Introduction There have been several studies of Moyamoya disease (MMD) with concurrent autoimmune (AI) disease. It has been proposed that there may be a causal or synergistic effect of autoimmune and inflammatory conditions in patients with MMD. As such, in the present study we seek to characterizethe surgical outcomes of adults and children with MMD and coexisting AI conditions. Methods A systematic review was performed of published studies reporting patient level data of Moyamoya disease and concomitant autoimmune disease. Relevant studies were identified using Preferred reporting Items for Systematic Reviews and Meta‐Analysis criteria. Results A total of 149 studies were included, encompassing 257 patients. Of the 257 patients with concurrent AI and MMD, 107 (88 females, 19 males) underwent surgery. The average age was 31.3 years (Range 5–65; SD 12.5). Five cases presented with hemorrhagic symptoms, 87 ischemic, and 15 were unspecified. The most commonly associated AI conditions were Graves’ disease (71 cases, 66.4%), Lupus (17 cases, 15.9%), and T1DM (6 cases, 5.6%).Follow‐up was reported in 25 cases and was an average of 17.8 months. Surgical outcomes were reported in 82 cases; 63 (76.8%) improved, 7 (8.5%) were stable, 9 (10.9%) developed a new deficit, and 3 (3.7%) died at most recent follow up. Eight patients (7.5%) had disease progression. 63 (58.8%) patients were treated with additional autoimmune therapy although this therapy did not lead to any difference in outcomes. Twenty patients (18.7%) required additional supportive medical therapy (eg: aspirin, statins). Conclusions Moyamoya disease may be multifactorial. Patients with concomitant AI and Moyamoya disease may be at increased risk and warrant additional surveillance or intervention. The ideal therapy for these patients remains to be understood. These findings warrant further study into optimal diagnostic and therapeutic techniques for concomitant MMD and AI conditions.
Abstract Number ‐ 281: Clinical and Epidemiological Characteristics of Moyamoya Disease Presenting with Concomitant Autoimmune Conditions
Introduction Moyamoya disease was first described in the 1950’s. After over six decades, however, the underlying etiology of Moyamoya still remains unknown. It has been postulated that underlying inflammatory or autoimmune (AI) processes may play a role in the development of this condition. In order to further investigate this hypothesis, we studied the clinical and epidemiological attributes of patients with concurrent Moyamoya disease and AI condition(s). Methods A systematic review was performed including studies reporting patient level data of Moyamoya disease and concomitant autoimmune disease. Relevant studies were identified using Preferred reporting Items for Systematic Reviews and Meta‐Analysis criteria. Results There were 739 results of which 149 were included in this study. The first reports of concurrent Moyamoya and AI occurred in 1991 and involved 2 cases of Moyamoya and associated Graves’ disease. These patients were treated with antithyroid medication alone and showed improvement. Altogether, our search yielded a total of 257 patients (192 females, 48 males, 17 unspecified). A total of 158 cases were reported as “Moyamoya Disease” and 99 were reported as “Moyamoya Syndrome/Vasculopathy.” Most cases were ischemic (175 ischemic, 15 hemorrhagic) in nature. Bilaterality of the disease was reported in 130 cases, unilaterality in 34 cases. The most commonly reported ethnicity was Chinese (26 cases), followed by Japanese (17 cases), and Korean (17 cases). The most common co‐presenting AI conditions were Graves’ disease (150 cases, 58.4%), Lupus (26 cases, 10.1%), antiphospholipid syndrome (19 cases, 7.4%), Type 1 Diabetes (19 cases, 7.4%), and Multiple Sclerosis (7 cases, 2.7%). Conclusions There are numerous reports describing patients diagnosed with Moyamoya in addition to coexisting AI conditions. Our study shows that the demographic characteristics of co‐presentation align with the common autoimmune (female predominance) and Moyamoya (high east Asian prevalence) attributes. These results warrant further investigation into a possible causal or synergistic relationship between Moyamoya and AI disease.
Promoting diversity in neurosurgery through a virtual symposium
OBJECTIVE The rates of women and underrepresented racial and ethnic minority (UREM) students successfully matching into neurosurgical residency are extremely low and do not reflect the makeup of the general population. As of 2019, only 17.5% of neurosurgical residents in the United States were women, 4.95% were Black or African American, and 7.2% were Hispanic or Latinx. Earlier recruitment of UREM students will help to diversify the neurosurgical workforce. Therefore, the authors developed a virtual educational event for undergraduate students entitled "Future Leaders in Neurosurgery Symposium for Underrepresented Students’’ (FLNSUS). The primary objectives of the FLNSUS were to expose attendees to 1) neurosurgeons from diverse gender, racial, and ethnic backgrounds; 2) neurosurgical research; 3) opportunities for neurosurgical mentorship; and 4) information about life as a neurosurgeon. The authors hypothesized that the FLNSUS would increase student self-confidence, provide exposure to the specialty, and reduce perceived barriers to a neurosurgical career. METHODS To measure the change in participant perceptions of neurosurgery, pre- and postsymposium surveys were administered to attendees. Of the 269 participants who completed the presymposium survey, 250 participated in the virtual event and 124 completed the postsymposium survey. Paired pre- and postsurvey responses were used for analysis, yielding a response rate of 46%. To assess the impact of participant perceptions of neurosurgery as a field, pre- and postsurvey responses to questions were compared. The change in response was analyzed, and a nonparametric sign test was performed to check for significant differences. RESULTS According to the sign test, applicants showed increased familiarity with the field (p < 0.001), increased confidence in their abilities to become neurosurgeons (p = 0.014), and increased exposure to neurosurgeons from diverse gender, racial, and ethnic backgrounds (p < 0.001 for all categories). CONCLUSIONS These results reflect a significant improvement in student perceptions of neurosurgery and suggest that symposiums like the FLNSUS may promote further diversification of the field. The authors anticipate that events promoting diversity in neurosurgery will lead to a more equitable workforce that will ultimately translate to enhanced research productivity, cultural humility, and patient-centered care in neurosurgery.