management strategies that incorporate follow-up imaging, MRA every 5 years is the best strategy with the next highest effectiveness (mean [SD] quality-adjusted life-years, 18.05 [0.62]). The conclusion remains robust in probabilistic and 1-way sensitivity analyses. No routine follow-up remains the optimal strategy when the annual growth rate and risk of rupture of growing aneurysms are varied. When the annual risk of rupture of nongrowing UIAs is less than 1.7% (0.23% in base case scenario), no follow-up is the optimal strategy. If annual risk of rupture is more than 1.7%, coiling should be performed directly. Conclusions and Relevance: Given the current literature, no preventive treatment or imaging follow-up is the most effective strategy in patients with aneurysms that are 3 mm or smaller, resulting in better health outcomes. More aggressive imaging surveillance for aneurysm growth or preventive treatment should be reserved for patients with a high risk of rupture. Given these findings, it is important to critically evaluate the appropriateness of current clinical practices, and potentially determine specific guidelines to reflect the most effective management strategy for patients with incidental, tiny UIAs.

Risk of natalizumab-associated progressive multifocal leukoencephalopathy in patients with multiple sclerosis: a retrospective analysis of data from four clinical studies.

BACKGROUND: Previous estimates of risk of progressive multifocal leukoencephalopathy (PML) in patients with multiple sclerosis receiving natalizumab were stratified by three risk factors: anti-John Cunningham virus (JCV) antibodies in serum, previous immunosuppressant use, and treatment duration, which were estimated using population-based assumptions. We aimed to calculate PML risk estimates from patient-level risk-factor data and to stratify risk by concentrations of anti-JCV antibody in serum (anti-JCV antibody index). METHODS: Data on natalizumab-treated patients were pooled from four large, observational, open-label studies: STRATIFY-2, STRATA, TOP, and TYGRIS. Data were analysed with and without imputation for missing values of anti-JCV antibody status and previous immunosuppressant use. For anti-JCV antibody-positive patients in this pooled cohort, cumulative PML risk with or without previous immunosuppressant use was estimated using Kaplan-Meier analysis. Annual PML risks (per 12 natalizumab infusions) for patients without PML in the preceding year were estimated using conditional probability based on the life table method. For anti-JCV antibody-positive patients without previous immunosuppressant use, risk estimates were further stratified using a probability distribution for anti-JCV antibody index values, separately for patients with or without PML. Anti-JCV antibody index cutoffs were selected via sensitivity and specificity assessments for identifying PML cases in an index cohort. FINDINGS: 156 (<1%) of 37 249 patients in the pooled cohort had PML. We imputed missing values on anti-JCV antibody status (3912 patients) and on previous immunosuppresant use (544 patients) using a multiple imputation method. For anti-JCV antibody-negative patients (n=13 996), estimated PML risk was less than 0·07 per 1000 patients (95% CI 0·00-0·40). In anti-JCV antibody-positive patients (n=21 696), estimated cumulative PML probability over 6 years (72 infusions of natalizumab) was 2·7% (95% CI 1·8-4·0) in patients with previous immunosuppressant use and 1·7% (1·4-2·1) in those without. In patients without previous immunosuppressant use (n=18 616), estimated annual PML risks per 1000 patients, conditional on having no PML before that year, ranged from 0·01 (0·00-0·03) in year 1 (1-12 infusions) to 0·6 (0·0-1·5) in year 6 (61-72 infusions) for people with an index of 0·9 or less; from 0·1 (0·0-0·2) in year 1 to 3·0 (0·2-5·8) in year 6 for those with an index of more than 0·9 up to and including 1·5; and from 0·2 (0·0-0·5) in year 1 to 10·0 (5·6-14·4) in year 6 for those with an index of more than 1·5. INTERPRETATION: Our risk estimates calculated from patient-level clinical data allow individualised annual prediction of risk of PML in patients receiving natalizumab for multiple sclerosis, supporting yearly benefit-risk re-evaluation in clinical practice. Further, our estimates are generally consistent with previously calculated estimates. Incorporating anti-JCV antibody index allows further risk stratification for anti-JCV antibody-positive patients who have not previously taken immunosuppressants. FUNDING: Biogen.

Classification of Meningiomas Based on Their Surgical Removal, World Health Organization Grade, and Cytogenetic Profile: A Treatment Algorithm.

BACKGROUND: Meningiomas are the most common primary intracranial tumor, but the lack of prospective randomized trials has led to different guidelines for their treatment. We proposed a classification of meningiomas that considers surgical removal, histology, and cytogenetic profile, based on a literature review of these 3 criteria. The classification can be used to guide adjuvant treatment and follow-up. METHODS: A retrospective literature review was performed of PubMed from 2007 to 2016. Search terms were "meningioma," "surgery," "WHO classification," "cytogenetic," and "algorithm." RESULTS: Meningiomas were classified into 5 groups (A-E) according to the Simpson resection grade, World Health Organization grade, and cytogenetic profile. Adjuvant therapy, follow-up magnetic resonance imaging, and management of recurrence and/or regrowth were proposed according to the classification. CONCLUSIONS: The proposed meningioma classification was based on our experience and retrospective evidence collated from the literature and supported by recommendations. The application of the classification criteria yielded an algorithm for treatment and follow-up of patients with meningioma.

Genetic Landscape of Congenital Myasthenic Syndromes From Turkey: Novel Mutations and Clinical Insights.

Congenital myasthenic syndromes are clinically and genetically heterogeneous disorders of neuromuscular transmission. Most are treatable, but certain subtypes worsen with cholinesterase inhibitors. This underlines the importance of genetic diagnosis. Here, the authors report on cases with genetically proven congenital myasthenic syndromes from Turkey. The authors retrospectively reviewed their experience of all patients with congenital myasthenic syndromes, referred over a 5-year period (2011-2016) to the Child Neurology Department of Dokuz Eylül University, Izmir, Turkey. In addition, PubMed was searched for published cases of genetically proven congenital myasthenic syndromes originating from Turkey. In total, the authors identified 43 (8 new patients, 35 recently published patients) cases. Defects in the acetylcholine receptor (n = 15; 35%) were the most common type, followed by synaptic basal-lamina associated (n = 14; 33%) and presynaptic syndromes (n = 10; 23%). The authors had only 3 cases (7%) who had defects in endplate development. One patient had mutation GFPT1 gene (n = 1; 2%). Knowledge on congenital myasthenic syndromes and related genes in Turkey will lead to prompt diagnosis and treatment of these rare neuromuscular disorders.