Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 GeneTranslocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures

Ewing sarcoma and peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant tumor that is most prevalent in children and young adults and often radiologically mimics a meningioma. A 38-year-old female patient visited our hospital with complaints of right-sided tinnitus, right hemiparesis, and imbalance. She underwent preoperative imaging and was subsequently diagnosed as having a meningioma on the petrous ridge. After partial resection, EWSR1-FLI1 gene fusion was confirmed, and she was diagnosed with ES/pPNET. The tumor was successfully treated using a multidisciplinary approach of adjuvant chemo- and radiotherapy. This case is noteworthy because it is an extremely rare case of an intracranial ES/pPNET, and it is worth sharing our clinical experience that the tumor was successfully treated through a multidisciplinary therapeutic approach even though complete resection was not achieved.

Influence of Concurrent and Adjuvant Temozolomide on Health-Related Quality of Life of Patients with Grade III Gliomas: A Secondary Analysis of a Randomized Clinical Trial (KNOG-1101 Study) / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#The KNOG-1101 study showed improved 2-year PFS with temozolomide during and after radiotherapy compared to radiotherapy alone for patients with anaplastic gliomas. This trial investigates the effect of concurrent and adjuvant temozolomide on health-related quality of life (HRQoL). @*Materials and Methods@#In this randomized, open-label, phase II trial, 90 patients with World Health Organization grade III glioma were enrolled across multiple centers in South Korea between March 2012 to February 2015 and followed up through 2017. The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire 30 (EORTC QLQ-C30) and 20-item EORTC QLQ-Brain Neoplasm (QLQ-BN20) were used to compare HRQoL between patients assigned to concurrent chemoradiotherapy with temozolomide followed by 6 cycles of adjuvant temozolomide (arm A) and radiotherapy (RT) alone (arm B). @*Results@#Of the 90 patients in the study, 84 patients (93.3%) completed the baseline HRQoL questionnaire. Emotional functioning, fatigue, nausea and vomiting, dyspnea, constipation, appetite loss, diarrhea, seizures, itchy skin, drowsiness, hair loss, and bladder control were not affected by the addition of temozolomide. All other items did not differ significantly between arm A and arm B throughout treatment. Global health status particularly stayed consistent at the end of adjuvant temozolomide (p=0.47) and at the end of RT (p=0.33). @*Conclusion@#The addition of concurrent and adjuvant temozolomide did not show negative influence on HRQoL with improvement of progression-free survival for patients with anaplastic gliomas. The absence of systematic and clinically relevant changes in HRQoL suggests that an overall long-term net clinical benefit exists for concurrent and adjuvant temozolomide.

MRI Findings in Parkinson’s Disease: Radiologic Assessment of Nigrostriatal Degeneration

Parkinson’s disease (PD) is a movement disorder that develops due to degenerative loss of dopaminergic cells in the substantia nigra of the midbrain. Recent advances in MRI techniques have demonstrated various imaging findings that can reflect the underlying pathophysiological processes occurring in Parkinson’s disease. Many imaging studies have shown that such findings can assist in the diagnosis of Parkinson’s disease and its differentiation from atypical parkinsonism. In this review, we present MRI techniques that can be used in clinical assessment, such as nigrosome imaging and neuromelanin imaging, and we provide the detailed imaging features of Parkinson’s disease reflecting nigrostriatal degeneration.

Delayed Lesions on Diffusion-Weighted Imaging in Initially Lesion-Negative Stroke Patients / 대한뇌졸중학회지

Background@#and Purpose Lesions on diffusion-weighted imaging (DWI) occasionally appear on follow-up magnetic resonance imaging (MRI) among initially DWI-negative but clinically suspicious stroke patients. We established the prevalence of positive conversion in DWI-negative stroke and determined the clinical factors associated with it. @*Methods@#This retrospective, observational, single-center study included 5,271 patients hospitalized due to stroke/transient ischemic attack (TIA) in a single university hospital during 2010 to 2017. Patients without initial DWI lesions underwent follow-up DWI imaging as a routine practice. Adjusted hazard ratios (aHRs) for recurrent stroke risk according to positive conversion were determined using Cox proportional hazard regression. Adjusted odds ratios (aORs) and 95% confidence intervals (CIs) for positive conversion among initially DWI-negative patients were estimated. @*Results@#In total, 694 (13.2%) patients (mean±standard deviation age, 62.9±13.7 years; male, 404 [58.2%]) were initially DWI-negative. Among them, 22.5% had positive-conversion on follow-up DWI. Positive conversion was associated with a higher risk of recurrent stroke (aHR, 3.12; 95% CI, 1.56 to 6.26). Early neurologic deterioration (aOR, 15.1; 95% CI, 5.71 to 47.66), atrial fibrillation (aOR, 6.17; 95% CI, 3.23 to 12.01), smoking (aOR, 3.76; 95% CI, 2.19 to 6.63), pre-stroke dependency (aOR, 1.62; 95% CI, 1.15 to 2.27), objective hemiparesis (aOR, 4.39; 95% CI, 1.90 to 10.32), longer symptom duration (aOR, 2.17; 95% CI, 1.57 to 3.08), high cholesterol (aOR, 4.70; 95% CI, 1.78 to 12.77), National Institutes of Health Stroke Scale score (aOR, 1.44; 95% CI, 1.08 to 1.91), and high systolic blood pressure (aOR, 1.01; 95% CI, 1.00 to 1.02) were associated with a higher incidence of lesions with delayed appearance. Regarding the location of lesions on follow-up DWI, 34.6% and 21.2% were in the cortex and brainstem, respectively. @*Conclusions@#In DWI-negative stroke/TIA, positive conversion is associated with a higher risk of recurrent stroke. DWI-negative stroke with factors related to positive conversion may require follow-up MRI for a definitive diagnosis.

Concurrent and Adjuvant Temozolomide for Newly Diagnosed Grade IIIGliomas without 1p/19q Co-deletion: A Randomized, Open-Label,Phase 2 Study (KNOG-1101 Study) / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#We investigated the efficacy of temozolomide during and after radiotherapy in Korean adultswith anaplastic gliomas without 1p/19q co-deletion. @*Materials and Methods@#This was a randomized, open-label, phase 2 study and notably the first multicenter trial forKorean grade III glioma patients. Eligible patients were aged 18 years or older and hadnewly diagnosed non-co-deleted anaplastic glioma with an Eastern Cooperative OncologyGroup performance status of 0-2. Patients were randomized 1:1 to receive radiotherapyalone (60 Gy in 30 fractions of 2 Gy) (control group, n=44) or to receive radiotherapy withconcurrent temozolomide (75 mg/m2/day) followed by adjuvant temozolomide (150-200mg/m2/day for 5 days during six 28-day cycles) (treatment group, n=40). The primary endpointwas 2-year progression-free survival (PFS). Seventy patients (83.3%) were availablefor the analysis of the isocitrate dehydrogenase 1 gene (IDH1) mutation status. @*Results@#The two-year PFS was 42.2% in the treatment group and 37.2% in the control group. Overallsurvival (OS) did not reach to significant difference between the groups. In multivariableanalysis, age was a significant risk factor for PFS (hazard ratio [HR], 2.08; 95% confidenceinterval [CI], 1.04 to 4.16). The IDH1mutation was the only significant prognostic factor forPFS (HR, 0.28; 95% CI, 0.13 to 0.59) and OS (HR, 0.19; 95% CI, 0.07 to 0.50). Adverseevents over grade 3 were seen in 16 patients (40.0%) in the treatment group and werereversible. @*Conclusion@#Concurrent and adjuvant temozolomide in Korean adults with newly diagnosed nonco-deleted anaplastic gliomas showed improved 2-year PFS. The survival benefit of this regimenneeds further analysis with long-term follow-up at least more than 10 years.

Clinical Characteristics of High-Grade Glioma with Primary Leptomeningeal Seeding at Initial Diagnosis in a Single Center Study

Background@#High-grade glioma (HGG) with primary leptomeningeal seeding (PLS) at initial diagnosis is rare. The purpose of this study was to identify its clinical features and to describe the clinical treatment outcomes. @*Methods@#We retrospectively reviewed the medical records of patients with HGG (World Health Organization grade III or IV) at our institution between 2004 and 2019, and patients with PLS at the initial diagnosis were enrolled in the study. Clinical features, such as the location of leptomeningeal seeding, surgical methods, and degree of resection, were sorted based on electronic medical records also containing performance scale, and hematological and serological evaluations. Radiological findings and immunohistochemical categories were confirmed. Furthermore, we sought to determine whether controlling intracranial pressure (ICP) via early cerebrospinal fluid (CSF) diversion increases overall survival (OS) after the initial diagnosis. @*Results@#Of the 469 patients with HGG in our institution, less than 2% had PLS at the initial diagnosis. Most patients suffered from headache, diplopia, and dizziness. Pathological findings included 7 glioblastomas and 2 anaplastic astrocytomas. Seven of the 9 patients underwent CSF diversion. All patients were administered concurrent chemoradiotherapy (CCRT) with temozolomide, 89% of which started adjuvant temozolomide and 33% of which completed the six cycles of adjuvant temozolomide.The OS of patients with HGG and PLS was 8.7 months (range, 4-37), an extremely poor result compared to that of other studies. Also, the 1-year and 2-year OS rates were 44.4% and 16.7%, respectively. @*Conclusion@#Diagnosis and treatment of HGG with PLS are challenging. Aggressive control of ICP followed by early initiation of standard CCRT seems to be helpful in improving symptoms. However, despite aggressive treatment, the prognosis is poor. A multicenter trial and research may be necessary to create a standardized protocol for this disease.

Correlation Between Vanishing White Matter Disease and Novel Heterozygous EIF2B3 Variants Using Next-Generation Sequencing: A Case Report

Vanishing white matter (VWM) disease is an autosomal recessive disorder that affects the central nervous system of a patient, and is caused by the development of pathogenic mutations in any of the EIF2B1-5 genes. Any dysfunction of the EIF2B1-5 gene encoded eIF2B causes stress-provoked episodic rapid neurological deterioration in the patient, followed by a chronic progressive disease course. We present the case of a patient with an infantile-onset VWM with the pre-described specific clinical course, subsequent neurological aggravation induced by each viral infection, and the noted consequent progression into a comatose state. Although the initial brain magnetic resonance imaging did not reveal specific pathognomonic signs of VWM to distinguish it from other types of demyelinating leukodystrophy, the next-generation sequencing studies identified heterozygous missense variants in EIF2B3, including a novel variant in exon 7 (C706G), as well as a 0.008% frequency reported variant in exon 2 (T89C). Hence, the characteristic of unbiased genomic sequencing can clinically affect patient care and decisionmaking, especially in terms of the consideration of genetic disorders such as leukoencephalopathy in pediatric patients.

Dichotomizing Level of Pial Collaterals on Multiphase CT Angiography for Endovascular Treatment in Acute Ischemic Stroke: Should It Be Refined for 6-Hour Time Window? / 신경중재치료의학

PURPOSE: Although endovascular treatment is currently thought to only be suitable for patients who have pial arterial filling scores >3 as determined by multiphase computed tomography angiography (mpCTA), a cut-off score of 3 was determined by a study, including patients within 12 hours after symptom onset. We aimed to investigate whether a cut-off score of 3 for endovascular treatment within 6 hours of symptom onset is an appropriate predictor of good functional outcome at 3 months. MATERIALS AND METHODS: From April 2015 to January 2016, acute ischemic stroke patients treated with mechanical thrombectomy within 6 hours of symptom onset were enrolled into this study. Pial arterial filling scores were semi-quantitatively assessed using mpCTA, and clinical and radiological parameters were compared between patients with favorable and unfavorable outcomes. Multivariate logistic regression analysis was then performed to investigate the independent association between clinical outcome and pial collateral score, with the predictive power of the latter assessed using C-statistics. RESULTS: Of the 38 patients enrolled, 20 (52.6%) had a favorable outcome and 18 had an unfavorable outcome, with the latter group showing a lower mean pial arterial filling score (3.6±0.8 vs. 2.4±1.2, P=0.002). After adjusting for variables with a P-value of 2 vs. ≤2. CONCLUSION: A pial arterial filling cut-off score of 2 as determined by mpCTA appears to be more suitable for predicting clinical outcomes following endovascular treatment within 6 hours of symptom onset than the cut-off of 3 that had been previously suggested.

Differentiation of Deep Subcortical Infarction Using High-Resolution Vessel Wall MR Imaging of Middle Cerebral Artery

OBJECTIVE: To evaluate the utility of high-resolution vessel wall imaging (HR-VWI) of middle cerebral artery (MCA), and to compare HR-VWI findings between striatocapsular infarction (SC-I) and lenticulostriate infarction (LS-I). MATERIALS AND METHODS: This retrospective study was approved by the Institutional Review Board, and informed consent was waived. From July 2009 to February 2012, 145 consecutive patients with deep subcortical infarctions (SC-I, n = 81; LS-I, n = 64) who underwent HR-VWI were included in this study. The degree of MCA stenosis and the characteristics of MCA plaque (presence, eccentricity, location, extent, T2-high signal intensity [T2-HSI], and plaque enhancement) were analyzed, and compared between SC-I and LS-I, using Fisher's exact test. RESULTS: Stenosis was more severe in SC-I than in LS-I (p = 0.040). MCA plaque was more frequent in SC-I than in LS-I (p = 0.028), having larger plaque extent (p = 0.001), more T2-HSI (p = 0.001), and more plaque enhancement (p = 0.002). The eccentricity and location of the plaque showed no significant difference between the two groups. CONCLUSION: Both SC-I and LS-I have similar HR-VWI findings of the MCA plaque, but SC-I had more frequent, larger plaques with greater T2-HSI and enhancement. This suggests that HR-VWI may have a promising role in assisting the differentiation of underlying pathophysiological mechanism between SC-I and LS-I.

Influence of B₁-Inhomogeneity on Pharmacokinetic Modeling of Dynamic Contrast-Enhanced MRI: A Simulation Study

OBJECTIVE: To simulate the B₁-inhomogeneity-induced variation of pharmacokinetic parameters on dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). MATERIALS AND METHODS: B₁-inhomogeneity-induced flip angle (FA) variation was estimated in a phantom study. Monte Carlo simulation was performed to assess the FA-deviation-induced measurement error of the pre-contrast R₁, contrast-enhancement ratio, Gd-concentration, and two-compartment pharmacokinetic parameters (K(trans), v(e), and v(p)). RESULTS: B₁-inhomogeneity resulted in −23–5% fluctuations (95% confidence interval [CI] of % error) of FA. The 95% CIs of FA-dependent % errors in the gray matter and blood were as follows: −16.7–61.8% and −16.7–61.8% for the pre-contrast R₁, −1.0–0.3% and −5.2–1.3% for the contrast-enhancement ratio, and −14.2–58.1% and −14.1–57.8% for the Gd-concentration, respectively. These resulted in −43.1–48.4% error for K(trans), −32.3–48.6% error for the v(e), and −43.2–48.6% error for v(p). The pre-contrast R₁ was more vulnerable to FA error than the contrast-enhancement ratio, and was therefore a significant cause of the Gd-concentration error. For example, a −10% FA error led to a 23.6% deviation in the pre-contrast R₁, −0.4% in the contrast-enhancement ratio, and 23.6% in the Gd-concentration. In a simulated condition with a 3% FA error in a target lesion and a −10% FA error in a feeding vessel, the % errors of the pharmacokinetic parameters were −23.7% for K(trans), −23.7% for v(e), and −23.7% for v(p). CONCLUSION: Even a small degree of B₁-inhomogeneity can cause a significant error in the measurement of pharmacokinetic parameters on DCE-MRI, while the vulnerability of the pre-contrast R₁ calculations to FA deviations is a significant cause of the miscalculation.

Efficacy of Maximum Intensity Projection of Contrast-Enhanced 3D Turbo-Spin Echo Imaging with Improved Motion-Sensitized Driven-Equilibrium Preparation in the Detection of Brain Metastases

OBJECTIVE: To evaluate the diagnostic benefits of 5-mm maximum intensity projection of improved motion-sensitized driven-equilibrium prepared contrast-enhanced 3D T1-weighted turbo-spin echo imaging (MIP iMSDE-TSE) in the detection of brain metastases. The imaging technique was compared with 1-mm images of iMSDE-TSE (non-MIP iMSDE-TSE), 1-mm contrast-enhanced 3D T1-weighted gradient-echo imaging (non-MIP 3D-GRE), and 5-mm MIP 3D-GRE. MATERIALS AND METHODS: From October 2014 to July 2015, 30 patients with 460 enhancing brain metastases (size > 3 mm, n = 150; size ≤ 3 mm, n = 310) were scanned with non-MIP iMSDE-TSE and non-MIP 3D-GRE. We then performed 5-mm MIP reconstruction of these images. Two independent neuroradiologists reviewed these four sequences. Their diagnostic performance was compared using the following parameters: sensitivity, reading time, and figure of merit (FOM) derived by jackknife alternative free-response receiver operating characteristic analysis. Interobserver agreement was also tested. RESULTS: The mean FOM (all lesions, 0.984; lesions ≤ 3 mm, 0.980) and sensitivity ([reader 1: all lesions, 97.3%; lesions ≤ 3 mm, 96.2%], [reader 2: all lesions, 97.0%; lesions ≤ 3 mm, 95.8%]) of MIP iMSDE-TSE was comparable to the mean FOM (0.985, 0.977) and sensitivity ([reader 1: 96.7, 99.0%], [reader 2: 97, 95.3%]) of non-MIP iMSDE-TSE, but they were superior to those of non-MIP and MIP 3D-GREs (all, p 0.75) for all lesions in both sequences. CONCLUSION: MIP iMSDE-TSE showed high detectability of brain metastases. Its detectability was comparable to that of non-MIP iMSDE-TSE, but it was superior to the detectability of non-MIP/MIP 3D-GREs. With a shorter reading time, the false-positive results of MIP iMSDE-TSE were greater. We suggest that MIP iMSDE-TSE can provide high diagnostic performance and low false-positive rates when combined with 1-mm sequences.

Duplicated Origin of the Left Vertebral Artery: A Case Report and Embryological Review / 신경중재치료의학

The duplicated origin of vertebral artery (VA) is a very rare condition. It could be easily misdiagnosed as an arterial dissection on selective catheter angiography, especially in a patient with acute cerebellar infarction of unknown etiology. We report a patient with an acute cerebellar infarction and duplicated origin of the left VA, which was found during the selective catheter angiography.

A Comparison between Mechanical Thrombectomy and Intra-arterial Fibrinolysis in Acute Basilar Artery Occlusion: Single Center Experiences / 대한뇌졸중학회지

BACKGROUND AND PURPOSE: Recent advances in intra-arterial techniques and thrombectomy devices lead to high rate of recanalization. However, little is known regarding the effect of the evolvement of endovascular revascularization therapy (ERT) in acute basilar artery occlusion (BAO). We compared the outcome of endovascular mechanical thrombectomy (EMT) versus intra-arterial fibrinolysis (IAF)-based ERT in patients with acute BAO. METHODS: After retrospectively reviewed a registry of consecutive patients with acute ischemic stroke who underwent ERT from September 2003 to February 2015, 57 patients with acute BAO within 12 hours from stroke onset were enrolled. They were categorized as an IAF group (n=24) and EMT group (n=33) according to the primary technical option. We compared the procedural and clinical outcomes between the groups. RESULTS: The time from groin puncture to recanalization was significantly shorter in the EMT group than in the IAF group (48.5 [25.3 to 87.8] vs. 92 [44 to 179] minutes; P=0.02) The rate of complete recanalization was significantly higher in the EMT group than in the IAF group (87.9% vs 41.7%; P<0.01). The good outcome of the modified Rankin Scale score≤2 at 3 months was more frequent in the EMT group than in the IAF group, but it was not statistically significant (39.4% vs 16.7%; P=0.06). CONCLUSIONS: EMT-based ERT in patients with acute BAO is superior to IAF-based ERT in terms of the reduction of time from groin puncture to recanalization and the improvement of the rate of complete recanalization.

Quantitative Magnetic Resonance Angiography in Internal Carotid Artery Occlusion with Primary Collateral Pathway / 대한뇌졸중학회지

BACKGROUND AND PURPOSE: Quantitative magnetic resonance angiography (Q-MRA) enables direct measurement of volume flow rate (VFR) of intracranial arteries. We aimed to evaluate the collateral flows in internal carotid artery (ICA) occlusion with primary collateral pathway via circle of Willis using Q-MRA, and to compare them between patients who recently developed ipsilateral symptomatic ischemia and those who did not. METHODS: Between 2012 and 2014, 505 patients underwent Q-MRA in our institution. Among these, 33 patients who had unilateral ICA occlusion with primary collateral pathway were identified, and grouped into asymptomatic patients, stable patients with chronic infarction, and symptomatic patients with acute/subacute infarction. Mean VFR (mVFR) in intracranial arteries was measured and compared between the patients' groups. Kruskal-Wallis test was used for statistical analysis. RESULTS: Six patients were asymptomatic, fifteen with chronic infarction were stable, and twelve with acute/subacute infarction were symptomatic. The mVFR of ipsilateral middle cerebral artery in symptomatic patients was significantly lower than those in stable or asymptomatic patients (73.7+/-45.6 mL/min vs. 119.9+/-36.1 mL/min vs. 121.8+/-42.0 mL/min; P = 0.04). Total sum of the mVFR of ipsilateral anterior, middle, and posterior cerebral arteries was significantly lower in symptomatic patients than those in other groups (229.3 +/- 51.3 mL/min vs. 282.0+/-68.6 mL/min vs. 314.0+/-44.4 mL/min; P = 0.02). CONCLUSIONS: Q-MRA could be helpful to demonstrate the difference in the degree of primary collateral flow in ICA occlusion between the patients with recent symptomatic ischemia and those without.

Pseudoprogression and Pseudoresponse in the Management of High-Grade Glioma : Optimal Decision Timing According to the Response Assessment of the Neuro-Oncology Working Group

OBJECTIVE: We evaluated pseudoprogression (PsPD) following radiation therapy combined with concurrent temozolomide (TMZ), and we assessed pseudoresponse following anti-angiogenic therapy for patients with recurrent disease using the Response Assessment of the Neuro-Oncology Working Group. METHODS: Patients who were pathologically confirmed as having high-grade glioma received radiotherapy with concurrent TMZ followed by adjuvant TMZ. Bevacizumab (Avastin) with CPT-11 were used as a salvage option for cases of radiologic progression. Magnetic resonance imaging (MRI) was routinely performed 1 month after concurrent radiochemotherapy (CRT) and every 3 months thereafter. For cases treated with the bevacizumab-containing regimen for progressive disease, MRI was performed every 2 months. RESULTS: Of 55 patients, 21 (38%) showed radiologic progression within 4 weeks after CRT. Of these patients, 16 (29%) showed progression at second post-CRT MRI (etPD) and five (9%) showed improvement (PsPD). Seven of thirty-four initially non-progressed patients showed progression at the second post-CRT MRI (ltPD). No difference in survival was observed between the etPD and ltPD groups (p=0.595). Five (50%) of ten patients showed a radiological response after salvage bevacizumab therapy. Four of those patients exhibited rapid progression immediately after discontinuation of the drug (drug holiday). CONCLUSION: Twelve weeks following treatment could be the optimal timing to determine PsPD or true progression. MRI with gadolinium enhancement alone is not sufficient to characterize tumor response or growth. Clinical correlation with adequate follow-up duration and histopathologic validation may be helpful in discriminating PsPD from true progression.

Influence of 3 Stigmas of Small Vessel Disease on FLAIR Change in Acute Ischemic Stroke: Case-Control Study

BACKGROUND: Attempts have been made to use the signal changes of fluid-attenuated inversion recovery (FLAIR) MRI as "a tissue clock," defined as a surrogate marker of the tissue damage resulting from acute ischemic stroke. The evolution of FLAIR signals after stroke onset has never been fully explained solely by time. The aim of this study was to determine whether cerebral small-vessel disease (SVD) affects FLAIR changes following acute ischemic stroke. METHODS: Based on data from a prospective stroke registry, consecutive patients who were hospitalized to the stroke center within 12 hours of stroke onset between January 2004 and May 2011 and had occlusion of the major cerebral arteries in the anterior circulation, as evidenced by MR angiography, were enrolled. Cases with FLAIR changes and controls without FLAIR changes were matched according to the time elapsed from stroke onset to MR study. RESULTS: Among the 130 patients who met the eligibility criteria, 62 (47.7%) had FLAIR changes. The time interval between stroke onset and MR study differed significantly between those with and without FLAIR changes (5.2 hours vs. 3.0 hours). FLAIR changes were more common among males and smokers. Comparisons between cases and controls matched on a one-to-one basis did not reveal any difference in the three signs of cerebral SVD: white-matter hyperintensities, lacunae, and cerebral microbleeds. CONCLUSIONS: This study failed to find any data supporting the hypothesis that cerebral SVD affects FLAIR changes after acute ischemic stroke.

Diagnosis of Vertebral Artery Ostial Stenosis on Contrast-Enhanced MR Angiography: Usefulness of a Thin-Slab MIP Technique

It is a well-known clinical fact that contrast-enhanced magnetic resonance angiography exaggerates vertebral arterial ostial stenosis and sometimes shows pseudostenosis. Considering the clinical significance of a lesion in the posterior circulation ischemia, the importance of an accurate imaging diagnosis of ostial stenosis should not be underestimated. We were able to differentiate pseudostenosis of the ostium from true stenosis using thin-slab maximum-intensity-projection(MIP) images which are thought to be helpful for minimizing standard full thickness MIP images.

Treatment Strategy Based on Multimodal Management Outcome of Cavernous Sinus Dural Arteriovenous Fistula (CSDAVF) / 신경중재치료의학

PURPOSE: Angiographic finding including venous drainage pattern should be correlated to the presenting symptom pattern (SxP) in CSDAVF. We present outcome of CSDAVF management and suggest a strategy according to SxP and type of treatment based on our experience. MATERIALS AND METHODS: We evaluated SxP, angiographic type (proliferative, restrictive or late restrictive), mode of treatment (embolization, Gamma Knife Radiation (GKR) or conservative management), mode of embolization (transarterial or transvenous), and final clinical status (cure, improvement, aggravation or no change). Ninety consecutive patients were included from a prospective database. The mean follow-up was 17 months. We compared the outcomes according to SxP, angiographic type, mode of treatment, and embolization using the chi-square or Fisher's exact test. RESULTS: Ninety patients with 34 proliferative, 40 restrictive, and 16 late restrictive types of CSDAVF were treated by embolization (n = 63), GKR (n = 7), and conservative management (n = 20). Cure or improvement was 91% after embolization, 88% after conservative management, and 72% after GKR. Following embolization, 100% of 24 proliferative types, 87% of 30 restrictive types, and 90% of 10 late restrictive types were cured or improved. Cure or improvement after transvenous embolization was 98% (43/44) compared with 88% (15/17) after transarterial embolization (p = 0.003). CONCLUSION: Various factors of SxP, angiographic type, and mode of treatment should be considered in order to obtain a more favorable outcome for patients with CSDAVF. Embolization via venous approach tended to result in a more complete cure than that via arterial approach.

Use of Self-Expanding Stents for the Treatment of Vertebral Artery Ostial Stenosis: a Single Center Experience

OBJECTIVE: To evaluate our early experience using self-expanding stents to treat atherosclerotic vertebral artery ostial stenosis (VAOS), with respect to technical feasibility and clinical and imaging follow-up results. MATERIALS AND METHODS: A total of 20 lesions in 20 patients underwent stenting of the VAOS using a self-expanding stent (Precise RX; Cordis Neurovascular, Miami Lakes, FL). Two patients were asymptomatic. We analyzed the technical success rate, causes of technical failure, occurrence of any vascular or neurological event, and the occurrence of any neurological abnormality or in-stent restenosis (ISR) seen on follow-up. The imaging follow-up was performed with Doppler ultrasound (DUS) as a primary screening modality. RESULTS: One instance of technical failure was caused by failure of the guidewire passage. The stent diameter was 5 mm, and post-stenting balloon dilatations were necessary in all cases. Stent misplacement requiring placement of an additional stent occurred in four cases. Following a 14.8 month average clinical follow-up time, two patients showed anterior circulation ischemia, which was not attributed to the VAOS we treated. Following a 13.7 month average DUS follow-up, five patients showed a mild degree of diffuse or focal intimal thickening in the stent lumen; however, none of the stenosis showed luminal loss of more than 50% and no stent fracture was noted. CONCLUSION: The use of self-expanding stents for treating VAOS was technically feasible and helped to improve artery patency during our limited follow-up interval.

A Case of Hydranencephaly With Exotropia

PURPOSE: To report a large-angle exotropia, limited adduction, epiblepharon, high myopia and no pupillary light reflex in a patient with hydranencephaly. CASE SUMMARY: A ten-year-old girl with mental retardation presented with exotropia. The patient could fix only with the right eye and was unable to follow with either eye. The Krimsky test revealed 95 prism diopters of exotropia, and adduction was severely limited in both eyes. Pupillary light reflex was absent in both eyes. Cycloplegic refraction showed high myopia in both eyes. Slit lamp examination revealed lower lid epiblepharon and inferior corneal opacity in the right eye. No abnormal findings in the fundus examination were detected. A computed tomogram of the brain showed that the cerebral hemispheres were replaced by a cystic space filled with cerebrospinal fluid, compatible with hydranencephaly. Recession of the lateral rectus muscle and resection of the medial rectus muscle with epiblepharon repair of the lower lid were performed in both eyes. One week postoperatively, the epiblepharon was corrected, and the Krimsky test showed 16 prism diopters of left intermittent exotropia at near. CONCLUSIONS: When a combined manifestation of mental retardation, limited adduction, no pupillary light reflex and a large-angle exotropia is present, the possibility of a congenital developmental anomaly of the central nervous system including hydranencephaly should be suspected.