De novo and inherited monoallelic variants in TUBA4A cause ataxia and spasticity.

Alpha-tubulin 4A encoding gene (TUBA4A) has been associated with familial amyotrophic lateral sclerosis (fALS) and fronto-temporal dementia (FTD), based on identification of likely pathogenic variants in patients from distinct ALS and FTD cohorts. By screening a multicentric French cohort of 448 unrelated probands presenting with cerebellar ataxia, we identified ultra-rare TUBA4A missense variants, all being absent from public databases and predicted pathogenic by multiple in-silico tools. In addition, gene burden analyses in the 100,000 genomes project (100KGP) showed enrichment of TUBA4A rare variants in the inherited ataxia group compared to controls (OR: 57.0847 [10.2- 576.7]; p = 4.02 x10-07). Altogether, we report 12 patients presenting with spasticity and/or cerebellar ataxia and harboring a predicted pathogenic TUBA4A missense mutation, including 5 confirmed de novo cases and a mutation previously reported in a large family presenting with spastic ataxia. Cultured fibroblasts from 3 patients harboring distinct TUBA4A missense showed significant alterations in microtubule organisation and dynamics, providing insight of TUBA4A variants pathogenicity. Our data confirm the identification of a hereditary spastic ataxia disease gene with variable age of onset, expanding the clinical spectrum of TUBA4A associated phenotypes.

Intracranial aneurysms in patients with acute ischemic stroke prevalence and influence on mechanical thrombectomy over 14 years in a tertiary-care center.

INTRODUCTION: The prevalence of intracranial aneurysms (IA) in patients with acute ischemic stroke (AIS) requiring mechanical thrombectomy (MT) is unclear. OBJECTIVE: To describe the prevalence of IA in patients with AIS and their influence on MT. MATERIALS & METHODS: This is a retrospective cohort study on all patients admitted with a diagnosis of AIS from January 2008 to March 2022 at a tertiary academic center. The records were reviewed for demographic, clinical, imaging, and outcomes data. Only patients who had CTA at admission were included in this analysis. RESULTS: Among 2265 patients admitted with AIS, this diagnosis was confirmed in 2113 patients (93.3 %). We included 1111 patients (52.6 %) who had head CTA and 321 (28.9 %) who underwent MT. The observed prevalence of aneurysms on CTA was 4.5 % (50/1111 patients), and 8 (16 %) had multiple aneurysms. MT was performed in 7 patients harboring IAs: 6 ipsilateral (5 proximal and 1 distal to the occlusion)and 1 contralateral aneurysm.. The patient with a contralateral aneurysm had a TICI 2B score In patients with ipsilateral aneurysms, TICI 2B or 3 was achieved in 3 cases (50 %), which is significantly lower than historical control of MT (91.6 %) without IA (p = 0.01). No aneurysms ruptured during MT. The aneurysm noted distal to the occlusion was mycotic. CONCLUSION: In this analysis, the observed prevalence of IA in patients with AIS was 4.5%. Ipsilateral aneurysms (proximal or distal to the occlusion site) deserve particular attention, given the potential risk of rupture during MT. Aneurysms located distal to the occlusion were mycotic and the rate of recanization in patients with ipsilateral aneurysms was low compared to historical controls. Further studies are needed to improve the outcomes in patients with IA requiring MT.

Flexible low-profile external ventricular drain catheter for real-time brain monitoring.

External ventricular drainage is one of the most common neurosurgical procedures in the world for acute hydrocephalus, which must be performed carefully by a neurosurgeon. Although various neuromonitoring external ventricular drain (EVD) catheters have been utilized, they still suffer from rigidity and bulkiness to mitigate post-EVD placement trauma. Here, we introduce a flexible and low-profile smart EVD catheter using a class of technologies with sensitive electrical materials, seamless integration, and flexible mechanics, which serves as a highly soft and minimally invasive device to monitor electrical brain signals. This device reliably captures biopotentials in real time while exhibiting remarkable flexibility and reliability. The seamless integration of its sensory system promises a minimally invasive EVD placement on brain tissue. This work validates the device's distinct characteristics and performances through in vitro experiments and computational analysis. Collectively, this device's exceptional patient- and user-friendly attributes highlight its potential as one of the most practical EVD catheters.

Asymptomatic Females Are at Higher Risk for Perioperative TIA/Stroke and Males Are at Higher Risk for Long-Term Mortality after Carotid Artery Stenting: A Vascular Quality Initiative Analysis.

The study aims to review the sex differences with respect to transient ischemic attack (TIA)/stroke and death in the perioperative period and on long-term follow-up among asymptomatic patients treated with carotid stenting (CAS) in the vascular quality initiative (VQI). All cases reported to VQI of asymptomatic CAS (ACAS) patients were reviewed. The primary end point was risk of TIA/stroke and death in the in-hospital perioperative period and in the long-term follow-up. The secondary end point was to evaluate predictors of in-hospital perioperative TIA/stroke and mortality on long-term follow-up after CAS. There were 22,079 CAS procedures captured from January 2005 to April 2019. There were 5,785 (62.7%) patients in the ACAS group. The rate of in-hospital TIA/stroke was higher in female patients (2.7 vs. 1.87%, p = 0.005) and the rate of death was not significant (0.03 vs. 0.07%, p = 0.66). On multivariable logistic regression analysis, prior/current smoking history (odds ratio = 0.58 [95% confidence interval or CI = 0.39-0.87]; p = 0.008) is a predictor of in-hospital TIA/stroke in females. The long-term all-cause mortality is significantly higher in male patients (26.9 vs. 15.7%, p < 0.001). On multivariable Cox-regression analysis, prior/current smoking history (hazard ratio or HR = 1.17 [95% CI = 1.01-1.34]; p = 0.03), coronary artery disease or CAD (HR = 1.15 [95% CI = 1.03-1.28]; p = 0.009), chronic obstructive pulmonary disease or COPD (HR = 1.73 [95% CI = 1.55-1.93]; p < 0.001), threat to life American Society of Anesthesiologists (ASA) class (HR = 2.3 [95% CI = 1.43-3.70]; p = 0.0006), moribund ASA class (HR = 5.66 [95% CI = 2.24-14.29]; p = 0.0003), and low hemoglobin levels (HR = 0.84 [95% CI = 0.82-0.86]; p < 0.001) are the predictors of long-term mortality. In asymptomatic carotid disease patients, women had higher rates of in-hospital perioperative TIA/stroke and a predictor of TIA/stroke is a prior/current history of smoking. Meanwhile, long-term all-cause mortality is higher for male patients compared with their female counterparts. Predictors of long-term mortality are prior/current smoking history, CAD, COPD, higher ASA classification of physical status, and low hemoglobin level. These data should be considered prior to offering CAS to asymptomatic female and male patients and careful risks versus benefits discussion should be offered to each individual patient.

Awake Craniotomy Program Implementation.

Importance: Implementing multidisciplinary teams for treatment of complex brain tumors needing awake craniotomies is associated with significant costs. To date, there is a paucity of analysis on the cost utility of introducing advanced multidisciplinary standardized teams to enable awake craniotomies. Objective: To assess the cost utility of introducing a standardized program of awake craniotomies. Design, Setting, and Participants: A retrospective economic evaluation was conducted at Mayo Clinic Florida. All patients with single, unilateral lesions who underwent elective awake craniotomies between January 2016 and December 2021 were considered eligible for inclusion. The economic perspective of the health care institution and a time horizon of 1 year were considered. Data were analyzed from October 2022 to May 2023. Exposure: Treatment with an awake craniotomy before standardization (2016-2018) compared with treatment with awake craniotomy after standardization (2018-2021). Main Outcomes and Measures: Patient demographics, perioperative, and postoperative outcomes, including length of stay, intensive care (ICU) admission, extent of resection, readmission rates, and 1-year mortality were compared between patients undergoing surgery before and after standardization. Direct medical costs were estimated from Medicare reimbursement rates for all billed procedures. A cost-utility analysis was performed considering differences in direct medical costs and in 1-year mortality within the periods before and after standardization of procedures. Uncertainty was explored in probability sensitivity analysis. Results: A total of 164 patients (mean [SD] age, 49.9 [15.7] years; 98 [60%] male patients) were included in the study. Of those, 56 underwent surgery before and 108 after implementation of procedure standardization. Procedure standardization was associated with reductions in length of stay from a mean (SD) of 3.34 (1.79) to 2.46 (1.61) days (difference, 0.88 days; 95% CI, 0.33-1.42 days; P = .002), length of stay in ICU from a mean (SD) of 1.32 (0.69) to 0.99 (0.90) nights (difference, 0.33 nights; 95% CI, 0.06-0.60 nights; P = .02), 30-day readmission rate from 14% (8 patients) in the prestandardization cohort to 5% (5 patients) (difference, 9%; 95% CI, 19.6%-0.3%; P = .03), while extent of resection and intraoperative complication rates were similar between both cohorts. The standardized protocol was associated with mean (SD) savings of $7088.80 ($12 389.50) and decreases in 1-year mortality (dominant intervention). This protocol was found to be cost saving in 75.5% of all simulations in probability sensitivity analysis. Conclusions and Relevance: In this economic evaluation of standardization of awake craniotomy, there was a generalized reduction in length of stay, ICU admission time, and direct medical costs with implementation of an optimized protocol. This was achieved without compromising patient outcomes and with similar extent of resection, complication rates, and reduced readmission rates.

Diffuse Cerebral Edema With Uncal Herniation Leading to Brain Death in Patient With Fulminant Susac syndrome: Rare Complication of Rare Disease.

Susac Syndrome was first described as an inflammatory microangiopathy of the brain and retina. Since then, multiple articles have been published in attempts to improve the understanding of this rare disease. Clinically Susac Syndrome is known to present with triad of encephalopathy, sensorineural hearing loss and branch of retinal artery occlusion (BRAO), along with characteristic "snowball" or "spoke" appearing white matter lesions of the corpus callosum. It has been characterized by vast heterogeneity in terms of its presenting symptoms, severity, and clinical course. Although subset of patients present with severe forms of Susac Syndrome and can develop prominent residual neurologic deficits, it has been reported to be mostly non-life-threatening and only few fatal cases have been described in the literature. Based on the available case reports with fatal outcome, mortality has been related to the systemic complications either during acute disease flare or during chronic-progressive phase. We describe a case of fulminant Susac Syndrome complicated by the sudden and rapid progression of diffuse cerebral edema leading to brain herniation and ultimate brain death, in order to increase awareness of this rare and catastrophic complication.

Safety and Feasibility of a Fast-Track Pathway for Neurosurgical Craniotomy Patients: Bypassing the Intensive Care Unit.

Objective: To describe the safety and feasibility of a fast-track pathway for neurosurgical craniotomy patients receiving care in a neurosciences progressive care unit (NPCU). Patients and Methods: Traditionally, most craniotomy patients are admitted to the neurosciences intensive care unit (NSICU) for postoperative follow-up. Decreased availability of NSICU beds during the coronavirus disease-2019 delta surge led our team to establish a de-novo NPCU to preserve capacity for patients requiring high level of care and would bypass routine NSICU admissions. Patients were selected a priori by treating neurosurgeons on the basis of the potential need for high-level ICU services. After operation, selected patients were transferred to the postoperative care unit, where suitability for NPCU transfer was reassessed with checklist-criteria. This process was continued after the delta surge. Results: From July 1, 2021 to September 30, 2022, 57 patients followed the NPCU protocol. Thirty-four (59.6%) were women, and the mean age was 56 years. Fifty-seven craniotomies for 34 intra-axial and 23 extra-axial lesions were performed. After assessment and application of the checklist-criteria, 55 (96.5%) were transferred to NPCU, and only 2 (3.5%) were transferred to ICU. All 55 patients followed in NPCU had good safety outcomes without requiring NSICU transfer. This saved $143,000 and led to 55 additional ICU beds for emergent admissions. Conclusion: This fast-track craniotomy protocol provides early experience that a surgeon-selected group of patients may be suitably monitored outside the traditional NSICU. This system has the potential to reduce overall health care expenses, increase capacity for NSICU bed availability, and change the paradigm of NSICU admission.

Multimodal monitoring: practical recommendations (dos and don'ts) in challenging situations and uncertainty.

With the advancements in modern medicine, new methods are being developed to monitor patients in the intensive care unit. Different modalities evaluate different aspects of the patient's physiology and clinical status. The complexity of these modalities often restricts their use to the realm of clinical research, thereby limiting their use in the real world. Understanding their salient features and their limitations can aid physicians in interpreting the concomitant information provided by multiple modalities to make informed decisions that may affect clinical care and outcomes. Here, we present a review of the commonly used methods in the neurological intensive care unit with practical recommendations for their use.

Balamuthia mandrillaris Encephalitis Presenting as a Symptomatic Focal Hypodensity in an Immunocompromised Patient.

We present the case of a 61-year-old woman with a history of orthotopic heart transplant who was hospitalized with new-onset headache. Magnetic resonance imaging (MRI) of the brain revealed T2 hyperintense signal involving the left occipital lobe with leptomeningeal enhancement and mild vasogenic edema. Initial neurologic examination was normal; however, after 7 days she developed imbalance, visual disturbances, night sweats, bradyphrenia, alexia without agraphia, and right hemianopsia. Brain MRI showed enlargement of the left occipital mass and worsening edema. Stereotactic needle biopsy showed nondiagnostic necrosis. The patient continued to deteriorate despite dexamethasone. Cerebrospinal fluid (CSF) suggested infection, and cytomegalovirus CSF polymerase chain reaction (PCR) was positive. The patient received vancomycin, imipenem, and ganciclovir. After obtaining a positive serum beta-D-glucan (Fungitell), amphotericin was added. Despite best medical efforts, the patient died. Postmortem broad-range PCR sequencing of the brain tissue was positive for rare amoeba Balamuthia mandrillaris.

Management of Poor-Grade Aneurysmal Subarachnoid Hemorrhage and Key Pearls for Achieving Favorable Outcomes: An Illustrative Case.

Poor-grade aneurysmal subarachnoid hemorrhage (aSAH) is associated with high patient mortality. Despite recent advances in management strategies, the prognosis for poor-grade aSAH remains dismal. We present a challenging case of a patient presenting with poor-grade aSAH. A 46-year-old female presented to the emergency department after losing consciousness following a sudden headache. The examination showed a dilated left pupil and a Glasgow Coma Scale of 4. Imaging revealed a ruptured anterior communicating artery (ACoM) aneurysm, after which the patient was subsequently taken to the neuro-interventional radiology suite. We showed that carefully managing blood pressure and intracranial pressure (ICP) makes it possible to achieve a favorable outcome and reduce the risk of secondary brain injury in aSAH, regardless of patient presentation. We propose maintaining blood pressure at <160 mmHg prior to intervention, after which it can be permitted to increase to 160-240 mmHg for the purpose of preventing vasospasm. Additionally, transcranial doppler (TCD) is essential to detect vasospasm due to the subtility of symptoms in patients with aSAH. Once identified, vasospasm can be successfully treated with balloon angioplasty. Finally, targeted temperature management (TTM), mannitol, hypertonic saline, and neuromuscular paralysis are essential for the postoperative management of ICP levels.

Intracranial Aneurysms in Loeys-Dietz Syndrome: A Multicenter Propensity-Matched Analysis.

BACKGROUND: Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by a classic triad of hypertelorism, bifid uvula and/or cleft palate, and generalized arterial tortuosity. There are limited data on the prevalence and rupture risk of intracranial aneurysms (IAs) in the setting of LDS, with no established guidelines. OBJECTIVE: To analyze the prevalence and rupture risk of IA in LDS. METHODS: Electronic medical records of patients with a confirmed diagnosis of LDS and available cerebrovascular imaging were reviewed. Patients were divided into 2 groups based on the presence of IA. Unmatched and propensity-matched analyses were used to identify potential risk factors for aneurysm formation. RESULTS: Records of 1111 patients were screened yielding a total of 60 patients with a diagnosis of LDS. Eighteen (30%) patients had IA, 4 (22.2%) of whom had multiple aneurysms for a total of 24 IAs. Twenty-three (95.8%) aneurysms were located in the anterior circulation; none of them were ruptured. On unmatched analysis, age ( P = .015), smoking history ( P = .034), hypertension ( P = .035), and number of extracranial aneurysms ( P < .001) were significantly higher in patients with IA. After matching for age, sex, race, stroke history, family history, and extracranial aneurysms, smoking history ( P = .009) remained significant. CONCLUSION: Patients with LDS have an increased risk of IAs, especially with a history of smoking. The prevalence rate of IAs in our series was 30%. Screening imaging should be considered at diagnosis, and patients should be encouraged to abstain from smoking. Further studies are needed to elucidate the risk of IA rupture and treatment considerations in this unique population.

Improving the Telemedicine Evaluation of Patients With Acute Vision Loss: A Call to Eyes.

Acute vision loss related to cerebral or retinal ischemia is a time-sensitive emergency with potential treatment options including IV or intra-arterial thrombolysis and mechanical thrombectomy. However, patients either present in a delayed fashion or present to an emergency department that lacks the subspecialty expertise to recognize and treat these conditions in a timely fashion. Moreover, health care systems in the United States are becoming increasingly reliant on telestroke and teleneurology services for acute neurologic care, making the accurate diagnosis of acute vision loss even more challenging due to critical limitations to the remote video evaluation, including the inability to perform routine ophthalmoscopy. The COVID-19 pandemic has led to a greater reliance on telemedicine services and helped to accelerate the development of novel tools and care pathways to improve remote ophthalmologic evaluation, but these tools have yet to be adapted for use in the remote evaluation of acute vision loss. Permanent vision loss can be disabling for patients, and efforts must be made to increase and improve early diagnosis and management. Herein, the authors outline the importance of improving acute ophthalmologic diagnosis, outline key limitations and barriers to the current video-based teleneurology assessments, highlight opportunities to leverage new tools to enhance the remote assessment of vision loss, and propose new avenues to improve access to emergent ophthalmology subspecialty.

Effect of Early vs Standard Approach to Tracheostomy on Functional Outcome at 6 Months Among Patients With Severe Stroke Receiving Mechanical Ventilation: The SETPOINT2 Randomized Clinical Trial.

Importance: Many patients with severe stroke have impaired airway protective reflexes, resulting in prolonged invasive mechanical ventilation. Objective: To test whether early vs standard tracheostomy improved functional outcome among patients with stroke receiving mechanical ventilation. Design, Setting, and Participants: In this randomized clinical trial, 382 patients with severe acute ischemic or hemorrhagic stroke receiving invasive ventilation were randomly assigned (1:1) to early tracheostomy (≤5 days of intubation) or ongoing ventilator weaning with standard tracheostomy if needed from day 10. Patients were randomized between July 28, 2015, and January 24, 2020, at 26 US and German neurocritical care centers. The final date of follow-up was August 9, 2020. Interventions: Patients were assigned to an early tracheostomy strategy (n = 188) or to a standard tracheostomy (control group) strategy (n = 194). Main Outcomes and Measures: The primary outcome was functional outcome at 6 months, based on the modified Rankin Scale score (range, 0 [best] to 6 [worst]) dichotomized to a score of 0 (no disability) to 4 (moderately severe disability) vs 5 (severe disability) or 6 (death). Results: Among 382 patients randomized (median age, 59 years; 49.8% women), 366 (95.8%) completed the trial with available follow-up data on the primary outcome (177 patients [94.1%] in the early group; 189 patients [97.4%] in the standard group). A tracheostomy (predominantly percutaneously) was performed in 95.2% of the early tracheostomy group in a median of 4 days after intubation (IQR, 3-4 days) and in 67% of the control group in a median of 11 days after intubation (IQR, 10-12 days). The proportion without severe disability (modified Rankin Scale score, 0-4) at 6 months was not significantly different in the early tracheostomy vs the control group (43.5% vs 47.1%; difference, -3.6% [95% CI, -14.3% to 7.2%]; adjusted odds ratio, 0.93 [95% CI, 0.60-1.42]; P = .73). Of the serious adverse events, 5.0% (6 of 121 reported events) in the early tracheostomy group vs 3.4% (4 of 118 reported events) were related to tracheostomy. Conclusions and Relevance: Among patients with severe stroke receiving mechanical ventilation, a strategy of early tracheostomy, compared with a standard approach to tracheostomy, did not significantly improve the rate of survival without severe disability at 6 months. However, the wide confidence intervals around the effect estimate may include a clinically important difference, so a clinically relevant benefit or harm from a strategy of early tracheostomy cannot be excluded. Trial Registration: ClinicalTrials.gov Identifier: NCT02377167.

Pro-resolving lipid mediators in traumatic brain injury: emerging concepts and translational approach.

Despite the high mortality and disability associated with traumatic brain injury (TBI), effective pharmacologic treatments are lacking. Of emerging interest, bioactive lipids, including specialized pro-resolving lipid mediators of inflammation (SPMs), act to attenuate inflammation after injury resolution. The SPM lipidome may serve as a biomarker of disease and predictor of clinical outcomes, and the use of exogenous SPM administration represents a novel therapeutic strategy for TBI. This review article provides a comprehensive discussion of the current pre-clinical and clinical literature supporting the importance of bioactive lipids, including SPMs, in TBI recovery. We additionally propose a translational approach to answer important clinical and scientific questions to advance the study of bioactive lipids and SPMs towards clinical research. Given the morbidity and mortality associated with TBI with limited treatment options, novel approaches are needed.

Pattern of changes in cross sectional area of arterial branches after jailing with pipeline embolization device: beyond parent neck artery patency.

OBJECTIVE: The Pipeline-Embolization-Device (PED) has been used increasingly for intracranial-aneurysms. Despite the high-patency-rate of jailed branches following PED deployment, little is known about changes in these vessels size. This study measured change in size after PED. METHODS: This retrospective-study screened a database of 183-consecutive-patients treated with PED (07/2011-07/2017) across inclusion criteria. We included patients in whom the ophthalmic artery (OA) and/or the posterior communicating artery (PComA) were jailed by the PED. MRA was used to calculate change in cross-sectional-area (CSA) of these vessels. 29 patients who had MRA before and after treatment were included in the study. The CSA was measured automatically using Syngo®.via-software at fixed points along the analyzed vessels. After exclusion of low-quality and software non-capturable MRA-images, 16 OA and 23 PComA were included in the final analysis. Statistical Package for Social Sciences was used for analysis. RESULTS: The mean CSA of PComA, P1-segement of posteriror-cerebral-artery (P1-PCA), and OA was 3.3 ± 1.3, 4.1 ± 1.2, and 3.2 ± 0.9 mm2 at baseline and 1.9 ± 1.4, 4.3 ± 1.2, and 3.1 ± 0.7 mm2 at follow-up, respectively. The average follow-up was approximately 26 months. While the decrease in CSA of PComA was statistically significant, the increase in P1-PCA CSA was not. The change in OA CSA was not statistically significant. CONCLUSION: Jailing PComA with a PED resulted in a statistically significant decrease in PComA CSA and a statistically non-significant increase in ipsilateral P1 CSA. No statistically significant change in the CSA of OA was noted. Changes might be due to a balance between flow demand through the jailed ostium and presence of collateral flow.

Comprehensive Perioperative Approach to Complex Spine Deformity Management.

OBJECTIVE: Study perioperative strategies for optimizing neuroprotection in complex spine deformity correction surgery. METHODS: We report the case of a patient with severe lumbar dextroscoliosis, thoracolumbar junction hyperkyphosis with a 40-degree Cobb angle levoconvex scoliosis who underwent spinal deformity correction with loss of neuromonitoring during surgery. We performed a literature review on perioperative management of complex spine deformity. RESULTS: A 50-year-old man presented with lumbar pain and right L4 radiculopathy. Surgical intervention for deformity correction and decompression was indicated with T4-L4 posterior instrumentation L2/L3 and L3/L4 transforaminal lumbar interbody fusion. Surgery was aborted due to the loss of neuromonitoring. Postsurgery, the patient had left sensory deficit and the neurocritical care team clinically suspected and deduced the anatomic location of the spinal cord compression. Magnetic resonance imaging confirmed a T10-T11 hyperintensity suggestive of cord ischemia due to osteophyte compressing the spinal cord. The patient underwent a second corrective surgery with no intraoperative events and has no long-term neurological sequela. CONCLUSIONS: This case illustrates that a comprehensive perioperative approach and individualized risk factor assessment is useful in complex spine deformity surgery. Further research is needed to determine how this individualized comprehensive approach can lead to intraoperative and postoperative countermeasures that improved spine surgery outcomes. LEVEL OF EVIDENCE: Level V.

Intracranial Aneurysms in Patients with Marfan Syndrome: A Multicenter Propensity-Matched Analysis.

OBJECTIVE: We sought to identify risk factors for intracranial aneurysms (IAs) in Marfan syndrome (MFS) patients and to describe their characteristics. METHODS: Patients with confirmed MFS and vessel-dedicated brain imaging from January 1, 1980-December 1, 2020 were categorized according to the presence (IA) or absence (NIA) of IAs. Unmatched logistic regression analysis and propensity score matching were used for comparison. RESULTS: We included 159 patients, of whom 18 (11.3%) patients had radiographic diagnosis of IAs. Three patients (16.7%) had multiple lesions for a total of 24 IAs. One patient (5.5%) had de novo IA formation. Four patients (22.2%) underwent treatment: Two (11.1%) had open surgical clipping for ruptured aneurysms, and two (11.1%) patients had endovascular embolization for growth. In the unmatched analysis, current tobacco smoker status (odds ratio [OR]: 4.20; confidence interval [CI]: 1.11-15.6; P = 0.027) and history of coronary artery disease (CAD) (OR: 5.79; CI 1.76-20.2; P = 0.004) increased the odds for IA. Propensity score matching yielded 18 IA and 18 NIA patients matched for age, gender, race, prior stroke, and family history of aneurysms. History of CAD (IA = 11 [61.1%] vs. NIA = 4 (22.2%), P = 0.043) and current smoker status (IA = 6 [33.3%] vs. NIA = 0 (0%), P < 0.01) were significantly higher in the IA cohort. Body mass index (P = 0.622), diabetes (P = 0.180), hypertension (P = 0.732), prior stroke (P = 1.00), family history (P = 0.732), alcohol (P = 0.314), recreational drugs (P = 1.00), and other aneurysms (P = 0.585) were not statistically significant. CONCLUSIONS: Prevalence of IAs in our series of MFS patients was 11.3%, and de novo formation was 5.5%. MFS patients with a history of CAD and current smoker status had an increased risk of IA. Neurovascular radiographic screening should be considered in all patients with MFS, particularly in patients who smoke or have a history of heart disease.