ABSTRACT
Hemangioblastomas (HBMs) are rare vascular tumors commonly located in the posterior fossa of adults. A mid-50s patient presented with sudden unconsciousness. Computed tomography scans revealed acute hemorrhages around the posterior fossa, predominantly in the subarachnoid space. Digital subtraction angiography (DSA) revealed an 8-mm round lesion filled with contrast agent, fed by the C1 segmental artery of the left vertebral artery (VA), showing early venous drainage to the spinal cord and brainstem. Emergent embolization was attempted under suspicion of a ruptured dural arteriovenous fistula, resulting in parent artery occlusion due to feeder selection failure. Follow-up DSA after a month depicted a persistent aneurysm via collaterals from both VAs. Consequently, the decision was made to proceed with surgical intervention, leading to the resection of the lesion, confirming its diagnosis as a HBM through histological examination. This case underscores the potential for misdiagnosis when HBMs with an intratumoral shunt mimic vascular shunt lesions.
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Purpose@#This study aimed to evaluate the effectiveness, safety, and technical considerations of flow diverter (FD) treatment using a Flow Re-direction Endoluminal Device (FRED) for unruptured intracranial vertebral artery dissecting aneurysms (VADAs). @*Materials and Methods@#We conducted a retrospective study of 23 patients with unruptured intracranial VADAs who underwent FD treatment using a FRED between June 2017 and August 2021. Symptoms, imaging findings, treatment strategies, and angiographic and clinical outcomes were evaluated. Dissections were categorized according to the dominance of the VA in which they occurred: dominant VA, co-dominant VA, and non-dominant VA. @*Results@#All patients successfully underwent FD treatment with either a FRED (n=11) or FRED Jr. (n=12). Complete occlusion rates were 78.3% at 6-month follow-up magnetic resonance angiography and 91.3% at 12-month. There were no instances of complications, recurrence, or retreatment during a median follow-up of 20 months. Dissections occurred in the dominant VA in 3 cases (13.0%), the co-dominant VA in 13 cases (56.5%), and the non-dominant VA in 7 cases (30.4%). Intimal flap and true lumen stenosis were observed in 39.1% and 30.4% of cases, respectively. Four cases required a bilateral VA approach due to technical difficulties, all in the non-dominant VA. @*Conclusion@#Flow diversion treatment using a FRED for unruptured intracranial VADAs proved feasible and safe, yielding satisfactory occlusion rates. Technical challenges were more likely in lesions involving non-dominant VAs in the acute or subacute stage, mainly due to associated intraluminal lesions compromising the arterial lumen.
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Objective@#Familial intracranial aneurysms (FIAs) are found in approximately 6%–20% of patients with intracranial aneurysms (IAs), suggesting that genetic predisposition likely plays a role in its pathogenesis. The aim of this study was to identify possible IA-associated variants using whole exome sequencing (WES) in selected Korean families with FIA. @*Materials and Methods@#Among the 26 families in our institutional database with two or more IA-affected first-degree relatives, three families that were genetically enriched (multiple, early onset, or common site involvement within the families) for IA were selected for WES. Filtering strategies, including a family-based approach and knowledge-based prioritization, were applied to derive possible IA-associated variants from the families. A chromosomal microarray was performed to detect relatively large chromosomal abnormalities. @*Results@#Thirteen individuals from the three families were sequenced, of whom seven had IAs. We noted three rare, potentially deleterious variants (PLOD3 c.1315G>A, NTM c.968C>T, and CHST14 c.58C>T), which are the most promising candidates among the 11 potential IA-associated variants considering gene-phenotype relationships, gene function, co-segregation, and variant pathogenicity. Microarray analysis did not reveal any significant copy number variants in the families. @*Conclusion@#Using WES, we found that rare, potentially deleterious variants in PLOD3, NTM, and CHST14 genes are likely responsible for the subsets of FIAs in a cohort of Korean families.
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Purpose@#Spontaneous cervicocephalic dissection (SCAD) is an important cause of stroke and shows various lesion locations and clinical features. The purpose of this study was to analyze the location of SCAD and its clinical and radiologic patterns in Korean patients. @*Materials and Methods@#Patients with SCAD who were evaluated between 2013 and 2018 at a tertiary center in Korea were reviewed. We classified and compared the morphological (aneurysm or steno-occlusion) and presenting (hemorrhage or infarction) patterns according to the lesion locations (anterior circulation [AC] vs. posterior circulation [PC]; intradural [ID] vs. extradural [ED]). @*Results@#A total of 166 patients were included in this study. The SCAD most commonly occurred in the PC-ID location (65.1%), followed by AC-ID (13.3%), AC-ED (13.3%), and PC-ED (8.4%). Aneurysm and steno-occlusion patterns were observed in 66.9% and 57.8% of the cases, respectively. The aneurysm pattern was significantly more common in the PC-ID location (78.7%) than in other locations. As for the presenting pattern, cerebral infarction was the most common pattern (39.8%), and intracranial hemorrhage was observed only in the ID location (7.2%). @*Conclusion@#In Korean patients, PC-ID, especially ID vertebral artery, was the most common location of SCAD, and most cases were accompanied by an aneurysm. It also suggested that these location trends differ by population or ethnicity.
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Purpose@#Reversible aggravation of myelopathy symptoms was observed after the intake of taurine-rich foods in patients with venous congestive myelopathy (VCM) caused by a spinal arteriovenous shunt (SAVS), and the taurine-challenge test was applied to demonstrate an association between taurine and VCM. @*Materials and Methods@#The current study reviewed any aggravation history of myelopathy symptoms, including walking difficulty, after consuming taurine-rich foods among 133 consecutive patients with a SAVS from a prospective institutional database from June 2013 to February 2021. The type of taurine-rich foods, demographic data, arteriovenous shunt level, and follow-up periods were obtained. For the controlled taurine challenge test, Bacchus® (Dong-A Pharmaceutical, Seoul, Korea), a taurine-rich drink, was given to patients who fulfilled test criteria of recovered VCM (pain-sensory-motor-sphincter scale ≥2, improvement of spinal cord signal intensity on magnetic resonance imaging, and follow-up >6 months after SAVS treatment) to confirm the disappearance of such aggravation. @*Results@#Ten patients had an aggravation history related to food. Webfoot octopus, small octopus, squid, crab, scallop, and taurine-rich energy drink (Bacchus®) were related to such aggravation in patients with VCM. Aggravation appeared about 30 minutes after food intake followed by expressions such as ‘I could not walk and collapsed to the ground’ and usually lasted for about 3 hours, followed by a slow recovery after taking rest. Four patients who met the test criteria underwent the taurine challenge with Bacchus® and revealed no further symptom aggravation, suggesting that taurine did not affect patients after recovery from VCM. @*Conclusion@#The association between taurine-rich food and reversible symptom aggravation can appear in patients with VCM and disappear after VCM treatment. Aggravation of venous hypertension in the spinal cord is suggested as a mechanism but further elucidation is needed.
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Objective@#This study aimed to assess the outcomes of outpatient day-care management of unruptured intracranial aneurysm (UIA), and to present the risks associated with different management strategies by comparing the outcomes and adverse events between outpatient day-care management and management with longer admission periods. @*Materials and Methods@#This retrospective cohort study used prospectively registered data and was approved by a local institutional review board. We enrolled 956 UIAs from 811 consecutive patients (mean age ± standard deviation, 57 ± 10.7 years; male:female = 247:564) from 2017 to 2020. We compared the outcomes after embolization among the different admission-length groups (1, 2, and ≥ 3 days). The outcomes included pre- and post-modified Rankin Scale (mRS) scores and rates of adverse events, cure, recurrence, and reprocedure. Events were defined as any cerebrovascular problems, including minor and major stroke, death, or hemorrhage. @*Results@#The mean admission period was 2 days, and 175 patients (191 aneurysms), 551 patients (664 aneurysms), and 85 patients (101 aneurysms) were discharged on the day of the procedure, day 2, and day 3 or later, respectively. During the mean 17-month follow-up period (range 6–53 months; 2757 patient years), no change in post-mRS was observed compared to pre-mRS in 99.6% of patients. Cure was achieved in 95.6% patients; minimal recurrence that did not require re-procedure occurred in 3.5% patients, and re-procedure was required in 2.3% (22 of 956) patients due to progressive enlargement of the recurrent sac during follow up (mean 17 months, range, 6–53 months). There were eight adverse events (0.8%), including five cerebrovascular (two major stroke, two minor strokes and one transient ischemic stroke), and three non-cerebrovascular events. Statistical comparison between groups with different admission lengths (1, 2, and ≥ 3 days) revealed no difference in the outcomes. @*Conclusion@#This study revealed no difference in outcomes and adverse events according to the admission period, and suggested that UIA could be managed by outpatient day-care embolization.
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Purpose@#Spinal epidural arteriovenous fistulas (SEDAVFs) show an epidural venous sac often with venous congestive myelopathy (VCM) due to intradural reflux at a remote level to which a transarterial approach would be difficult. We present 12 cases of SEDAVF with VCM and describe 3 main tactics for effective transarterial embolization. @*Materials and Methods@#Among 152 patients with spinal vascular malformations diagnosed in our tertiary hospital between 1993 and 2019, 12 SEDAVF patients with VCM were included. Three different transarterial embolization tactics were applied according to the vascular configuration and microcatheter accessibility. We evaluated treatment results and clinical outcomes before and after treatment. @*Results@#Transarterial embolization with glue (20–30%) was performed in all patients. The embolization tactics applied in 12 patients were preferential flow (n=2), plug-and-push (n=6), and filling of the venous sac (n=4). Total occlusion of the SEDAVF, including intradural reflux, was achieved in 11 (91.7%) of 12 patients, and partial occlusion was achieved in 1 patient. No periprocedural complications were reported. Spinal cord edema was improved in all patients for an average of 18 months after treatment. Clinical functional outcome in terms of the pain, sensory, motor, and sphincter scale and modified Rankin scores improved during a mean 25-month follow-up (6.3 vs. 3.3, P=0.002; 3.6 vs. 2.3, P=0.002, respectively). @*Conclusion@#Endovascular treatment for 12 SEDAVF patients with VCM achieved a total occlusion rate of 91.7% without any periprocedural complication. The combined embolization tactics can block intradural reflux causing VCM, resulting in overall good clinical outcomes.
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Purpose@#Coil embolization of paraclinoid aneurysms should be simple, safe, and effective considering the benign nature of the aneurysm. Here, we present a microcatheter stabilization technique using a partially inflated balloon for the treatment of paraclinoid aneurysms. @*Materials and Methods@#This retrospective study included 58 patients who underwent balloon-assisted coiling (BAC) for unruptured paraclinoid aneurysms at a tertiary neuro-intervention center between January 2019 and March 2020. We applied a technique to stabilize the microcatheter’s position using the modified BAC technique in paraclinoid aneurysms showing various projections around the ophthalmic curve of the internal carotid artery. The basic concept of the technique is to place a partially inflated balloon just distal to the aneurysm neck and support the distal curve of the microcatheter using the proximal bottom of the balloon. Immediate radiological outcomes were analyzed, and clinical outcomes were evaluated with modified Rankin Scale (mRS) scores. @*Results@#The BAC was successfully performed in 51 of 58 patients (88%). We treated the remaining seven patients by switching to stent-assisted coiling. We obtained a 37% mean packing density resulting in favorable occlusion in all 58 aneurysms (complete occlusion in 35 and residual neck in 23). There were no intraprocedural thromboembolic or hemorrhagic events except one that revealed an asymptomatic infarction after the procedure (1.7%). Magnetic resonance angiography follow-up was performed in 37 patients at an average of 11.8 months, in which 11 minor recurrences (29.7%) were found. There was no major recurrence nor retreatment. The mRS score was 0 in all patients during a mean follow-up of 17.7 months (range, 12–25 months). @*Conclusion@#The modified balloon-assisted coiling technique using a partially inflated balloon was safe and effective and could serve as an option for treating paraclinoid aneurysms.
ABSTRACT
Rare neurovascular diseases (RNVDs) have not been well-recognized in Korea. They involve the central nervous system and greatly affect the patients’ lives. However, these diseases are difficult to diagnose and treat due to their rarity and incurability. We established a list of RNVDs by referring to the previous literature and databases worldwide to better understand the diseases and their current management status. We categorized 68 RNVDs based on their pathophysiology and clinical manifestations and estimated the prevalence of each disease in Korea. Recent advances in genetic, molecular, and developmental research have enabled further understanding of these RNVDs. Herein, we review each disease, while considering its classification based on updated pathologic mechanisms, and discuss the management status of RNVD in Korea.
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Background@#and Purpose Long-term changes in post-stroke depression (PSD), post-stroke emotional incontinence (PSEI), and post-stroke anger (PSA) have rarely been studied. @*Methods@#This is a sub-study of EMOTION, a randomized, placebo-controlled trial, that examined the efficacy of escitalopram on PSD, PSEI, and PSA in patients with stroke. We interviewed patients at the long-term period (LTP) using predefined questionnaires: Montgomery-Åsberg depression rating scale (MADRS) for PSD, modified Kim’s criteria for PSEI, and Spielberger trait anger scale for PSA. Additionally, the ENRICHD Social Support Instrument (ESSI) for the social support state and the modified Rankin Scale (mRS) were measured. We investigated the changes in and factors behind PSD, PSEI, and PSA at LTP. @*Results@#A total of 222 patients were included, and the median follow-up duration was 59.5 months (interquartile range, 50 to 70). Compared to the data at 6 months post-stroke, the prevalence of PSEI (11.7% at 6 months, 6.3% at LTP; P=0.05) and mean anger score (21.62, 16.24; P<0.01) decreased, while the prevalence of PSD (35.6%, 44.6%; P=0.03) and mean MADRS (6.16, 8.67; P<0.01) increased at LTP. ESSI was associated with PSD and PSA, but not with PSEI. The effect of the baseline National Institutes of Health Stroke Scale score on PSD decreased over time. The effect of low social support on PSD was greater than that of mRS at LTP. @*Conclusions@#The prevalence and degree of PSD significantly increased, while those of PSEI and PSA decreased at LTP. PSD in this stage appeared to be more closely associated with a lack of social support than patients' physical disabilities.
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Background@#and Purpose Long-term changes in post-stroke depression (PSD), post-stroke emotional incontinence (PSEI), and post-stroke anger (PSA) have rarely been studied. @*Methods@#This is a sub-study of EMOTION, a randomized, placebo-controlled trial, that examined the efficacy of escitalopram on PSD, PSEI, and PSA in patients with stroke. We interviewed patients at the long-term period (LTP) using predefined questionnaires: Montgomery-Åsberg depression rating scale (MADRS) for PSD, modified Kim’s criteria for PSEI, and Spielberger trait anger scale for PSA. Additionally, the ENRICHD Social Support Instrument (ESSI) for the social support state and the modified Rankin Scale (mRS) were measured. We investigated the changes in and factors behind PSD, PSEI, and PSA at LTP. @*Results@#A total of 222 patients were included, and the median follow-up duration was 59.5 months (interquartile range, 50 to 70). Compared to the data at 6 months post-stroke, the prevalence of PSEI (11.7% at 6 months, 6.3% at LTP; P=0.05) and mean anger score (21.62, 16.24; P<0.01) decreased, while the prevalence of PSD (35.6%, 44.6%; P=0.03) and mean MADRS (6.16, 8.67; P<0.01) increased at LTP. ESSI was associated with PSD and PSA, but not with PSEI. The effect of the baseline National Institutes of Health Stroke Scale score on PSD decreased over time. The effect of low social support on PSD was greater than that of mRS at LTP. @*Conclusions@#The prevalence and degree of PSD significantly increased, while those of PSEI and PSA decreased at LTP. PSD in this stage appeared to be more closely associated with a lack of social support than patients' physical disabilities.
ABSTRACT
Purpose@#Coil embolization of paraclinoid aneurysms should be simple, safe, and effective considering the benign nature of the aneurysm. Here, we present a microcatheter stabilization technique using a partially inflated balloon for the treatment of paraclinoid aneurysms. @*Materials and Methods@#This retrospective study included 58 patients who underwent balloon-assisted coiling (BAC) for unruptured paraclinoid aneurysms at a tertiary neuro-intervention center between January 2019 and March 2020. We applied a technique to stabilize the microcatheter’s position using the modified BAC technique in paraclinoid aneurysms showing various projections around the ophthalmic curve of the internal carotid artery. The basic concept of the technique is to place a partially inflated balloon just distal to the aneurysm neck and support the distal curve of the microcatheter using the proximal bottom of the balloon. Immediate radiological outcomes were analyzed, and clinical outcomes were evaluated with modified Rankin Scale (mRS) scores. @*Results@#The BAC was successfully performed in 51 of 58 patients (88%). We treated the remaining seven patients by switching to stent-assisted coiling. We obtained a 37% mean packing density resulting in favorable occlusion in all 58 aneurysms (complete occlusion in 35 and residual neck in 23). There were no intraprocedural thromboembolic or hemorrhagic events except one that revealed an asymptomatic infarction after the procedure (1.7%). Magnetic resonance angiography follow-up was performed in 37 patients at an average of 11.8 months, in which 11 minor recurrences (29.7%) were found. There was no major recurrence nor retreatment. The mRS score was 0 in all patients during a mean follow-up of 17.7 months (range, 12–25 months). @*Conclusion@#The modified balloon-assisted coiling technique using a partially inflated balloon was safe and effective and could serve as an option for treating paraclinoid aneurysms.
ABSTRACT
Rare neurovascular diseases (RNVDs) have not been well-recognized in Korea. They involve the central nervous system and greatly affect the patients’ lives. However, these diseases are difficult to diagnose and treat due to their rarity and incurability. We established a list of RNVDs by referring to the previous literature and databases worldwide to better understand the diseases and their current management status. We categorized 68 RNVDs based on their pathophysiology and clinical manifestations and estimated the prevalence of each disease in Korea. Recent advances in genetic, molecular, and developmental research have enabled further understanding of these RNVDs. Herein, we review each disease, while considering its classification based on updated pathologic mechanisms, and discuss the management status of RNVD in Korea.