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
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
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.
Abstract Number ‐ 270: Assessing Biceps Brachii Functional Recovery after Stroke: Comprehensive Analysis of Two Decades of Patient Data
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
Introduction Severe loss of muscle strength is common following stroke, and rehabilitation in the form of targeted physical therapy is often needed to recover partial muscular function. Although lower limb weakness is typical in stroke patients, upper body complications presenting as decreased grip strength and hand motor coordination can also occur. The biceps brachii, one of the most frequently used upper body muscles, often shows weakness post‐stroke. Although recent literature has touched on various techniques to help regain biceps brachii function, studies looking at quantifiable recovery of muscular strength are limited.In this review, we summarize strength recordings of the biceps brachii from stroke patients collected over the past 20 years to better characterize upper extremity functional recovery after stroke. Methods Using PRISMA guidelines, we queried 3 databases to identify all studies reporting findings of stroke recovery including isolated strength measurement of the bicep brachii muscle in newtons (N). Results 15 studies describing 384 patients (mean age range: 55.7‐73.6 years) were included for formal analysis. Baseline biceps strength range following stroke was 12.1‐91.23 N. At 4 weeks, strength ranged from 16.3‐85.8N, and this range improved to 85.31‐97.38N and 29–125.04N at 5 and 8 weeks, respectively. Additionally,Folkerts et al. found a significant improvement in biceps function from the 5 week value (97.38N) to the 10 week final follow‐up (108.46N). Over 12 weeks post‐stroke, Donaldson and colleagues observed a significant improvement in bicep strength range (27.1‐61.0N vs. 59.5‐76.1N). Conclusions Our study is the first systematic review assessing the recovery of biceps function with time following stroke in the current literature. Singular timepoint studies showed a significant loss of bicep function post‐stroke. Multiple timepoint studies showed semi‐linear relationships between biceps strength recovery and time within studies but no patterns when comparing between studies. Further research is required to quantify biceps strength recovery in a sex‐specific manner.
Abstract Number ‐ 212: Endovascular vs. Open Surgery for Craniocervical Junction Dural Arteriovenous Fistulas: Assessing Safety and Efficacy
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
Introduction Arteriovenous fistulas at the craniocervical junction (CCAVFs) are challenging to treat due to variation in angioarchitecture and shunting sites. No consensus exists as to whether open surgery or endovascular approaches are better in regards to outcomes, complications, and need for reintervention. As such, weexplore the efficacy, outcomes, and complications of endovascular versus open surgery for dural arteriovenous fistulas at the craniocervical junction. Methods A systematic review was performed using the EMBASE, PubMed,and Cochrane databases in April 2022. MeSH search terms included (craniocervical) AND (arteriovenous OR fistula OR AVF). Inclusion criteria were defined as patients with CCAVFs who were treated with open surgery or endovascular therapy with reported clinical outcomes at 6 months or later. Results A total of 357 studies were screened; of which 75 were included. A total of 416 patients (309 males, 91 females) were included with an average age of 61.9 years. A total of 236 patients underwent surgery (mean age: 61.5), 71 patients underwent embolization (mean age: 62.6), and 25 patients underwent a combination (mean age: 54.7). Outcomes were reported in 39 surgery cases (mean follow up time: 31.4 months), and 54 endovascular cases (mean follow up time: 24.2 months). Within the open surgery group 47 (57.3%) had complete symptom resolution, 23 (28%) had symptom improvement or stabilization, 11 (13.4%) permanent deficit, and 1 (1.2%) resulted in death. Within the endovascular group 26 (56.5%) had complete symptom resolution, 5 (10.9%) had symptom improvement or stabilization, 15 (32.6%) permanent deficit, and 0 (0%) resulted in death. Conclusions Open surgery and embolization yield comparable symptomatic improvement and stabilization. However, the rate of permanent deficits is higher withembolization, while mortality is higher with open surgery. Further studies are required to better associate these outcomes to baseline parameters including treatment decision making, size of the lesion, patient age, and co‐morbidities.
Abstract Number ‐ 202: Multispecialty Involvement in Neuroendovascular Interventions
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
Introduction While neurovascular surgeries have largely been performed by neurosurgeons, the advent of endovascular procedures has produced increasing involvement of other specialists in this arena (i.e. neurologists and radiologists).This study aims to analyzethe involvement of different specialties within theneuroendovascular workforce by characterizingthem by their primary specialty. Methods The Medicare Provider Utilization Database was queried from 2013 to 2019 for all primary Current Procedural Terminology (CPT) codes identified by the American Medical Association as pertaining to endovascular procedures of the nervous system (61623, 61624, 61630, 61635, 61626, 61645, and 61650). We counted the number of unique providers in respective fields (neurology, neurosurgery, and radiology) who performed these procedures. Statistical analyses, including data aggregation, exploratory analysis, and propensity adjustment, were performed using R, version 4.0.1 (The R Foundation, Vienna, Austria). The two‐sided Mann‐Kendall trend test, a non‐parametric statistical test that assesses for monotonic trends in either an upward or downward trend, was utilized to assess for trends over time. Within each year analyzed, a Chi‐squared test for trend in proportions was used. A significance level of 0.05 was used. Results In 2013, neurosurgeons comprised 45% of the workforce performing these procedures, neurologists were 9%, and radiologists were 47%. By 2019, neurosurgeons decreased to 34.5% of the workforce, while neurologists increased to 21.5%, and radiologists decreased slightly to 44%. Neurologists were the only field in which the proportional shift in providers was noted to have a significant change in trajectory (p:0.04), an upward trajectory. Overall proportional differences between respective specialists were statistically significant each year (p< 0.01). Conclusions Neurologists are increasingly performing endovascular procedures. Increased communication and coordination between specialties can improve disparities in care to ensure adequate access to neurovascular care across the US.
Abstract Number ‐ 201: Increased Utilization of Neuro‐Endovascular Procedures: 2013–2019
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
Introduction Endovascular procedures have become increasingly popular in recent years due to preference of minimally invasive approaches and technological advancement.This study aims to characterize the trends in procedural rates of common neuro‐endovascular procedures performed by neurologists, neurosurgeons, and radiologists. Methods The Medicare Provider Utilization Database was queried from 2013 to 2019 for all primary Current Procedural Terminology codes identified by the American Medical Association as pertaining to endovascular procedures of the nervous system (61623, 61624, 61630, 61635, 61626, 61645, and 61650). Statistical analyses, including data aggregation, exploratory analysis, and propensity adjustment, were performed using R, version 4.0.1 (The R Foundation, Vienna, Austria). The two‐sided Mann‐Kendall trend test, a non‐parametric statistical test that assesses for monotonic trends in either an upward or downward trend, was utilized to assess for trends over time. A significance level of 0.05 was used. Results Between 2013 to 2019 a total of 40,730 endovascular procedures were performed by neurosurgeons, neurologists and radiologists treating Medicare patients. When all codes were combined, there was a significant increase in the number of procedures done by all specialists: Radiology (2013:1080; 2019:4938; p:0.02), Neurosurgery (2013:1230; 2019:4508; p< 0.01), and Neurology (2013:205; 2019:2397; p:0.04). Significant increases in mechanical thrombectomy (CPT61645) were observed in all fields: Radiology (2016:1287, 2019:3542; p< 0.01), Neurosurgery (2016:610; 2019:2326; p< 0.01), Neurology (2016:569; 2019:1951; p< 0.01). Increases in transcatheter permanent occlusion (CPT61624) were observed in neurosurgery (2013:1230; 2019:2049; p:0.04). Increases in temporary balloon artery occlusion (CPT61623) were noted in neurology (2013:21; 2019:27; p:0.04). Conclusions Endovascular procedures have seen a dramatic rise in utilization over a short amount of time, particularly mechanical thrombectomy. Future investigation should include more inclusive datasets beyond the Medicare population.
Abstract Number ‐ 198: Management of poor grade aneurysmal subarachnoid hemorrhage: illustrative case and literature review
Stroke: Vascular and Interventional Neurology | April 25, 2023 | Volume: 3 | Issue: S1 | Pages: No page available
Introduction Poor‐grade aneurysmal subarachnoid hemorrhage (aSAH) is associated with high mortality. Overall, 20–30% of aSAH are described as poor grade. Despite recent advances in management strategies, prognosis of poor‐grade aSAH remains dismal. As such, we review the most current literature regarding management strategies that can be used to decrease the risk of the four most common causes of secondary brain injury: 1) aneurysm re‐rupture, 2) hydrocephalus, 3) cerebral salt wasting, and 4) delayed cerebral ischemia/vasospasm. Methods We present a 46‐year‐old female patient with poor‐grade aSAH after a loss of consciousness following thunderclap headache. Examination showed a dilated left pupil and Glasgow Coma Scale (GCS) of 4. Imaging revealed a ruptured anterior communicating artery aneurysm, and she was subsequently taken to the neuro‐interventional radiology suite for EVD placement. On POD 7 Transcranial Doppler (TCD) suggested moderate vasospam of left MCA. Two successful balloon angioplasties of the basilar, ACA‐A1, and MCA‐M1 were successfully performed on separate consecutive days. By POD 10, intermittent severe ICP spikes required targeted temperature management (TTM) with hypothermia. On POD 24, her ICP normalized, she was no longer comatose, and her EVD was clamped and removed. She was rapidly weaned off ventilator and transitioned to rehabilitation. 3‐months postoperatively, Modified Rankin Scale score was 0. Results Based upon the result of our literature review, we propose maintaining blood pressure below 160 mmHg prior to intervention, after which it can be increased to 160–240 mmHg to prevent vasospasm. Transcranial Doppler is essential to detect vasospasm as the aSAH symptoms subtle; once identified, vasospasm can be successfully treated with balloon angioplasty. Finally, targeted temperature management, mannitol, hypertonic saline, and neuromuscular paralysis are essential for postoperative management of ICP levels. Conclusions The multimodal approach should be considered when managing poor grade aSAH. Further research in aSAH management would benefit the patients who present with poor prognosis, thereby improving patient care and leading to a standard, effective approach for aSAH management.
Surgical Management of Petrous Apex Cholesteatomas in the Pediatric Population
Journal of Neurological Surgery Part B: Skull Base | March 20, 2023 | Volume: 84 | Issue: S 01 | Pages: S1-S344