Incidental finding of aberrant left common carotid artery during intracerebral hemorrhage examination.

We report a case involving an aortic arch anomaly that has not been previously documented. The patients were a 76-year-old female who was urgently transported to the hospital because of a sudden disturbance of consciousness. Neurological symptoms indicated impending brain herniation, and the patient was diagnosed with hypertensive intracerebral hemorrhage (left putamen-thalamus) and acute hydrocephalus, thereafter she died approximately 6 hours after arrival. Head and neck-to-chest computed tomography angiography revealed an atypical aortic arch branching pattern. From the proximal side, the left common carotid artery, right common carotid artery, right subclavian artery, and left subclavian artery originated sequentially, suggesting an aberrant left common carotid artery. In the context of acute stroke management, understanding such aortic arch variations is crucial for planning access routes and treatment strategies for neuroendovascular procedures.

Central motor conduction time predicts new pyramidal MRI lesion and stroke-in-evolution in acute ischemic stroke.

Stroke is one of the leading causes of disability worldwide. Stroke-in-evolution is an essential issue as it is often associated with a worse outcome. Central motor conduction time (CMCT) is the time required for neural impulses to travel through the central nervous system to the target muscles. CMCT prolongation indicates dysfunction of the corticospinal tract. This study aims to investigate the impact of CMCT on clinical features and MRI characteristics in patients with acute ischemic stroke. A total of 94 patients with suspected acute ischemic stroke, with an average age of 67.13 ± 10.73 years old and 69.15 % being male, were enrolled in this study. All patients underwent evaluation for stroke risk factors, medical record review, CMCT examination (with CMCT (+) indicating CMCT prolongation), cranial MRI examinations, and data analysis. Compared to CMCT (-), the number of CMCT (+) subjects was significantly higher in all groups except the "Ever Stroke" group. The CMCT (+) group exhibited significantly higher values of "NIHSS" and "mRS" compared to the CMCT (-) group. After ANCOVA adjustment, the number of CMCT (+) subjects remained significantly higher only in the radiologically classified "New Pyramidal Lesion on MRI" and clinically classified "Stroke-In-Evolution" groups. In conclusion, CMCT serves as both a diagnostic indicator of acute ischemic stroke with weakness accompanied by new pyramidal lesions on brain MRI, rather than weakness associated with old lesions on brain MRI, and as a predictive marker for stroke progression during hospitalization.

Poor oral status at stroke onset negatively affects activities of daily living at discharge: A propensity-score analysis.

BACKGROUND & AIMS: Poor oral status during stroke recovery can cause malnutrition, which can markedly affect activities of daily living and prevent patients from being discharged home. Therefore, prompt evaluation and treatment of oral disorders immediately after stroke is essential. This study aimed to examine the impact of poor oral status on the post-stroke recovery of independence in activities of daily living and home discharge. METHODS: This single-center retrospective observational study included 137 patients with acute stroke, divided into two groups based on their Oral Assessment Guide (OAG) scores: the normal OAG group (score = 8) and the impaired OAG group (scores ≥9). Propensity-score matching was performed to minimize confounding variables. The χ2 test and odds ratios were used to compare the percentage of independence and home discharges between the two groups. RESULTS: The normal and impaired OAG groups exhibited no difference in neurological severity or nutritional intake after matching. The percentage of patients achieving independence in activities of daily living in the normal OAG group (86.8 %) was significantly higher than that in the impaired OAG group (65.8 %) (p = 0.03, odds ratio [OR] 0.29, 95 % confidence interval [CI] 0.09-0.92). Conversely, no significant difference in the percentage of patients discharged home was found (p = 0.15, OR 0.49, 95%CI 0.19-1.29). CONCLUSIONS: This study found that poor oral status after stroke onset was an independent factor affecting independence in activities of daily living at discharge, irrespective of neurological severity and dietary intake.

Life-threatening presentation of an acute cerebellar ischemic stroke secondary to a protein C deficiency.

Acute cerebellar ischemic stroke is a rare disease in children. Typically, patients present with ataxia and cranial nerve palsy. Rarely, some patients show a severe intracranial hypertension syndrome with a life-threatening clinical presentation. We report a case of a 2-year-old male child who was admitted for deterioration of his consciousness level and vomiting. Cerebral imaging revealed a right cerebellar and brainstem infarction with an obstructive hydrocephalus and a tonsillar herniation. Angiography identified an occlusion of the right anterior inferior cerebellar artery. The child was referred to a neurosurgeon for the treatment of acute hydrocephalus and posterior fossa craniectomy. Etiological investigations revealed a protein C deficiency. Initially, the patient was maintained under fluid restriction and received mannitol several times as he had clinical and radiological signs of intracranial hypertension. He was also kept on mechanical ventilation and monitored. However, the evolution was complicated by the occurrence of a thrombosis of the vena cava and the renal vein.

Association Between Blood-Brain Barrier Disruption and Stroke-Associated Pneumonia in Acute Ischemic Stroke Patients After Endovascular Therapy: A Retrospective Cohort Study.

Background: Stroke, particularly due to large vessel occlusion (LVO), is a major cause of mortality and disability globally. Endovascular therapy (ET) significantly improves outcomes for acute ischemic stroke (AIS) patients, but complications such as stroke-associated pneumonia (SAP) increase mortality and healthcare costs. This study investigates the association between blood-brain barrier (BBB) disruption and the increased risk of SAP and explores the relationship between BBB disruption and medium-term functional outcomes. Methods: The retrospective cohort study was performed on AIS patients enrolled between January 2019 to February 2023 who underwent ET. Patients were divided into two groups: BBB disruption and without BBB disruption. Multiple logistic regression model was conducted to measure the association between BBB disruption and SAP. Mediation analysis was used to estimate the potential mediation effects on the associations of BBB disruption with SAP. A restricted cubic spline (RCS) regression model was used to further outline the connection between the highest CT value of hyperattenuated lesions areas and the risk of SAP. Results: The study included 254 patients who underwent endovascular therapy, with 155 patients in the BBB disruption group (exposure) and 99 patients in the without BBB disruption group (control). Multiple logistic regression analysis revealed a significantly increased risk of SAP in patients with BBB disruption (OR = 2.337, 95% CI: 1.118-4.990, p = 0.025). Furthermore, mediation analysis suggested that this association may be partly due to malignant cerebral oedema and haemorrhagic transformation. The study found an inverse L-shaped dose-response relationship between the maximum CT values of BBB disruption areas and the incidence of SAP. SAP partially mediated the association between BBB disruption and 3-month poor functional outcome. Conclusion: BBB disruption are a potential risk factor for SAP. BBB disruption may affect short- and medium-term prognosis of patients after ET in part through SAP.

National Assessment of Clinicians Participating in Stroke Treatment Decisions at StrokeNet Regional Coordinating Centers: Clinicians Making Stroke Treatment Decisions.

OBJECTIVES: Since the introduction of thrombolytics, stroke teams evolved to improve thrombolytic care delivery. The impact of the advent of endovascular therapy on the composition of acute stroke teams is unknown. MATERIALS AND METHODS: A two-part pilot-tested survey was deployed to site-Principal Investigators (PIs) of the 27 StrokeNet Regional Coordinating Centers (RCCs) regarding institutional acute stroke teams. Part A inquired about the participation of personnel in each type of stroke response. Part B identified stroke team physicians and the types of responses in which they participate to assess training background of stroke team members. RESULTS: Response rates for Part A and B were 66% and 48%, respectively. In Part A, 67% (12/18) of sites reported trainees always responded to ED stroke activations with significant autonomy. 44% (7/16) and 27% (4/15) of sites reported NP and PA response to ED stroke alerts, respectively, but with limited autonomy. In Part B, 124 physicians involved in ED stroke alerts were identified, the large majority of whom 79% (95% CI: 71-85) were vascular neurology trained. The 39 (23%) stroke team members involved in endovascular therapy had the following training: 49% (34-64) neurosurgery, 28% (17-44) radiology, 18% (9-33) vascular neurology 5% (1-16) neurology. CONCLUSIONS: We identified modest heterogeneity in the composition of acute stroke team members across StrokeNet RCCs. Individuals performing endovascular therapy had a variety of clinical specializations, reflecting the evolving multidisciplinary nature of interventional acute stroke care. At StrokeNet RCCs, teams have significant trainee involvement in both ED and inpatient acute stroke responses.

Prefrontal Cortex Oxygenation During Walking in an Acute Ischaemic Stroke Patient with Severe Internal Carotid Artery Stenosis: A Case Report.

This study investigated the oxygenation in the prefrontal cortex (PFC) during walking in a 75-year-old male patient in the acute phase of a left frontal lobe cerebral infarction complicated by severe left internal carotid artery stenosis. The patient regained independent ambulation on the fifth day after the onset of symptoms, and the study was conducted on the eighth day after the onset of symptoms. The patient rested for 10 s before walking for 70 s. Then he rested for 60 s. The levels of oxyhaemoglobin (O2Hb), deoxyhaemoglobin (HHb), and total haemoglobin (THb) in the PFC were quantified during the rest and walking phases using a wearable near-infrared spectroscopy device. Comparative analysis with baseline values during rest revealed decreases in the O2Hb, HHb, and THb in both hemispheres of the PFC during walking (O2Hb: -19.76/ -14.88 µmol/L, HHb: -1.18/ -2.00 µmol/L, THb: -20.96/ -16.88 µmol/L, right/left, respectively). The recovery of the O2Hb was delayed by 15.8 seconds in the affected hemisphere. These findings support the effectiveness of wearable NIRS for the evaluation of cerebral oxygenation during rehabilitation in patients with acute stroke to facilitate evaluations and individualise patient care.

Anesthetic Management of Acute Ischemic Stroke Undergoing Mechanical Thrombectomy: An Overview.

Ischemic stroke, caused by the interruption of the blood supply to the brain, requires prompt medical intervention to prevent irreversible damage. Anesthetic management is pivotal during surgical treatments like mechanical thrombectomy, where precise strategies ensure patient safety and procedural success. This narrative review highlights key aspects of anesthetic management in ischemic stroke, focusing on preoperative evaluation, anesthetic choices, and intraoperative care. A rapid yet thorough preoperative assessment is crucial, prioritizing essential diagnostic tests and cardiovascular evaluations to determine patient frailty and potential complications. The decision between general anesthesia (GA) and conscious sedation (CS) remains debated, with GA offering better procedural conditions and CS enabling continuous neurological assessment. The selection of anesthetic agents-such as propofol, sevoflurane, midazolam, fentanyl, remifentanil, and dexmedetomidine-depends on local protocols and expertise balancing neuroprotection, hemodynamic stability, and rapid postoperative recovery. Effective blood pressure management, tailored airway strategies, and vigilant postoperative monitoring are essential to optimize outcomes. This review underscores the importance of coordinated care, incorporating multimodal monitoring and maintaining neuroprotection throughout the perioperative period.

VISIT STROKE: non-inferiority of telemedicine-based neurological consultation for post-acute stroke patients - protocol of a prospective observational controlled multi-center study.

BACKGROUND: Telemedicine provides specialized medical expertise in underserved areas where neurological expertise is frequently not available on a daily basis for hospitalized stroke patients. While tele-consultations are well established in acute stroke assessment, the value of telemedicine-based ward-rounds in the subsequent in-patient stroke management is unknown. METHODS: Four telemedicine stroke networks in Germany, implemented in eight out of 16 federal states, participate in this prospective observational multi-center study. We plan to enroll 523 patients hospitalized due to acute (suspected or confirmed) stroke or transient ischemic attack. Each recruited patient will receive both a tele-consultation and an on-site consultation at the same day within the first three days after hospital admission. We will test non-inferiority of telemedicine-based assessments in ward-rounds in terms of quality of medical assessment and recommendations for hospitalized stroke patients. The correctness of the medical assessment and recommendation is defined as positive evaluation (binary, correct vs. in-correct) of six out of six predefined quality indicators by at least two out of three blinded independent raters. The non-inferiority margin for the difference in proportions of correct assessments is set to 5%-points. DISCUSSION: If non-inferiority of telemedicine-based ward-rounds compared to on-site ward-rounds by a neurologist were demonstrated, telemedicine-based neurological consultation for post-acute stroke patients may contribute to deliver evidence-based high-quality stroke care more easily in underserved regions. TRIAL REGISTRATION: DRKS - DRKS00028671 ( https://drks.de/search/de/trial/DRKS00028671 ; registration date 09-27-2022).

Streamlining Acute Stroke Processes and Data Collection: A Narrative Review.

(1) Background: Acute ischemic stroke treatment has been thoroughly studied to identify strategies to reduce treatment times. However, many centers still struggle to achieve fast treatment times. Additionally, studies primarily focus on larger, more advanced centers; yet, smaller centers often face longer treatment times. (2) Objectives: The aim of this study is to analyze the existing literature reviewing stroke treatment processes in primary and comprehensive stroke centers that investigated or reduced treatment times. The articles identified were categorized based on the focus areas and approaches used. (3) Results: Three main categories of improvements were identified in the literature: (1) standardization of processes, (2) resource management, and (3) data collection. Both primary and comprehensive stroke centers were able to reduce treatment times through standardization of the processes. However, challenges such as variations in hospital resources and difficulties incorporating data collection software into workflow were highlighted. Additionally, many strategies to optimize resources and data collection that can benefit primary stroke centers were only conducted in comprehensive stroke centers. (4) Conclusions: Many existing strategies to improve stroke treatment times, such as pre-notification and mass stroke team alerts, have been implemented in both primary and comprehensive stroke centers. However, tools such as simulation training are understudied in primary stroke centers and should be analyzed. Additionally, while data collection and feedback are recognized as crucial for process improvement, challenges persist in integrating consistent data collection methods into clinical workflow. Further development of easy-to-use software tailored to clinician needs can help improve stroke center capabilities to provide feedback and improve treatment processes.

Disparities in Emergency Medical Services Use, Prehospital Notification, and Symptom Onset to Arrival in Patients With Acute Stroke.

BACKGROUND: Disparities in time to hospital presentation and prehospital stroke care may be important drivers in inequities in acute stroke treatment rates, functional outcomes, and mortality. It is unknown how patient-level factors, such as race and ethnicity and county-level socioeconomic status, affect these aspects of prehospital stroke care. METHODS: Cross-sectional study of patients with ischemic stroke, intracerebral hemorrhage, and subarachnoid hemorrhage in the Get With the Guidelines-Stroke registry, presenting from July 2015 to December 2019, with symptom onset <24 hours. Multivariable logistic regression and quantile regression were used to investigate the outcomes of interest: emergency medical services (EMS) transport (versus private vehicle), EMS prehospital notification (versus no prehospital notification), and stroke symptom onset to time of arrival at the emergency department. Prespecified covariates included patient-level, hospital-level, and county-level characteristics. RESULTS: The inclusion criteria was met by the 606 369 patients. Of the patients, 51.2% were men and 69.9% White, with a median National Institutes of Health Stroke Severity of 4 (IQR, 2-10), and median social deprivation index (SDI) of 51 (IQR, 27-75). Median symptom onset to arrival time was 176 minutes (IQR, 64-565). Black race was significantly associated with prolonged symptom onset to emergency department arrival time (+28.21 minutes [95% CI, 25.59-30.84]), and decreased odds of EMS prehospital notification (OR, 0.80 [95% CI, 0.78-0.82]). SDI was not associated with differences in EMS use but was associated with lower odds of EMS prehospital notification (upper SDI tercile versus lowest, OR, 0.79 [95% CI, 0.78-0.81]). SDI was also significantly associated with stroke symptom onset to emergency department arrival time (upper SDI tercile versus lowest +2.56 minutes [95% CI, 0.58-4.53]). CONCLUSIONS: In this national cross-sectional study, Black race was associated with prolonged onset to time of arrival intervals and significantly decreased odds of EMS prehospital notification, despite similar use of EMS transport. Greater county-level deprivation was also associated with reduced odds of EMS prehospital notification and slightly prolonged stroke symptom onset to emergency department arrival time. Efforts to reduce place-based disparities in stroke care must address significant inequities in prehospital care of acute stroke and continue to address health inequities associated with race and ethnicity.

Effectiveness of the Stroke e-Learning Module on Malaysian Doctors' Knowledge of Acute Ischaemic Stroke Management.

Background: The Stroke e-Learning Module (SEM) is a nationwide initiative to improve stroke care in Malaysia. This study aimed to assess the module's effectiveness in improving knowledge on acute ischaemic stroke (AIS) management among Malaysian doctors. Methods: This was a pre-post study design. Medical officers, specialists and general practitioners from various disciplines who work in healthcare facilities in Malaysia were recruited virtually from those who registered for the SEM on the Docquity platform between 1 February 2021 and 31 January 2022. The Acute Stroke Management Questionnaire (ASMaQ), an existing validated questionnaire, was used to measure the doctors' knowledge of AIS management before and after the SEM. The ASMaQ had three domains: i) general stroke knowledge (GSK), ii) hyperacute stroke management (HSM) and iii) advanced stroke management (ASM). The paired t- and the McNemar-tests were used to evaluate the effectiveness of the module. Results: One hundred and seventy-one participants voluntarily responded to the pre- and post-module questionnaires. The paired t-test revealed statistically significant improvement for the ASM knowledge scores (mean difference = 2.5; 95% CI: 1.8, 3.2; P < 0.001). The baseline proportion of participants with good knowledge of GSK, HSM and ASM were 92.4%, 64.9%, and 76%, respectively. The McNemar test showed that approximately 14% of the participants had significant improvement in ASM knowledge (P < 0.001). However, no significant changes were noted for GSK (-0.6%) and HSM (4.1%). Conclusion: The SEM has been shown to increase Malaysian doctors' knowledge on ASM. However, greater effort should be made to improve GSK and HSM knowledge, particularly in areas related to stroke thrombolysis.

Trial selection criteria should not be used for clinical decisions and recommendations: the thrombectomy trials example.

BACKGROUND: Despite multiple calls for more inclusive studies, most clinical trial eligibility criteria remain too restrictive. Thrombectomy trials have been no exception. METHODS: We review the landmark trials that have shown the benefits of thrombectomy, their eligibility criteria, and consequences on clinical practice. We discuss the rationale behind various reasons for exclusions. We also examine the logical problem involved in using eligibility criteria as indications for treatment. RESULTS: Most thrombectomy trials have been too restrictive. This has been shown by a plethora of follow-up studies that have refuted most of the previously recommended trial eligibility restrictions. Meanwhile, the effect of clinical recommendations based on restrictive eligibility criteria is that treatment has been denied to the majority of patients who could have benefitted. Trial eligibility criteria cannot be used to make clinical decisions or recommendations unless, like any other medical diagnosis, they have been shown capable of reliably differentiating patients into those that will, and those that will not benefit from treatment. This goal can only be achieved with all-inclusive pragmatic trials. CONCLUSION: Restrictive eligibility criteria render clinical trials incapable of guiding medical decisions or recommendations.

Endovascular Therapy of Ruptured Aneurysms on Moyamoya Collateral Vessels: Two Cases.

Background: Using two case reports of adult women with moyamoya disease presenting with intracranial hemorrhage from ruptured aneurysms on moyamoya collateral vessels, we aim to demonstrate the potential for effective endovascular treatment navigated by CT angiography, digital subtraction angiography, and flat panel CT. Case 1 Presentation: A 45-year-old female patient with sudden onset of headache, followed by somnolency. CT scan showed a four-ventricle hematocephalus caused by a 27 × 31 × 17 mm hematoma located in the left basal ganglia. Angiography revealed a 3 mm aneurysm on hypertrophic lenticulostriate artery bridging the M1 occlusion. Selective catheterization and distal embolisation with acrylic glue was done. Case 2 Presentation: A 47-year-old woman was admitted for a sudden onset of severe headache, CT scan showed four-ventricle hematocephalus. A 4 mm aneurysm on the collateral vessel-anterior chorioidal artery bridging the closure of the terminal segment of the internal carotid artery was diagnosed as the source of bleeding. Selective catheterization and distal embolisation with acrylic glue was done. Conclusions: Selective embolisation of ruptured aneurysms on moya moya collaterals is a simple, effective, and safe procedure when relevant microcatheters are used with imaging software navigation such as 3D DSA, 3D road map and flat-panel CT.

Medical Management of Acute Stroke based on Japan Stroke Society Guidelines and the Japan Stroke Data Bank.

Stroke is a leading cause of death and disability in Japan, necessitating standardized treatment guidelines. The Japan Stroke Society (JSS) periodically revises its guidelines to incorporate new research. This review provides a short overview of acute stroke management based on JSS Guideline 2021 (revised 2023) and the Japan Stroke Data Bank (JSDB), and discusses future directions in stroke management. Acute stroke management emphasizes systemic support and complication management. Risk factor control during acute hospitalization is also crucial for preventing recurrent strokes in the chronic phase.In ischemic stroke, super-acute recanalization therapies, including intravenous thrombolysis and mechanical thrombectomy, are the most important and effective. Antiplatelet therapy, particularly aspirin and clopidogrel, is recommended for noncardiogenic stroke and high-risk transient ischemic attack. In cardioembolic stroke, early initiation of direct oral anticoagulants might be considered according to stroke severity.For brain hemorrhage, early blood pressure management is recommended. Specific reversal agents are advised for patients on anticoagulant therapy. Minimally invasive hematoma removal may improve outcomes for intracerebral hemorrhage.Subarachnoid hemorrhage treatments reported from Japan include intravenous drugs to prevent vasospasm.The JSDB revealed improvements in functional outcomes in patients with ischemic stroke over the past 20 years, although patients with hemorrhagic stroke showed no clear improvement. The evolving guidelines and research underscore the importance of stratified and timely intervention in stroke care.

Hospital overcrowding and care of stroke patients: Irish national audit of stroke.

INTRODUCTION: Hospital overcrowding where patient admissions exceed capacity is associated with worse outcomes in Emergency Department. Developments in emergency stroke care have been associated with improvements in stroke outcome but are dependent on effective, organised care. We examined if overcrowding in the hospital system was associated with negative changes in stroke outcome. METHODS: Data on overcrowding were obtained from the Irish Nurses and Midwives Organisation (INMO) 'Trolley Count' database recording the number of patients cared for on trolleys/chairs in all acute hospitals each midnight. These were compared with quarterly data from the Irish National Audit of Stroke from 2013 to 2021 inclusive. Variables analysed were inpatient mortality rate, thrombolysis rate for ischaemic stroke, median door to needle time and median length of stay. RESULTS: 579449 patient episodes were recorded by Trolley Watch over the period, (Quarterly Median 16719.5, range 3389-27015). Average Quarterly Thrombolysis rate was 11.3% (sd 1.3%) Median Quarterly Inpatient Mortality rate was 11.8% (Range 8.9-14.0%). Median Quarterly Length of stay was 9 days (8-11 days). Median quarterly door to needle was 65 min (45-80 min). Q1 was typically the worst for overcrowding with on average 19777 incidences (sd 4786). This was significantly higher than for Q2 (mean 13540 (sd 4785) p = 0.005 t-test) and for Q3 (mean 14542 (sd 4753) p = 0.03). No significant correlation was found between quarterly Trolley watch episodes and inpatient mortality (r = 0.084, p = 0.63), median length of stay r=-0.15, p = 0.37) or thrombolysis rate (r = 0.089 p = 0.61). There was an unexpected significant negative correlation between trolley watch data and median door to needle time (r=-0.36, p = 0.03). CONCLUSION: Despite increasing hospital overcrowding, stroke services still managed to preserve standard of care. We could find no association between levels of overcrowding and deterioration in selected indices of patient care.

Some minutes matter more: Groin-to-recanalization is the main time-related predictor of outcome in acute ischemic stroke.

INTRODUCTION: Endovascular thrombectomy (EVT) is the standard of care for selected patients with acute ischemic stroke (AIS) and large vessel occlusion (LVO), associated with intravenous thrombolysis, when indicated. While many studies focused on pre-hospital and in-hospital pathways, only few analyzed the relationship between groin-to-recanalization (GTR) time and functional outcome. AIM: To explore whether GTR time is an independent predictor of outcome in patients undergoing EVT. METHODS: All patients with anterior circulation stroke treated with EVT at a high-volume center from January 2021 to December 2023 were included. The cohort was divided into two groups according to GTR time shorter or longer than 30 min. Regression analysis assessed the association between GTR time and 3-month good outcome, defined as modified Rankin Scale 0-2. RESULTS: The study included 419 patients. The groups had similar baseline characteristics and similar onset to recanalization (OTR) time. Regression analysis showed shorter GTR time is an independent predictor of favorable outcome (OR 2.49 [95% CI 1.26-4.94]). Age, baseline NIHSS, ASPECT score and bridging IVT were also found to be independently associated with outcome. DISCUSSION AND CONCLUSIONS: Our study showed GTR time is an independent predictor of good outcome in patients undergoing EVT with similar OTR time, emphasizing procedural time as a key prognostic factor, even greater than other well-known pre-hospital and in-hospital time-dependent variables. These findings may raise the issue of developing alternative approaches or early "rescue" strategies for complicated procedures.

Clinical features, hemorrhage risk and epilepsy outcomes of familial cerebral cavernous malformation: A 20-year observational pragmatic single-center study.

INTRODUCTION: Familial Cerebral Cavernous Malformations (fCCMs) are rare, hereditary conditions characterized by multiple central nervous system lesions. Despite their rarity, CCMs can cause significant clinical challenges when symptomatic, manifesting as seizure and symptomatic hemorrhage (CASH). Guidelines suggest neurosurgical intervention for symptomatic or previously symptomatic lesions, while conservative management is recommended for new-onset epilepsy. However, the natural history and optimal management remain unclear, necessitating further research. OBJECTIVE: This study aims to provide a comprehensive analysis of the clinical features, hemorrhage risk, and epilepsy outcomes in fCCM patients over an extended follow-up period, offering a more precise estimate of CASH and epilepsy rates in this population. METHODS: This retrospective longitudinal cohort study included fCCM patients enrolled from 2001 to May 2024. Data collected included demographic information, new neurological symptoms, symptomatic hemorrhages, seizures, and modified Rankin Scale (mRS) scores. Incidence rates of first symptomatic events and Kaplan-Meier survival curves were calculated, with logistic and Cox-proportional hazard regression models used to evaluate outcomes. RESULTS: A total of 47 patients were included in this study, with a mean age at diagnosis of 37.51 years. At diagnosis, 68 % were symptomatic, with 30 % having CASH and 36 % experiencing seizures without CASH. During a median follow-up of 126.0 months (interquartile range, 110.5 months), 17 % had a new CASH event, 20 % had seizures without CASH, and 60 % remained asymptomatic. The bleeding rate was 1.02 % per patient-year, with new focal neurological symptoms at 2.045 per 1000 patient-years and new CASH at 10.225 per 1000 patient-years. Most patients maintained minimal or no disability (mRS 0 or 1). Presenting with epilepsy at baseline significantly increased the odds of future seizures (OR 18.13, p = 0.001). CONCLUSION: This study highlights the complex presentation and progression of fCCMs, emphasizing the necessity for long-term monitoring. Baseline epilepsy is a significant predictor of future seizures, underscoring the need for individualized management strategies. Future research with larger cohorts and standardized criteria is essential to refine the understanding and management of fCCMs.