Contemporary Statistics of Acute Ischemic Stroke and Transient Ischemic Attack in 2021: Insights From the CRCS-K-NIH Registry.

This report presents the latest statistics on the stroke population in South Korea, sourced from the Clinical Research Collaborations for Stroke in Korea-National Institute for Health (CRCS-K-NIH), a comprehensive, nationwide, multicenter stroke registry. The Korean cohort, unlike western populations, shows a male-to-female ratio of 1.5, attributed to lower risk factors in Korean women. The average ages for men and women are 67 and 73 years, respectively. Hypertension is the most common risk factor (67%), consistent with global trends, but there is a higher prevalence of diabetes (35%) and smoking (21%). The prevalence of atrial fibrillation (19%) is lower than in western populations, suggesting effective prevention strategies in the general population. A high incidence of large artery atherosclerosis (38%) is observed, likely due to prevalent intracranial arterial disease in East Asians and advanced imaging techniques. There has been a decrease in intravenous thrombolysis rates, from 12% in 2017-2019 to 10% in 2021, with no improvements in door-to-needle and door-to-puncture times, worsened by the coronavirus disease 2019 pandemic. While the use of aspirin plus clopidogrel for non-cardioembolic stroke and direct oral anticoagulants for atrial fibrillation is well-established, the application of direct oral anticoagulants for non-atrial fibrillation cardioembolic strokes in the acute phase requires further research. The incidence of early neurological deterioration (13%) and the cumulative incidence of recurrent stroke at 3 months (3%) align with global figures. Favorable outcomes at 3 months (63%) are comparable internationally, yet the lack of improvement in dependency at 3 months highlights the need for advancements in acute stroke care.

An unusual case of carotidynia preceding recurrent ischemic stroke with carotid thrombosis in a patient with systemic lupus erythematosus.

Carotid artery thrombosis following carotidynia is an uncommon manifestation of systemic lupus erythematosus. We report the case of a woman without evidence of a lupus flare-up who presented with the unusual clinical course of ipsilateral carotidynia and recurrent ischemic stroke due to carotid thrombosis. To our knowledge, this is the first case of such an unusual manifestation in lupus and highlights distinctive challenges in the diagnosis and management of carotid artery thrombosis following carotidynia.

Effect of Transport Time on the Use of Reperfusion Therapy for Patients with Acute Ischemic Stroke in Korea.

BACKGROUND: We investigated the association between geographic proximity to hospitals and the administration rate of reperfusion therapy for acute ischemic stroke. METHODS: We identified patients with acute ischemic stroke who visited the hospital within 12 hours of symptom onset from a prospective nationwide multicenter stroke registry. Reperfusion therapy was classified as intravenous thrombolysis (IVT), endovascular therapy (EVT), or combined therapy. The association between the proportion of patients who were treated with reperfusion therapy and the ground transport time was evaluated using a spline regression analysis adjusted for patient-level characteristics. We also estimated the proportion of Korean population that lived within each 30-minute incremental service area from 67 stroke centers accredited by the Korean Stroke Society. RESULTS: Of 12,172 patients (mean age, 68 ± 13 years; men, 59.7%) who met the eligibility criteria, 96.5% lived within 90 minutes of ground transport time from the admitting hospital. The proportion of patients treated with IVT decreased significantly when stroke patients lived beyond 90 minutes of the transport time (P = 0.006). The proportion treated with EVT also showed a similar trend with the transport time. Based on the residential area, 98.4% of Korean population was accessible to 67 stroke centers within 90 minutes. CONCLUSION: The use of reperfusion therapy for acute stroke decreased when patients lived beyond 90 minutes of the ground transport time from the hospital. More than 95% of the South Korean population was accessible to 67 stroke centers within 90 minutes of the ground transport time.

B-cell-activating factor is elevated in serum of patients with myasthenia gravis.

INTRODUCTION: Myasthenia gravis (MG) is a B-cell-mediated autoimmune disease. B-cell-activating factor (BAFF) is a major factor in B-cell development and activation. In this study we investigated serum BAFF levels in MG patients. METHODS: We compared the serum BAFF levels of 20 MG patients with gender-matched healthy controls. We assayed serum concentrations of BAFF and anti-acetylcholine receptor antibody (AChR) titers. RESULTS: Serum BAFF levels of MG patients with AChR antibodies were significantly higher than those of healthy controls. A significant positive correlation was observed between serum BAFF levels and anti-AChR antibody titers. BAFF values did not correlate with disease severity. CONCLUSIONS: BAFF may play a major role in the pathogenesis of MG, and it may provide a potential target for therapy in patients with MG. Muscle Nerve 54: 1030-1033, 2016.

A Case of Severe Delayed Vasospasm after Clipping Surgery for an Unruptured Intracranial Aneurysm.

Delayed ischemic stroke associated with intractable vasospasm after clipping of unruptured intracranial aneurysms (UIAs) has been rarely reported. We report a patient with delayed ischemic stroke associated with intractable vasospasm following UIA clipping. A middle-aged female underwent surgery for unruptured middle cerebral artery bifurcation aneurysms. The patient tolerated the neurosurgical procedure well. Seven days postoperatively, the headache was unbearable; a postcraniotomy headache persisted and abruptly presented with global aphasia and right-sided hemiplegia after a nap. Emergency digital subtraction angiography showed severe luminal narrowing with segmental vasoconstriction, consistent with severe vasospasm. The patient's neurological deficit improved after chemical angioplasty. Neurosurgeons should pay close attention to this treatable/preventive entity after neurological deterioration following UIA clipping, even in patients without subarachnoid hemorrhage.

Curve-centered plaques raise the risk of peri-operative neurological and cardiovascular complications during angioplasty and stenting for severe carotid stenosis.

Introduction: Carotid artery stenting is an alternative interventional treatment to carotid endarterectomy. However, preprocedural considerations and anatomical risk factor analyses for carotid artery stenting are currently insufficient. Therefore, we investigated the high-risk anatomical appearance of carotid artery stenting from the neurointerventionist perspective to predict periprocedural complications. Materials and methods: We retrospectively reviewed patients with carotid stenosis who underwent carotid artery stenting at a comprehensive stroke center between January 2012 and December 2021. We compared the demographic characteristics, medical history, and anatomical appearance of the stenotic segment in patients with and without complications. Results: We analyzed a total of 148 patients (64 women [43.2%]; median age, 73.0 [interquartile range, 65.5-79.0]). Complications occurred in 39 of the 148 patients, primarily minor and transient. Of baseline or procedural characteristics, a high initial National Institutes of Health Stroke Scale score (p = 0.04), symptomatic stenosis (p = 0.01), and curve-centered plaque of the proximal ICA (p = 0.01) were significantly associated with carotid artery stenting complications in unadjusted analysis. Curve-centered plaque remained an independent risk factor for carotid artery stenting complications after adjustment (odds ratio 2.23[1.02-4.88], p = 0.04). Conclusion: High-risk vascular anatomical features, such as curve-centered plaque, are associated with a high frequency of periprocedural complications of carotid artery stenting. Tailored patient selection for carotid stenosis is crucial to prevent complications. Patients with curve-centered plaque should consider alternative treatment options such as carotid endarterectomy to achieve optimal clinical results.

Mechanical Thrombectomy for Hyperacute Vertebrobasilar Ischemic Stroke via Nondominant Vertebral Artery : Clinical Experience and Considerations.

Objective: The dominant vertebral artery (VA) approach is primarily considered in mechanical thrombectomy (MT) for acute occlusion of the vertebrobasilar (VB) artery. As accessing the dominant artery is sometimes difficult, we present our experience treating acute VB stroke via a nondominant VA approach through a comparison with the dominant VA approach. Methods: Among 2,785 patients diagnosed with hyperacute ischemic stroke between January 2014 and December 2022, 50 patients with VB ischemic stroke underwent recanalization therapy through either dominant, nondominant, or bilateral VA approach. We evaluated patient characteristics and clinical course, highlighting the pros and cons of the access routes. Results: The patients with hyperacute VB ischemic stroke were predominantly male (72%), with a mean age of 68.12 years and an initial National Institutes of Health Stroke Scale (NIHSS) score mean of 17.1. Large-artery atherosclerosis (LAA, 48%) and cardio-embolism (CE, 36%) were the main etiologic factors in the TOAST (Trial of Org 10172 in Acute Stroke Treatment) classification. After thrombectomy, 45 (90%) cases had final modified thrombolysis in cerebral infarction (mTICI) score of 2b or higher. In summary, 41 patients were treated through the dominant VA, and 8 patients underwent the nondominant VA approach. However, there was no statistically significant difference in functional outcome or mortality between the two approaches. Conclusion: In MT for VB occlusions, nondominant VA approach may be an option in situations when the vessel is accessible, stable, or less risky, as recanalization can be achieved without rescue balloon angioplasty and/or stenting.

Prevalence of shoulder pain and its correlates in Jeju agricultural population: A retrospective cross-sectional study.

The aim of this study was to investigate the prevalence of shoulder pain and analyze its association with socio-demographic factors, agricultural work-related conditions, and biomechanical factors among farmers on Jeju Island. We utilized initial survey data from the Safety for Agricultural Injury of Farmers' cohort study involving adult farmers on Jeju Island. The presence and characteristics of shoulder pain, socio-demographics, agricultural work-related conditions, and biomechanical factors were assessed using semi-structured questionnaires. A total of 1206 participants were analyzed for prevalence and associated risk factors using frequency and multivariate logistic regression analyses. The overall prevalence of shoulder pain was 17%. Multivariate logistic regression analysis, adjusted for significant socio-demographic and agricultural work-related condition variables, revealed that stress level, type of farming, and experiencing an agricultural injury within the preceding year were significantly associated with shoulder pain (stress level: "occasional," adjusted odds ratio [aOR], 1.581; 95% confidence interval [CI], 1.079-2.318; "frequent," aOR, 1.964; 95% CI, 1.205-3.200; and "extreme," aOR, 2.999; 95% CI, 1.480-6.074 vs "rarely"; type of farming: "orchard," aOR, 0.82; 95% CI, 0.597-1.124; "livestock," aOR, 0.225; 95% CI, 0.079-0.641 vs "field"; and experienced an agricultural injury within the past year: "yes," aOR, 2.078; 95% CI, 1.269-3.405). In addition, 3 biomechanical factors significantly contributed to shoulder pain: activities requiring significant power such as shoveling, pickaxing, and hammering; repetitive use of a particular body part; and constant elevation of the arm above the head. The findings highlight the importance of postural education, injury prevention, and psychological support in managing shoulder pain among farmers.

Characteristics and Trends of Pediatric Trauma in Jeju Island, South Korea: A Community Level Serial Cross-sectional study.

Objective: This study aimed to investigate the characteristics and epidemiological trends of pediatric injuries among patients visiting emergency departments (EDs) on Jeju Island. Method: Employing a community-level serial cross-sectional analysis, we targeted pediatric patients aged 18 years or younger who visited EDs for injuries over a ten-year period. The data, sourced from the Jeju Injury Surveillance System (JISS), facilitated a comprehensive examination of injury characteristics and epidemiological trends. This included an evaluation of the annual incidence and overall trends in pediatric injury cases. Results: The study found toddlers (42.5% of cases) to be the most frequently injured age group. Males were more prone to injuries, with a male-to-female ratio of 1.7:1. Injuries among visitors accounted for 17.3% of cases, with a seasonal spike in Summer, evenings and weekends. Most incidents occurred at home, predominantly accidental in nature, with adolescents more likely to require EMS services. The common mechanisms of injuries were blunt force (49.2%), slips/falls (22.0%), and motor vehicle collisions (13.2%), leading to bruises, cuts, and sprains. Over the decade, a general increase in pediatric injuries was observed. Accidental injuries initially surged but later stabilized, while self-harm/suicide and assault/violence injuries showed a concerning upward trend. Age-specific analysis revealed increasing trends in infants and adolescents. Conclusion: The study underscores the crucial need for targeted injury prevention and resource allocation strategies, particularly for high-risk groups and times, to effectively mitigate pediatric trauma on Jeju Island.

Uncooperative patients suspected of acute stroke ineligible for prehospital stroke screening test by emergency medical service providers: final hospital diagnoses and characteristics.

OBJECTIVE: This study investigated the hospital diagnoses and characteristics of uncooperative prehospital patients suspected of acute stroke who could not undergo a prehospital stroke screening test (PHSST). METHODS: This retrospective observational study was conducted at a single academic hospital with a regional stroke center. We analyzed three scenario-based prehospital stroke screening performances using the final hospital diagnoses: (1) a conservative approach only in patients who underwent the PHSST, (2) a real-world approach that considered all uncooperative patients as screening positive, and (3) a contrapositive approach that all uncooperative patients were considered as negative. RESULTS: Of the 2,836 emergency medical services (EMS)-transported adult patients who met the prehospital criteria for suspicion of acute stroke, 486 (17.1%) were uncooperative, and 570 (20.1%) had a confirmed final diagnosis of acute stroke. The diagnosis in the uncooperative group did not differ from that in the cooperative group (22.0% vs. 19.7%, P=0.246). The diagnostic performances of the PHSST in the conservative approach were as follows: 79.5% sensitivity (95% confidence interval [CI], 75.5%-83.1%), 90.2% specificity (95% CI, 88.8%-91.6%), and 0.849 area under the receiver operating characteristic curve (AUC; 95% CI, 0.829-0.868). The sensitivity and specificity were 83.3% (95% CI, 80.0%-86.3%) and 75.2% (95% CI, 73.3%-76.9%), respectively, in the real-world approach and 64.6% (95% CI, 60.5%-68.5%) and 91.9% (95% CI, 90.7%-93.0%), respectively, in the contrapositive approach. No significant difference was evident in the AUC between the real-world approach and the contrapositive approach (0.792 [95% CI, 0.775-0.810] vs. 0.782 [95% CI, 0.762-0.803], P>0.05). CONCLUSION: We found overestimation (false positive) and underestimation (false negative) in the uncooperative group depending on the scenario-based EMS stroke screening policy for uncooperative prehospital patients suspected of acute stroke.

Dual antiplatelet Use for extended period taRgeted to AcuTe ischemic stroke with presumed atherosclerotic OrigiN (DURATION) trial: Rationale and design.

RATIONALE: The optimal duration of dual antiplatelet therapy (DAPT) with clopidogrel-aspirin for the large artery atherosclerotic (LAA) stroke subtype has been debated. AIMS: To determine whether the 1-year risk of recurrent vascular events could be reduced by a longer duration of DAPT in patients with the LAA stroke subtype. METHODS AND STUDY DESIGN: A total of 4806 participants will be recruited to detect a statistically significant relative risk reduction of 22% with 80% power and a two-sided alpha error of 0.05, including a 10% loss to follow-up. This is a registry-based, multicenter, prospective, randomized, open-label, blinded end point study designed to evaluate the efficacy and safety of a 12-month duration of DAPT compared with a 3-month duration of DAPT in the LAA stroke subtype. Patients will be randomized (1:1) to either DAPT for 12 months or DAPT for 3 months, followed by monotherapy (either aspirin or clopidogrel) for the remaining 9 months. STUDY OUTCOMES: The primary efficacy outcome of the study is a composite of stroke (ischemic or hemorrhagic), myocardial infarction, and all-cause mortality for 1 year after the index stroke. The secondary efficacy outcomes are (1) stroke, (2) ischemic stroke or transient ischemic attack, (3) hemorrhagic stroke, and (4) all-cause mortality. The primary safety outcome is major bleeding. DISCUSSION: This study will help stroke physicians determine the appropriate duration of dual therapy with clopidogrel-aspirin for patients with the LAA stroke subtype. TRIAL REGISTRATION: URL: https://cris.nih.go.kr/cris. CRIS Registration Number: KCT0004407.

Impact of Neuroimaging Patterns for the Detection of Atrial Fibrillation by Implantable Loop Recorders in Patients With Embolic Stroke of Undetermined Source.

Objectives: Atrial fibrillation (AF) is a well-known etiology of embolic stroke of undetermined source (ESUS), although the optimal detection strategy of AF was not been fully evaluated yet. We assessed AF detection rate by implantable loop recorder (ILR) in patients with ESUS and compared the clinical characteristics and neuroimaging patterns between the patients with AF and AF-free patients. Methods: We reviewed clinical characteristics and neuroimaging patterns of consecutive patients with who were admitted to our comprehensive stroke center for ESUS and underwent ILR insertion between August 1, 2019, and January 31, 202. The inclusion criteria were (1) 18 years of age or older; (2) classified as having cryptogenic stroke extracted from the group with undetermined stroke according to ESUS International Working Group; and (3) underwent ILR insertion during or after admission due to index ischemic events. Ischemic stroke pattern was classified as (1) tiny-scattered infarction, (2) whole-territorial infarction, (3) lobar infarction and (4) multiple-territorial infarction. Interrogations of data retrieved from the ILR were performed by cardiologists in every month after the implantation. Results: In this study, 41 ESUS patients who received an ILR implantation were enrolled (mean age, 64 years; male sex, 65.9%). The rate of AF detection at 6 months was 34% (14 patients), and the mean time from ILR insertion to AF detection was 52.5 days [interquartile range (IQR), 45.0-69.5]. The median initial NIH stroke scale scores were significantly greater in patients with AF than those without AF (6.5 vs. 3.0, p = 0.019). Whole-territorial infarction pattern was significantly more frequent in patients with AF than in those without AF (64.3% vs.11.1%, p = 0.002). Conclusions: Higher covert AF detection rates within the ESUS patients were most often associated with higher NIHSS and whole-territorial infarction patterns on brain imaging.

Unrecognized Ruptured Intracranial Aneurysm Presenting as Cerebral Vasospasm-Induced Ischemic Stroke: A Case Report.

A 44-year-old woman presented with acute confusion, apparently due to a clinically silent subarachnoid hemorrhage followed by vasospasm, which in turn led to an ischemic stroke. During the initial evaluation, an acute ischemic stroke in the left middle cerebral artery territory was observed. Magnetic resonance imaging revealed a late subacute hemorrhage in the left basal cistern. Digital subtraction angiography indicated the presence of a small saccular aneurysm that had recently ruptured, as well as vasospasm in the left circle of Willis. Balloon angioplasty and balloon-assisted coil embolization were performed for the vasospasm and saccular aneurysm, respectively. This case demonstrates that clinically silent subarachnoid hemorrhages resulting in ipsilateral vasospasm and infarction can occur as complications of a ruptured aneurysm.

Single Center Experience of the Balloon-Stent Technique for the Treatment of Unruptured Distal Internal Carotid Artery Aneurysms: Sharing a Simple and Reliable Tip to Use Scepter-Atlas Combination.

Purpose: The balloon-stent technique (BST) has certain strengths as an assisted technique for the treatment of complex aneurysms. After Atlas release, the BST can be executed without an exchange maneuver of the balloon to the stent-delivery catheter. The purpose of this article is to share our experience with the BST using the Scepter-Atlas combination. Materials and Methods: Device inspection led us to a simple method to avoid failure in loading Atlas to the Scepter. From March 2018 to December 2019, 57 unruptured distal internal carotid artery (dICA) aneurysms were treated with coil embolization; among which, 25 aneurysms in 23 patients were treated with BST. Clinical and angiographic data were retrospectively collected and reviewed. Results: The technical success rate of the Scepter-Atlas combination increased from 50% to 100% after careful inspection. BST angiographic results were comparable to the stent-assisted coil (SAC) group treated during the immediately post-embolization same period (modified Raymond-Roy classification [MRRC] 1 & 2 84% in BST, 96.3% in SAC) and during short-term follow-up (MRRC 1 & 2 95.8% in BST, 88.4% in SAC). A small number of patients showed periprocedural complications, but none had clinical consequences. Conclusion: BST using the Scepter-Atlas combination can provide an effective and safe method for the treatment of dICA aneurysms. Scepters can be used as delivery catheters for Atlas.

Clinical Outcomes of Endovascular Treatment for Carotid Artery Dissection Without Intracranial Large Vessel Occlusion in Patients With Cerebral Ischemia Presentation.

BACKGROUND AND PURPOSE: We describe the clinical characteristics and outcomes (including the long-term patency of endovascular treatment [EVT]) of patients with acute ischemic strokes (AISs) featuring carotid artery dissection (CAD) but not intracranial large vessel occlusion. METHODS: We retrospectively reviewed patients who underwent EVT for spontaneous or post-traumatic AISs with CAD over a 13 year period from September 2005 to November 2018. The indications for EVT in patients with AIS-related CAD were a pretreatment diffusion-weighted imaging-Alberta Stroke Program early computed tomography (ASPECT) score > 6 and, clinical-diffusion mismatch. But, if the patients showed fluctuated ischemic symptoms, the joint decision by a stroke neurologist and neurointerventionist was done according to the onset-to-door time, symptoms, patient data, and the initial neuroimaging findings whether indicated that EVT was appropriate. RESULTS: Twenty-two dissected carotid arteries underwent balloon angioplasty and/or stent placement. The patients were 6 women and 16 men of median age 46 years. Twelve lacked any trauma history. Recombinant tissue plasminogen activator was prescribed for two (9.1%) patients. Four developed symptomatic intracranial hemorrhages (18.2%) but 86.4% exhibited modified Rankin scores ≤ 2. CONCLUSIONS: Although attention to the hemorrhagic complication is required, EVT for selective patients with cerebral ischemia associated with CAD may be safe and acceptable treatment strategy for reconstruction of luminal patency, with good clinical outcomes. Prospective large-scale randomized studies are required to optimize EVT for CAD patients.

Prevalence of Low Back Pain and Associated Risk Factors among Farmers in Jeju.

BACKGROUND: We aimed to investigate the prevalence of low back pain (LBP) and its associated agricultural work-related, biomechanical factors among this population. METHODS: We analyzed initial survey data from the Safety for Agricultural Injury of Farmers cohort study involving adult farmers in Jeju Island. The prevalence of LBP was calculated with associated factors. RESULTS: In total, 1,209 participants were included in the analysis. The overall prevalence of LBP was 23.7%. Significant associations for LBP were the type of farming activity, length of farming career, prior agricultural injury within 1 year, and stress levels. Multivariate logistic regression analysis revealed three biomechanical factors significantly related to LBP: repetitive use of particular body parts; the inappropriate posture of the lower back and neck. CONCLUSIONS: Some occupational, and biomechanical risk factors contribute to LBP. Therefore, postural education, injury prevention education, and psychological support will be needed to prevent LBP.

Pathogenic NOTCH3 Variants Are Frequent Among the Korean General Population.

OBJECTIVE: This study aimed to determine the frequency of pathogenic NOTCH3 variants among Koreans. METHODS: In this cross-sectional study, we queried for pathogenic NOTCH3 variants in 2 Korean public genome databases: the Korean Reference Genome Database (KRGDB) and the Korean Genome Project (Korea1K). In addition, we screened the 3 most common pathogenic NOTCH3 variants (p.Arg75Pro, p.Arg544Cys, and p.Arg578Cys) for 1,000 individuals on Jeju Island, where the largest number of patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) have been reported in Korea. RESULTS: The pathogenic NOTCH3 variant (p.Arg544Cys) was found in 0.12% of sequences in the KRGDB, and 3 pathogenic variants (p.Arg75Pro, p.Arg182Cys, and p.Arg544Cys) were present in 0.44% of the Korea1K database. Of the 1,000 individuals on Jeju Island, we found 2 cysteine-altering NOTCH3 variants (p.Arg544Cys variant in 9 and p.Arg578Cys in 1 individual) in 1.00% of the participants (95% confidence interval: 0.48%-1.83%). The presence of cysteine-altering NOTCH3 variants was significantly associated with a history of stroke (p < 0.001). DISCUSSION: Pathogenic NOTCH3 variants are frequently found in the general Korean population. Such a high prevalence of pathogenic variants could threaten the brain health of tens of thousands to hundreds of thousands of older adults in Korea.

Seroreactivity to Coxiella burnetii in an Agricultural Population and Prevalence of Coxiella burnetii Infection in Ticks of a Non-Endemic Region for Q Fever in South Korea.

Coxiella burnetii infects humans and wild and domesticated animals. Although reported cases on Jeju Island, off the coast of South Korea, are rare, the region is considered to have a high potential for Q fever. We investigated the seroprevalence of antibodies to C. burnetii in 230 farmers living in ten rural areas on Jeju Island between January 2015 and December 2019. Blood samples were collected and examined for C. burnetii Phase I/II IgM and IgG antibodies. Trained researchers collected ticks from rural areas. Clone XCP-1 16S ribosomal RNA gene sequencing was performed to identify Coxiella species from the collected ticks. The overall seroprevalence of antibodies to C. burnetii in farmers was 35.7%. The seroprevalence was significantly higher in fruit farmers. Of the collected ticks, 5.4% (19/351) of the Haemaphysalis longicornis ticks harbored C. burnetti. A high seroprevalence of antibodies to C. burnetii was observed in this region of Jeju Island, confirming that C. burnetti is endemic. Physicians should thus consider Q fever in the differential diagnosis of patients that present with acute fever after participating in outdoor activities.

Apolipoprotein E ε4 Is Associated With the Development of Incident Dementia in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Patients With p.Arg544Cys Mutation.

BACKGROUND AND PURPOSE: To identify clinical, laboratory, and magnetic resonance imaging (MRI) features in predicting incident stroke and dementia in Korean patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). MATERIALS AND METHODS: We enrolled 87 Korean CADASIL patients who had undergone baseline clinical, laboratory, and MRI examinations between March 2012 and February 2015. The primary outcome of this study is the occurrence of stroke and dementia during the study period. The occurrence of incident stroke was confirmed by neuroimaging study, and dementia was defined by the diagnostic and statistical manual of mental disorders, fourth edition, criteria. RESULTS: Of the 87 patients, 57.5% were men, and the mean age was 63 ± 13 years (range 34-90 years), and 82 patients (94.3%) had p.Arg544Cys mutation. During an average follow-up of 67 months (interquartile range: 53-69 months), incident stroke occurred in 14 of 87 patients (16.1%) and incident dementia in 7 of 70 non-demented patients (10.0%). In adjusted analysis, increased systolic blood pressure was associated with increased risk of incident stroke [for every 10-mmHg increase; hazard ratio, 1.44 (1.02-2.03)]. Apolipoprotein E ε4 genotype was associated with an increased risk of incident dementia [hazard ratio, 10.70 (1.27-89.88)]. CONCLUSION: In this study, apolipoprotein E ε4 genotype was associated with the development of incident dementia, and higher blood pressure was associated with increased risk of incident stroke in CADASIL patients with predominant p.Arg544Cys mutation.