Cerebral Cavernous Malformation Bleeding Following Cerebrospinal Fluid Diversion Surgery: A Case Report and Literature Review.

Cavernous malformations (CM) are rare intracerebral vascular lesions occurring in the brain, or less commonly in the spine, with an annual bleeding risk of up to 1.1%. These lesions can be occult or present to signs and symptoms based on location or, more frequently, are a result of hemorrhagic events. The most challenging aspect of managing these cases is weighing the risks and benefits of surgical treatment and intervening before the onset of a devastating hemorrhagic event. Here, we present the second case of CM haemorrhage following the cerebrospinal fluid (CSF) diversion procedure with a literature review of theories explaining this phenomenon. We present a 37-year-old female who has a known case of brainstem cavernoma and underwent left sub-temporal resection with stable residual since 2011, then was managed conservatively due to patient preference till she had a deterioration in December 2021 manifested as confusion, diplopia, dysarthria, and significant left sided weakness leaving her wheelchair bound. CT showed supratentorial hydrocephalus with extensive periventricular transependymal edema and no clear haemorrhage. A ventriculoperitoneal (VP) shunt was inserted, with no intraoperative complications. A few hours post-VP shunt insertion, she experienced a worsening in her mental status, hemiparesis, and dysarthria. Subsequent imaging found evidence of acute haemorrhage in the location of the previously noted residual. She was managed by supportive care. Causative factors of CM haemorrhage are poorly understood, and current data only suggest that prior haemorrhage and CM location could increase bleeding risk. Only one case of CM bleeding post-shunt insertion was reported; however, studies on other types of intracranial vascular lesions suggest that alterations in transmural pressure (including cerebrospinal fluid diversion procedures) can increase the risk of haemorrhage by changing the hemodynamic flow in these abnormally formed and weak vascular structures.

Desmoplastic Small Round Cell Tumor Presenting as an Intra/Extracranial Mass.

Desmoplastic small round cell tumors (DSRCTs) are highly malignant tumors, with distinct reciprocal chromosome translocation (11;22)(p13;q12). Intracranial metastasis is a very rare complication of this tumor, with only a few cases reported in the literature. To our knowledge, this is the only case presenting an extracranial extension of intracranial metastasis of DSRCT. A 33-year-old man was diagnosed with DSRCT in the pelvic cavity. He presented with a scalp lump and right-sided weakness. A biopsy showed metastasis from DSRCT. Metastatic DSRCT to the brain is extremely rare. Surgical resection followed by adjuvant treatment, including chemotherapy and radiation, is indicated as it has a poor prognosis. Moreover, aggressive treatment is warranted to prevent progression and relapse.

Multifocal malignant peripheral nerve sheath tumor in patients with neurofibromatosis type I: Report of two cases and review of literature.

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are one of the rarest soft-tissue sarcomas with a prevalence of 0.001% in the general population. It is closely associated with a unique neurocutaneous stigmata under the spectrum of the dermatological manifestations of neurofibromatosis type 1 (NF1). Almost 81% of MPNST arises from a precursor neuroma, and multifocality of these lesions is extremely rare, making up to 0.001% of cases. Moreover, spinal cases are extremely uncommon with only four cases reported internationally. Here, we present the fifth and sixth spinal MPNST cases with a brief review of literature. Case Description: We describe two unusual cases of multifocal MPNST in relation to NF1 occurring in the spinal cord. Both patients presented with local pain and myelopathic symptoms. The two patients underwent wide surgical resection, followed by neoadjuvant radiotherapy and reported immediate postoperative improvement of the presented complaint; however, one patient suffered from rapid recurrence and metastasis. Conclusion: Due to the scarcity of spinal cases related to MPNST, no clear guidelines regarding the management of these cases are set in the literature. Histopathological diagnosis remains as the most pivotal diagnostic tool as they can mimic other peripheral nerve sheath lesions, such as neuromas and schwannomas, in imaging. Cases that were managed by early surgical intervention in addition to neoadjuvant radiotherapy reported the best outcome. However, cases of MPNST in concomitance with NF1 were found to be resistant to both chemo and radiotherapy and have high recurrence rate.

Bibliometric analysis of the top 100 most-cited articles in neurofibromatosis.

Background: Neurofibromatosis (NF) is an umbrella term that refers to three distinct disease entities: NF Type 1, Type 2, and schwannomatosis. Here, we reviewed the scientific performance and the most influential publications on NF. Methods: A keyword-based search was performed using the Scopus database. The top 100 articles were grouped based on NF types and the studied entities. The differences between the articles, authors, and journals were quantified based on certain parameters. Other parameters were collected for the complete citational analysis. Results: The top 100 articles were published between 1961 and 2020. The most trending period of research was in the 1990s and articles studying the clinical aspect and the underlying genetic correlation made up 84% of all articles from the list. The United States of America (USA) had the highest number of contributions (69 articles, 69%). The top institute of contribution to the list was the Howard Hughes Medical Institute, USA (14 articles, 14%). Author-based analysis reveals that the neurologist D. H. Gutmann from St. Louis Children's Hospital, USA, was the most active and authored 11 articles (11%) on the list. Conclusion: The publication trends show that articles studying medical and surgical management were of little interest. The top 100 articles did not include any randomized control trials, and the highest level of evidence was obtained from reviews of pooled knowledge as well as population-based and longitudinal studies.

Bibliometric overview of the Top 100 most cited articles on hydrocephalus.

Background: Hydrocephalus is one of the most common brain disorders and numerous articles were published to address the clinical aspect and its management. This study aims to highlight the most influential work on hydrocephalus on bibliometric basis. Methods: A thorough search of Scopus database was performed using the word "hydrocephalus." The 100 most cited articles were retrieved, and variables of importance were collected including the article's title, 1st author affiliation, country of origin, year and journal of publication, article's category, and citation count according to Scopus and Google scholar databases. Results: The 100 most cited articles were thoroughly analyzed. Publication dates ranged from 1946 to 2014, with most articles (45) published between 1998 and 2007. The mean number of citations per publication was 201 with total of 20,177 citations. The United States of America contributed half of the articles. The leading institution was the Canadian hospital for Sick Children University of Toronto having published 5 articles. Hydrocephalus in general and normal pressure hydrocephalus was the two major categories addressed with most studies fall under the topic of surgical management. Neurosurgery was the specialty with the greatest contribution (47%). The articles were published in 46 different journals led by the Journal of Neurosurgery with total of 17 articles. Conclusion: This bibliometric analysis delineates the landmark publications in hydrocephalus. The listed articles depict the myriad of studied aspects historically which helps in understanding hydrocephalus overall in evidence-based module for neurosurgeons and non-neurosurgeons.

Association of Alzheimer's Disease with Genetic Variants of Apolipoprotein E, Clusterin, TNF-α, and IL-6 Among Elderly Saudis.

BACKGROUND: In the wake of the warning by WHO that the prevalence of dementia may have a rise of 125% in the Middle East by 2050, identification of the genetic risk factors in Arab populations is urgent. OBJECTIVES: To investigate the association of Single Nucleotide Polymorphisms (SNPs) in apolipoprotein E (ApoE), clusterin (CLU), tumor necrotic factor- α (TNF-α) and interleukin-6 (IL-6) genes, with risk of Alzheimer's disease (AD) in Saudi Arabian participants. METHODS: A total of 42 Saudi AD patients and 23 age-matched control participants were genotyped for eight SNPs: rs429358, rs7412 (ApoE); rs11136000, rs1532278 (CLU); rs1800629, rs1799724 (TNF-α) and rs1800796, rs1800795(IL-6), by RT-PCR using the TaqMan assay. Serum concentrations of amyloid beta peptide 1-40(Aß1-40), amyloid beta peptide 1-42(Aß1- 42), CLU and some other biochemical markers were measured. RESULTS: A significant increase (p=0.004) in the serum CLU level was detected in the AD group (340.4 ± 74.6) compared with control group (265.0 ± 80.9). For rs1532278 (CLU), genotype GA was significantly higher in AD patients (57.1%) than in the control participants (26.1%), [p=0.024, OR = 4.00, 95% CI (1.20-13.28)]. For the ApoE SNP rs7412, 40.4% of patients carried a TT genotype, whereas it was completely absent in the controls [p = 0.020, OR = 30.53, 95% CI (1.73 - 540.05)].For rs429358 (ApoE), patients showed a significantly increased frequency of the TC genotype [p = 0.006, OR = 9.33, 95% CI (1.89-46.19)] and TT [p = 0.045, OR = 19.76, 95% CI (1.07-366.0)] genotype than controls. AD patients with CC genotype for ApoE rs429358 had significantly lower levels of Aß1-40 (p=0.04) in AD patients than controls. Carriers of genotype GG for rs1800629 (TNF-α) showed significantly higher levels of serum IL-6 (p = 0.04) in AD patients. CONCLUSION: Genetic variants in ApoE and CLU may influence susceptibility to AD among Saudi Arabian participants.

A Bibliometric Analysis of the Most-Cited Articles on Craniospinal Epidural Hematoma.

AIM: To analyze the most-cited articles on cranial and spinal epidural hematomas (EDHs). MATERIAL AND METHODS: A title-specific search was performed on the Scopus database using the term ?epidural hematoma? in June 2020, with no publication date restrictions. The top 100 most-cited articles were collected, reviewed, and analyzed. RESULTS: A total of 2165 articles were published on EDH from 1949 to 2020, and the top 100 most-cited ones were published between 1966 and 2014, receiving an average citation of 84.7 per paper. Most papers were published in Neurosurgery and Journal of Neurosurgery (JNS). 48% of the most-cited articles on EDH originated from the United States of America (USA). Notably, studies on spinal EDH represented 75% of the most-cited articles in our review. The most-cited article on EDH was published by Lawton et al. in 1995, receiving a total of 412 citations at an annual citation rate of 16.4%. CONCLUSION: This report identifies the most influential publications on EDH as well as the publications trends over the last 70 years. Recognition of the most impactful work is an important tool for clinicians and researchers as it can reflect the enormous changes in the clinical practice. This report can serve as a guide for developing evidence-based practices and identifying areas of research inadequacy.

A bibliometric analysis on the most-cited publications on carotid endarterectomy throughout history.

OBJECTIVE: Carotid endarterectomy (CEA) is the gold standard surgical procedure for managing carotid stenosis due to atherosclerosis and reducing the risk of ischemic stroke. This bibliometric analysis summarizes the most-cited articles on CEA and highlights the contributing articles to today's evidence-based practice. METHODS: A title-specific search using the Scopus database was used to perform the search. Pertinent article-based, journal-based, and author-based parameters were obtained for review. RESULTS: A total of 6,824 articles were published between 1970 and 2020. The top 100 most-cited articles accumulated a total of 54,153 citations with an average citation count (CC) of 541, with only a 4.53% self-citation rate for all authors. The publication trends peaked between 1997 and 2010, in which two-third of the highly cited works were published. The most prolific categories with top citations are the clinical, indications, and management, in a descending order. There were 41 published Randomized Controlled Trials (RCT) in the most-cited list. CONCLUSIONS: Citation analysis on carotid endarterectomy has witnessed a marked shift in the publication trends from studying the outcome and complications to comparing carotid stenting with endarterectomy. This analysis is a good introductory article to physicians interested in this topic, as it summarizes the highly impactful articles and enlists the most-cited RCT on CEA.

A potential new brainstem reflex: The oculoglossal phenomenon.

BACKGROUND: A synchronized involuntary movement of the tongue to the same side as voluntary movements of the eyes, termed the oculoglossal phenomenon, has been observed. A description of the hypothesized pathway of this phenomenon could guide the development of a rapid clinical evaluation of the long segment of the brainstem and help facilitate further studies to establish a new reflex, if possible. The aim of this study is to describe and propose the simple concept of this pathway/phenomenon, the oculoglossal phenomenon. METHODS: This is an observational study. Of a newly observe brainstem phenomenon evaluated on a subject at the National Neuroscience Institute in king Fahad Medical City (KFMC), Riyadh, Saudi Arabia. After being observed incidentally in a single patient, 60 participants were tested between January and March 2020 to confirm the presence of the phenomenon. Each subject was instructed to protrude the tongue and then move their eyes horizontally to the side. If the tongue simultaneously and involuntarily moved to the same side as the eyes, the test was deemed confirmatory. A literature review was performed, and possible anatomical pathway was proposed. RESULTS: The oculoglossal reflex was present in most (50/60, 83.3%) of the subjects. Our proposed pathway begins at the frontal cortex, followed by a projection to the paramedian pontine reticular formation, then to the contralateral medial longitudinal fasciculus and bilaterally to the hypoglossal nuclei. CONCLUSION: An accurate description of this phenomenon could lead to additional studies and possibly establishing it as a legitimate reflex, thus conceivably adding a new tool in the neurological examination to evaluate the brainstem's integrity.

The Top-100 most cited articles on Moyamoya disease: A bibliometric analysis.

OBJECTIVE: Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular phenomenon with unknown pathogenesis. Considering the abundance of articles addressing Moyamoya disease, a detailed analysis concerning the publication trends is of paramount importance. The aim of the study is to report the current knowledge of the top-100 most cited articles on Moyamoya disease in the literature. METHODS: A non-time restricted keyword-based search was performed in June 2020 using the Scopus database. The search keywords included the following: "Moyamoya", "Moyamoya disease", and "Moyamoya syndrome". The search result was used to rank the articles based on their citation count. The top-100 most-cited articles were obtained and classified into seven categories. RESULTS: A total of 3,543 articles on Moyamoya disease were published between 1955 and 2020. The Top-100 articles were published between 1977 and 2016 with a total of 16,119 citations, per year, and 7.23% rate of self-citation. The 1990s was the most productive decade (N=42). The most contributing country to the list was Japan (N=60). Stroke was the most active journal (N=23). Houkin, K., a Japanese neurosurgeon, was the most prolific author (N=15). CONCLUSIONS: Moyamoya disease has been extensively investigated in the literature throughout the years. The majority of articles published in the literature were addressing the surgical management and clinical outcome. Authors from neurosurgical backgrounds were the most active contributors to the field of Moyamoya disease.

Bibliometric analysis of the top 100 most-cited articles on astrocytoma.

BACKGROUND: Citation analysis reflects the scientific recognition and influential performance of a published article within its field. We aim to identify the top 100 most-cited articles on astrocytoma using this bibliometric analysis method. METHODS: In May 2020, we performed a thorough search in the Scopus database using the word "Astrocytoma." The top 100 most-cited articles were arranged based on citation count in descending order. The resultant articles were then analyzed with an assessment of pertinent factors. RESULTS: The most-cited articles on astrocytoma had been cited 23,720 times. The top-cited article received a total of 682 citations, with an average of 34.1 citations annually. The list comprised eight clinical trials, in which the highest cited article received 625 citations. Articles were published from 1975 to 2015 with the 1995-2005 era as the most prolific period. Neuropathology studies were the most studied category, followed by clinical studies. The United States of America was the most significant contributor, with 49 published articles. The University of California San Francisco was the most contributing institution by producing 11 articles. Articles were published in 32 different journals led by the Cancer Research Journal, with a total of 12 publications. Approximately 160 authors contributed to the list in which Scheithauer, B.W. contributed the most with a total of eight articles. CONCLUSION: This report clustered the most impactful articles on astrocytoma. It serves as an adequate tool to identify publication trends and helps in achieving evidence-based clinical practice.

The Top-100 most cited articles on dural arteriovenous fistula: A bibliometric analysis.

OBJECTIVE: Dural arteriovenous fistula (DAVF) is a rare pathological vascular lesion with variable clinical manifestations. Since 1968, several articles have been published to address spinal and cranial DAVFs. The aim of this study is to identify, analyse, and review the pertinent literature of the top-100 most cited articles on DAVFs published to date. METHODS: A title-specific, keyword-based search with no time restriction was performed in June 2020. The Scopus database was used to identify the top-100 most cited articles on DAVFs. The term "dural arteriovenous fistula" was used as a search keyword. The search results were arranged in descending order based on the total citation count. The top-100 articles were categorized into ten categories. RESULTS: Between 1968 and 2020, a total of 2298 articles were published on DAVFs. The top-100 most cited articles were published between 1983 and 2012. The total number of citations for the top-100 articles was 12393 (123 citations/article). Most articles (34%) were investigating the clinical aspect of DAVFs. The country contributing to the most impactful and highest volume of publications (46%) was the United States. The Mayo Clinic was the most active institute in contribution. Most articles (29%) were published by the Journal of Neurosurgery. CONCLUSIONS: In the top-100 most cited articles on DAVF, most studies were published in neurosurgery/neuroradiology-dedicated journals. This bibliometric analysis identifies the publication trends and provides a comprehensive overview of the most influential articles addressing DAVFs.

Bibliometric analysis of the top 100 most cited articles on craniosynostosis.

BACKGROUND: Craniosynostosis is the premature closure of cranial sutures and it continues to be a therapeutic challenge due to the diversity and complexity of the syndrome. Bibliometric analysis is a study of ranking citations and exploring the most impactful articles in a respective discipline. It also demonstrates the chronological trends of publications. METHODS: In May 2020, we performed a title-specific search of the Scopus database using "craniosynostosis" as our query term without publication date restrictions. The top 100 articles in craniosynostosis were retrieved and analyzed. RESULTS: The top 100 most-cited articles in craniosynostosis received a total 13,826 citations, and an average of 138 citations per paper. The publication dates ranged from 1920 to 2015, with a peak period of top publications between 1996 and 2005. The most common category is clinical, followed by neurogenetics. The top cited article received 540 citation counts and 19.29 citations per year. The USA was the most contributing country to the list. The Journal of Plastic and Reconstructive Surgery published the largest number of top cited articles. Neurosurgery as a specialty contributed to most articles in the list (27 articles). The institute who contributed the most was the Assistance Publique Hopitaux Paris. CONCLUSION: Bibliometric analysis in craniosynostosis revealed major trend changes of research over the years, with a focus on neurogenetics and the different types of surgical corrections. The current collection of highly cited publications may assist physicians in gaining a better understanding of the evidence-based approach in craniosynostosis.

Bibliometric Analysis of the Top 100 Most-Cited Articles on Intracranial Flow Diversion.

BACKGROUND: Flow diverters are novel, contemporary tools in treating intracranial aneurysms. There is rapid publication turnover, with newer devices introduced and expanding indications. The bibliometric analysis measures the impact of particular articles and summarizes the chronologic trends overall. METHODS: In July 2020, we performed a thorough search of the Scopus database using the terms "flow diversion," "flow diverter," and "flow diverting stent." The top 100 most impactful articles were arranged based on citation count in descending order. The collected articles were then analyzed with an assessment of relevant factors. RESULTS: Almost 1671 articles were published between 2007 and 2020. The gathered top 100 most-cited articles amassed 10,035 citations, with an average citation count of 100.35. An 8.8% self-citation rate was identified for all authors. The publication trends peaked in 2012, in which 24% of articles were published. Most prolific categories with top citations are on pipeline embolization, followed by the SILK flow diverter category. The United States published the highest number of articles. The University of Buffalo and Mayo Clinic published most of the articles among other institutions. American Journal of Neuroradiology was the most productive journal by producing 28 articles. CONCLUSIONS: This bibliometric analysis shows significant chronologic trends, with a shift from usefulness and outcome to short-term and long-term complications. Areas to improve in flow diverter research can be addressed after this analysis of the most impactful articles on this topic.

Bibliometric Analysis of the Top 100 Most Cited Articles on Cerebral Vasospasm.

OBJECTIVE: Bibliometric analysis reflects the scientific recognition and influential performance of a published article within its field. Our aim is to identify and analyze the top 100 most-cited articles on cerebral vasospasm. METHODS: A title-specific search was carried out using the Scopus database. The top 100 cited articles including the keywords "Cerebral Vasospasm" AND "Vasospasm" were retrieved and stratified in a descending order: title, authors, institution, publishing journal, country of origin, year of publication, and topic of each article were studied. RESULTS: The top 100 articles have an accumulative citation count of 20,972, with 209 average citations per article. Publication dates ranged from 1968 to 2012, with the most productive years between 1998 and 2005. Clinical studies are the most frequent category, followed by pathophysiology. The list includes 7 clinical trials, which received accumulative citations of 1525. The top cited article had received 2109 citations, with 52.7 citations per year. The top 100 articles were published across 14 countries, with most originating from the United States. The lead research institution was the University of Alberta. The most used journal was Journal of Neurosurgery. CONCLUSIONS: Bibliometric analysis has garnered major interest in recent years. It shows the publication trends, knowledge evolution, and evidence-based practice throughout the years. The collection of highly cited articles may assist physicians in gaining a better understanding of the nature of cerebral vasospasm and optimize their clinical practice.

The Top-100 most cited articles on dural arteriovenous fistula: A bibliometric analysis

Objective@#Dural arteriovenous fistula (DAVF) is a rare pathological vascular lesion with variable clinical manifestations. Since 1968, several articles have been published to address spinal and cranial DAVFs. The aim of this study is to identify, analyse, and review the pertinent literature of the top-100 most cited articles on DAVFs published to date. @*Methods@#A title-specific, keyword-based search with no time restriction was performed in June 2020. The Scopus database was used to identify the top-100 most cited articles on DAVFs. The term “dural arteriovenous fistula” was used as a search keyword. The search results were arranged in descending order based on the total citation count. The top-100 articles were categorized into ten categories. @*Results@#Between 1968 and 2020, a total of 2298 articles were published on DAVFs. The top-100 most cited articles were published between 1983 and 2012. The total number of citations for the top-100 articles was 12393 (123 citations/article). Most articles (34%) were investigating the clinical aspect of DAVFs. The country contributing to the most impactful and highest volume of publications (46%) was the United States. The Mayo Clinic was the most active institute in contribution. Most articles (29%) were published by the Journal of Neurosurgery. @*Conclusions@#In the top-100 most cited articles on DAVF, most studies were published in neurosurgeryeuroradiology-dedicated journals. This bibliometric analysis identifies the publication trends and provides a comprehensive overview of the most influential articles addressing DAVFs.

The Top-100 most cited articles on Moyamoya disease: A bibliometric analysis

Objective@#Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular phenomenon with unknown pathogenesis. Considering the abundance of articles addressing Moyamoya disease, a detailed analysis concerning the publication trends is of paramount importance. The aim of the study is to report the current knowledge of the top-100 most cited articles on Moyamoya disease in the literature. @*Methods@#A non-time restricted keyword-based search was performed in June 2020 using the Scopus database. The search keywords included the following: “Moyamoya”, “Moyamoya disease”, and “Moyamoya syndrome”. The search result was used to rank the articles based on their citation count. The top-100 most-cited articles were obtained and classified into seven categories. @*Results@#A total of 3,543 articles on Moyamoya disease were published between 1955 and 2020. The Top-100 articles were published between 1977 and 2016 with a total of 16,119 citations, per year, and 7.23% rate of self-citation. The 1990s was the most productive decade (N=42). The most contributing country to the list was Japan (N=60). Stroke was the most active journal (N=23). Houkin, K., a Japanese neurosurgeon, was the most prolific author (N=15). @*Conclusions@#Moyamoya disease has been extensively investigated in the literature throughout the years. The majority of articles published in the literature were addressing the surgical management and clinical outcome. Authors from neurosurgical backgrounds were the most active contributors to the field of Moyamoya disease.

A bibliometric analysis on the most-cited publications on carotid endarterectomy throughout history

Objective@#Carotid endarterectomy (CEA) is the gold standard surgical procedure for managing carotid stenosis due to atherosclerosis and reducing the risk of ischemic stroke. This bibliometric analysis summarizes the most-cited articles on CEA and highlights the contributing articles to today’s evidence-based practice. @*Methods@#A title-specific search using the Scopus database was used to perform the search. Pertinent article-based, journal-based, and author-based parameters were obtained for review. @*Results@#A total of 6,824 articles were published between 1970 and 2020. The top 100 most-cited articles accumulated a total of 54,153 citations with an average citation count (CC) of 541, with only a 4.53% self-citation rate for all authors. The publication trends peaked between 1997 and 2010, in which two-third of the highly cited works were published. The most prolific categories with top citations are the clinical, indications, and management, in a descending order. There were 41 published Randomized Controlled Trials (RCT) in the most-cited list. @*Conclusions@#Citation analysis on carotid endarterectomy has witnessed a marked shift in the publication trends from studying the outcome and complications to comparing carotid stenting with endarterectomy. This analysis is a good introductory article to physicians interested in this topic, as it summarizes the highly impactful articles and enlists the most-cited RCT on CEA.

The Top-100 most cited articles on dural arteriovenous fistula: A bibliometric analysis

Objective@#Dural arteriovenous fistula (DAVF) is a rare pathological vascular lesion with variable clinical manifestations. Since 1968, several articles have been published to address spinal and cranial DAVFs. The aim of this study is to identify, analyse, and review the pertinent literature of the top-100 most cited articles on DAVFs published to date. @*Methods@#A title-specific, keyword-based search with no time restriction was performed in June 2020. The Scopus database was used to identify the top-100 most cited articles on DAVFs. The term “dural arteriovenous fistula” was used as a search keyword. The search results were arranged in descending order based on the total citation count. The top-100 articles were categorized into ten categories. @*Results@#Between 1968 and 2020, a total of 2298 articles were published on DAVFs. The top-100 most cited articles were published between 1983 and 2012. The total number of citations for the top-100 articles was 12393 (123 citations/article). Most articles (34%) were investigating the clinical aspect of DAVFs. The country contributing to the most impactful and highest volume of publications (46%) was the United States. The Mayo Clinic was the most active institute in contribution. Most articles (29%) were published by the Journal of Neurosurgery. @*Conclusions@#In the top-100 most cited articles on DAVF, most studies were published in neurosurgeryeuroradiology-dedicated journals. This bibliometric analysis identifies the publication trends and provides a comprehensive overview of the most influential articles addressing DAVFs.

The Top-100 most cited articles on Moyamoya disease: A bibliometric analysis

Objective@#Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular phenomenon with unknown pathogenesis. Considering the abundance of articles addressing Moyamoya disease, a detailed analysis concerning the publication trends is of paramount importance. The aim of the study is to report the current knowledge of the top-100 most cited articles on Moyamoya disease in the literature. @*Methods@#A non-time restricted keyword-based search was performed in June 2020 using the Scopus database. The search keywords included the following: “Moyamoya”, “Moyamoya disease”, and “Moyamoya syndrome”. The search result was used to rank the articles based on their citation count. The top-100 most-cited articles were obtained and classified into seven categories. @*Results@#A total of 3,543 articles on Moyamoya disease were published between 1955 and 2020. The Top-100 articles were published between 1977 and 2016 with a total of 16,119 citations, per year, and 7.23% rate of self-citation. The 1990s was the most productive decade (N=42). The most contributing country to the list was Japan (N=60). Stroke was the most active journal (N=23). Houkin, K., a Japanese neurosurgeon, was the most prolific author (N=15). @*Conclusions@#Moyamoya disease has been extensively investigated in the literature throughout the years. The majority of articles published in the literature were addressing the surgical management and clinical outcome. Authors from neurosurgical backgrounds were the most active contributors to the field of Moyamoya disease.