Cognitive-communication performance following mild traumatic brain injury: Influence of sex, age, education, site of lesion and bilingualism.

BACKGROUND: Although previous research studies have defined several prognostic factors that affect cognitive-communication performance in patients with all traumatic brain injury (TBI) severity, little is known about what variables are associated with cognitive-communication impairment in complicated mild TBI (mTBI) specifically. AIMS: To determine which demographic and trauma-related factors are associated with cognitive-communication performance in the early recovery phase of acute care following a complicated mTBI. METHODS & PROCEDURES: Demographic and accident-related data as well as the scores on cognitive-communication skill measures in the areas of auditory comprehension (complex ideational material subtest of the Boston Diagnostic Aphasia Examination), verbal reasoning (verbal absurdities subtest of the Detroit Test of Learning Aptitude), confrontation naming (short form of the Boston Naming Test), verbal fluency (semantic category and letter category naming), and conversational discourse (conversational checklist of the Protocole Montréal d'évaluation de la communication) were retrospectively collected from the medical records of 128 patients with complicated mTBI admitted to a tertiary care trauma hospital. Multiple linear regressions analyses were carried out on the variables sex, age, education level, Glasgow Coma Scale (GCS) score, lesion site and bilingualism. OUTCOMES & RESULTS: Females performed better than males on letter-category naming, while those more advanced in age performed worse on most cognitive-communication measures. Patients with higher education achieved better confrontation and letter-category naming, whereas reading comprehension results were worse with a lower GCS score. Bilingual individuals presented more difficulty in conversational discourse skills than those who spoke only one language. In terms of site of lesion, the presence of a right frontal injury was associated with worse auditory and reading comprehension and an occipital lesion was related to worse confrontation naming. CONCLUSIONS & IMPLICATIONS: Cognitive-communication skills should be evaluated early in all patients with complicated mTBI, but especially in those who are advanced in age, those with fewer years of education and those who present with lower GCS scores, in order to determine rehabilitation needs. The findings of this study will allow acute care clinicians to better understand how various demographic and injury-related factors affect cognitive-communication skills after complicated mTBI and to better nuance the interpretation of their evaluation results in order to improve clinical care. Further study is required regarding the influence of lesion location, sex and bilingualism following complicated mTBI. What this paper adds What is already known on the subject In early acute recovery studies including all severity of TBI, cognitive-communication performance was poorer in individuals with more advanced age, those with fewer years of education and with more severe TBI. It is not yet known which demographic and injury-related variables predict cognitive-communication performance after a complicated mTBI specifically. What this paper adds to existing knowledge We confirmed that age, level of education and TBI severity, as measured with the GCS score, were associated with some areas of cognitive-communication performance for a group of patients in the acute stage of recovery from a complicated mTBI. We also identified that sex, bilingualism and site of lesion were new variables that show an influence on aspects of cognitive-communication skills in this group of patients. What are the potential or actual clinical implications of this work? The findings of this study on prognostic factors in the case of complicated mTBI will help acute care clinicians to better understand evaluation results knowing the variables that can influence cognitive-communication performance and to nuance the interpretation of these results with the goal of determining rehabilitation needs and enhancing clinical care.

Cognitive-communication skills and acute outcome following mild traumatic brain injury.

PURPOSE: Little is known about cognitive-communication skills post mild traumatic brain injury (mTBI). We aimed to determine how performance on cognitive-communication measures in the acute recovery period relates to early outcome following complicated mTBI. METHOD: Results of language and communication skill measures, demographic and accident-related data, length of stay (LOS), Glasgow Outcome Scale-Extended (GOSE) scores and discharge destinations were retrospectively gathered for 128 admitted patients with complicated mTBI. RESULTS: More than half of the individuals required rehabilitation services post discharge from hospital with over a third needing in-patient rehabilitation. Patients with poorer skills in auditory comprehension, verbal reasoning, confrontation naming, verbal fluency and conversational discourse were more likely to require in-patient rehabilitation. Subjects with worse skills in naming, conversational discourse and letter-category verbal fluency had a greater chance of being referred to out-patient rehabilitation services. Thus patients with both auditory comprehension and oral expression deficits were more likely to require in-patient services whereas those who had oral expression deficits but no significant difficulty in auditory comprehension were more often referred to out-patient services. Also, worse conversational discourse skills and semantic-category naming ability were related to lower GOSE scores and the chance of a longer LOS was greater when letter-category naming was poorer. CONCLUSION: The likelihood of individuals requiring rehabilitation services post mTBI was related to performance on several oral expression and auditory comprehension measures. It is therefore important to evaluate cognitive-communication skills early to determine rehabilitation needs.

Accuracy and Safety of External Ventricular Drain Placement by Physician Assistants and Nurse Practitioners in Aneurysmal Acute Subarachnoid Hemorrhage.

BACKGROUND: In the current dynamic health environment, increasing number of procedures are being completed by advanced practitioners (nurse practitioners and physician assistants). This is the first study to assess the clinical outcomes and safety of external ventricular drain (EVD) placements by specially trained advanced practitioners. OBJECTIVE: Compare the safety and outcomes of EVD placement by advanced practitioners in patients with subarachnoid hemorrhage (SAH). METHODS: A cohort comparison study was performed from an aneurysmal SAH database selecting patients treated with EVD from a single major academic institution in the USA between June 2007 and June 2017. Safety, accuracy, and complications of EVD placement were compared between advanced practitioners and neurosurgical physicians (attending neurosurgeon and subspecialty clinical fellow). Statistical analysis was performed using the Mann-Whitney test for continuous variables and χ2 test for categorical variables, with p values set at < 0.05 for significance. RESULTS: We identified 203 patients for this cohort with 238 EVD placements; eighty-seven (36.6%) placements were performed by advanced practitioners and 151 (63.4%) by neurosurgeons. Most of the ventriculostomies were placed in the emergency room (n = 114; 47.9%). Additional procedures performed concurrently with the EVD placements were significantly higher among the physicians' group (21.8 vs. 4.6%; p < 0.001). Bedside placement and usage of Ghajar guide were significantly higher among advanced practitioner's (58.3 vs. 98.9 and 9.9 vs. 64.4%, respectively, with a p < 0.001 for both). There were, however, no significant differences in terms of the number of attempts for insertion, intraprocedural complications, tract hemorrhages, accuracy, infection rates, catheter dislodgments, and need for repositioning/replacement of EVD. CONCLUSION: After appropriate training, EVD placement can be safely performed by advanced practitioners with an adequate accuracy of placement.

Basilar artery vasospasm after pretuncal non-aneurysmal subarachnoid hemorrhage responding to milrinone.

Pretruncal (perimesencephalic) non-aneurysmal subarachnoid hemorrhage (PNSAH) is uniformly associated with an excellent outcome. Although cerebral vasospasm remains a common complication of SAH and constitutes an important predictor of outcome, in the setting of PNSAH, it is extremely rare. Preturnal non-aneurysmal subarac refers to a subset of SAH patients with a characteristic pattern of localized blood on CT of the head, normal cerebral angiography, and benign course when compared to the aneurysmal SAH population. The presence of radiological or even clinical vasospasm does not exclude the diagnosis of PNSAH. To our knowledge, this is the first case of symptomatic cerebral vasospasm due to PNSAH that responded to milrinone.

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.

Initial experience with Scepter Mini dual lumen balloon for embolization of cerebrovascular diseases.

OBJECTIVE: Endovascular treatment of cerebrovascular diseases is often challenging due to small caliber, tortuous distal vessels. Several devices and techniques have evolved to overcome these challenges. Recently, a low profile dual lumen microballoon catheter, specifically designed for distal navigation is employed for neurovascular procedures. Due to its recent advent, scarce data is available on clinical utility and safety of Scepter Mini. The aim of this case series is to report our initial experience with Scepter Mini in the management of various cerebrovascular diseases. METHODS: All interventional neurovascular cases performed using Scepter Mini between January 2020 till April 2021 were included. Data regarding patient demographics, procedural details and complications was retrospectively collected from patient's electronic medical record and procedure reports. RESULTS: Total twelve embolization procedures were performed in eleven patients, including six brain arteriovenous malformation, two dural arteriovenous fistula, one vein of Galen malformation and three hyper-vascular glomus tumor embolizations. All procedures were successfully performed with adequate penetration of the embolic agent. Complete embolization was performed in six procedures, while intended partial embolization was performed in the rest of procedures. Scepter Mini was solely used in ten procedures, however in the other two embolization procedures it was used as an additional conjunct tool to complete the intended embolization. No balloon related complication was observed in any procedure. CONCLUSIONS: Scepter Mini dual lumen microballoon catheter is safe and feasible for delivery of liquid embolic agents for cerebrovascular embolization procedures.

Effectiveness of Lumbar Cerebrospinal Fluid Drain Among Patients With Aneurysmal Subarachnoid Hemorrhage: A Randomized Clinical Trial.

Importance: After aneurysmal subarachnoid hemorrhage, the use of lumbar drains has been suggested to decrease the incidence of delayed cerebral ischemia and improve long-term outcome. Objective: To determine the effectiveness of early lumbar cerebrospinal fluid drainage added to standard of care in patients after aneurysmal subarachnoid hemorrhage. Design, Setting, and Participants: The EARLYDRAIN trial was a pragmatic, multicenter, parallel-group, open-label randomized clinical trial with blinded end point evaluation conducted at 19 centers in Germany, Switzerland, and Canada. The first patient entered January 31, 2011, and the last on January 24, 2016, after 307 randomizations. Follow-up was completed July 2016. Query and retrieval of data on missing items in the case report forms was completed in September 2020. A total of 20 randomizations were invalid, the main reason being lack of informed consent. No participants meeting all inclusion and exclusion criteria were excluded from the intention-to-treat analysis. Exclusion of patients was only performed in per-protocol sensitivity analysis. A total of 287 adult patients with acute aneurysmal subarachnoid hemorrhage of all clinical grades were analyzable. Aneurysm treatment with clipping or coiling was performed within 48 hours. Intervention: A total of 144 patients were randomized to receive an additional lumbar drain after aneurysm treatment and 143 patients to standard of care only. Early lumbar drainage with 5 mL per hour was started within 72 hours of the subarachnoid hemorrhage. Main Outcomes and Measures: Primary outcome was the rate of unfavorable outcome, defined as modified Rankin Scale score of 3 to 6 (range, 0 to 6), obtained by masked assessors 6 months after hemorrhage. Results: Of 287 included patients, 197 (68.6%) were female, and the median (IQR) age was 55 (48-63) years. Lumbar drainage started at a median (IQR) of day 2 (1-2) after aneurysmal subarachnoid hemorrhage. At 6 months, 47 patients (32.6%) in the lumbar drain group and 64 patients (44.8%) in the standard of care group had an unfavorable neurological outcome (risk ratio, 0.73; 95% CI, 0.52 to 0.98; absolute risk difference, -0.12; 95% CI, -0.23 to -0.01; P = .04). Patients treated with a lumbar drain had fewer secondary infarctions at discharge (41 patients [28.5%] vs 57 patients [39.9%]; risk ratio, 0.71; 95% CI, 0.49 to 0.99; absolute risk difference, -0.11; 95% CI, -0.22 to 0; P = .04). Conclusion and Relevance: In this trial, prophylactic lumbar drainage after aneurysmal subarachnoid hemorrhage lessened the burden of secondary infarction and decreased the rate of unfavorable outcome at 6 months. These findings support the use of lumbar drains after aneurysmal subarachnoid hemorrhage. Trial Registration: ClinicalTrials.gov Identifier: NCT01258257.

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.

External Carotid Artery-Jugular Vein Fistula Caused by Blunt Trauma: A Case Report & Literature Review.

Acquired carotid-jugular fistula usually occurs due to neck stab wounds, gunshots, or central vein catheterization. Blunt trauma is a rare cause. These cases usually present with pulsatile swelling, tinnitus, and continued thrills in the neck. Both surgical and endovascular options have been used to manage these fistulas. Coil embolization is also applied in high-flow fistulas. We present a case of a 38-year-old woman free of any pre-existing medical conditions, presenting with a fistula between the external carotid artery and external jugular vein distally and with a high flow. She was treated with fistula embolization using coils while limiting the high flow via a balloon in the jugular vein. Our case highlights the possibility of using coils in high-flow fistulas in anatomically challenging fistulas. Furthermore, relevant literature review is presented to recapitulate unique features and effective management of carotid-jugular fistulas.

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.

Common ground, different path: Ulnar artery access for interventional neurovascular procedures.

BACKGROUND: Recently, radial artery access has gained popularity for interventional neurovascular procedures due to patient comfort and fewer complications. However, there are instances where the radial artery approach is not feasible. In such cases, trans-ulnar artery access (TUA) can offer an alternate route. There is limited data regarding neuro-interventional procedures performed via this approach. This study aims to evaluate the feasibility and safety of trans-ulnar approach for a wide range of interventional neurovascular procedures. MATERIALS AND METHODS: The data for all patients who underwent ulnar artery access for diagnostic or interventional neuroradiology procedures was retrospectively collected between September 2020 and March 2021. Patient demographics, procedural details, procedure success, and complications were recorded. RESULTS: During the study period, 23 patients underwent 24 trans-ulnar approach procedures. The mean age of patients was 50.1 ± 14.2 years. Fourteen diagnostic cerebral angiograms and ten interventional procedures were performed. All procedures were successfully completed via trans-ulnar approach without a switch to alternate access. No major access site complication was observed. CONCLUSION: Ulnar artery access is a safe and feasible option for neurovascular procedures. It can be effectively utilized for diagnostic cerebral angiography and a wide range of interventional procedures.

Effect of cerebrospinal fluid drainage on clinical outcomes following aneurysmal subarachnoid hemorrhage.

OBJECTIVES: We study the relationship between external ventricular drainage (EVD) of cerebrospinal fluid output and functional outcomes in patients with aneurysmal subarachnoid hemorrhage (aSAH). METHODS: A retrospective chart review of patients presenting to a single center with aSAH was performed. The primary outcome was good functional outcomes assessed by a composite of the modified Rankin scale (mRS 0-2) at last follow-up. Secondary outcomes were clinical and radiographic vasospasm. For data analysis, multivariable generalized estimating equations adjusting for potential confounders were used. RESULTS: A total of 119 patients were included; 91 (75.6%) presented with a modified Fisher grade 4 and 76 (63.9%) had hydrocephalus. The median EVD duration was 13 days. On average, most EVDs were set at 15 cmH2O (50, 42%). Follow-up was available in 109 patients; median time was 10.7 months; 69 (63.3%) had good outcomes. Multivariable analysis showed that EVDs set at 10 cmH2O had increased odds of good outcomes for every ml increase in the EVD output (OR = 1.02; 95% CI 1.01-1.03; p = 0.001). Post estimation analyses show that EVDs at 10 cmH2O with output close to 200 ml predicted a 50% probability of good outcomes. CONCLUSIONS: Increased EVD outputs were associated with favorable outcomes at the last follow-up.

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.

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.

Salvage flow diverter stent across the posterior communicating artery for persistent retrograde filling of a giant internal carotid artery aneurysm after parent vessel occlusion.

Giant internal carotid artery (ICA) aneurysms are complex vascular lesions which are difficult to treat with open as well as endovascular surgery. Parent vessel occlusion is a well-established treatment option for such aneurysms. However, there have been a few reported cases of ruptured aneurysms related to the persistent retrograde filling after parent vessel occlusion. We report a case which highlights the usage of the flow diverter stent as a potential treatment strategy for the management of retrograde filling of aneurysms. A 54-year-old female was found to have a giant left ICA aneurysm on a brain magnetic resonance imaging during workup for headaches. She underwent occlusion of the left ICA proximal to the aneurysm using multiple coils. However, follow up angiograms after 6 months and 2 years demonstrated persistent retrograde filling of the left ICA aneurysm through the posterior communicating (PCOM) artery. Eventually, she was successfully treated with a flow diverter stent across the PCOM artery into the distal ICA. Follow up angiogram after 6 months showed patent flow in the PCOM artery and the distal ICA. with complete occlusion of the aneurysm. Using a flow diverter stent after insufficient parent vessel occlusion for giant intracranial aneurysms may be a feasible treatment option and an addition to the neurovascular treatment armamentarium.

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.

Exploring the path less traveled: Distal radial access for diagnostic and interventional neuroradiology procedures.

BACKGROUND: Recently there is a trend for radial first which advocates radial artery access as the first choice to perform diagnostic and interventional neurovascular procedures. Although safer than the conventional common femoral artery access, it is associated with a high rate of radial artery occlusion. Distal radial artery access is recently proposed to avoid this complication. This study aims to assess the feasibility and safety of distal radial artery access across a wide range of interventional neurovascular procedures. MATERIALS AND METHODS: All Interventional neurovascular cases attempted via distal radial artery access from September 2019 till March 2021 were included in the study. Data regarding patient demographics, distal radial artery diameter, access site cannulation, size of the sheath, procedural details including success rate and complications were collected. RESULTS: During the study period, 102 patients underwent 114 procedures via the distal radial artery approach. The mean age of patients was 41.9 ± 15.2 years. Overall procedure success rate via DRA was 94.7% (108/114). 72 diagnostic cerebral angiograms and 36 interventional procedures were successfully completed while six procedures required switching to alternate access. CONCLUSION: Distal radial artery access is a safe and feasible option for diagnostic cerebral angiography and a wide range of neurovascular procedures.

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.