• Journal of Yeungnam Medical Science
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• v.23 no.1
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• pp.113-117
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• 2006

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect many organ systems including the nervous system. The immune response in patients with SLE can cause inflammation and other damage that can cause significant injury to the arteries and tissues. A 48-year-old woman was admitted to the hospital because of transient monocular blindness. Magnetic resonance imaging and conventional angiography showed severe stenosis of the distal intracranial internal carotid artery. The patient was diagnosed as having SLE but the antiphospholipid antibodies were negative. Amaurosis fugax has not been previously reported as an initial manifestation of SLE in Korea. We report a patient with a retinal transient ischemic attack as the first manifestation of SLE.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.199-205
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• 2022

Developmental venous anomalies (DVAs) are common intracranial vascular malformations and they are generally do not cause clinical complications. In cases showing DVA and hemorrhage, the hemorrhage is usually associated with adjacent cavernous malformations. Very few cases of intracerebral hemorrhage (ICH) caused by thrombosis in DVA have been reported in the literature. In this case report, we present an interesting case of a large ICH caused by thrombosis within a DVA with an unusual structure that may have potentiated the thrombosis.

• Journal of Korean Neurosurgical Society
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• v.64 no.5
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• pp.732-739
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• 2021

Objective : Early successful reperfusion is associated with favorable outcomes in acute ischemic stroke (AIS). The purpose of this study was to achieve successful recanalization by a combined mechanical thrombectomy technique, the Aspiration-Retriever Technique for Stroke (ARTS), which is composed of a flexible large lumen distal access catheter and a retrievable stent as the first-line strategy of mechanical thrombectomy. Methods : We retrospectively reviewed 62 patients with AIS who underwent mechanical thrombectomy from 2018 to 2019 at our institute by a senior neurointerventionalist. Among them, patients who were treated using the ARTS technique with the soft torqueable catheter optimized for intracranial access (SOFIA^®; MicroVention-Terumo, Tustin, CA, USA) as the first-line treatment were included. Patients who had tandem occlusions or underlying intracranial artery stenosis were excluded. The angiographic and clinical outcomes were evaluated. The angiographic outcome was analyzed by the rate of successful recanalization, defined as a Thrombolysis in Cerebral Infarction score of 2b or 3 at the end of all procedures and the rate of successfully achieving the first pass effect (FPE), defined as complete recanalization with a single pass of the device. The clinical outcomes included the National Institutes of Health Stroke Scale (NIHSS) score, modified Rankin Scale (mRS), and mortality. Results : A total of 27 patients (mean age, 59.3 years) fulfilled the inclusion criteria. The successful recanalization rate was 96% (n=26) while the FPE rate was 41% (n=11). The mean post-procedural NIHSS change was -3.0. Thirteen patients (48%) showed good clinical outcomes after thrombectomy with the ARTS technique (mRS at 90 days ≤2). Postoperative complications occurred in seven of 25 patients : hemorrhagic transformation in six patients (22%) and distal embolization in one patient (4%). Mortality was 15% (n=4). Conclusion : Although the clinical outcomes using the ARTS technique with a flexible large lumen distal access catheter performed as the frontline thrombectomy in patients with AIS were not significantly superior than those of other studies, this study showed a high rate of successful endovascular recanalization which was comparable to that of other studies. Therefore, ARTS using the SOFIA^® catheter can be considered as the first choice of treatment for AIS due to large vessel occlusion.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.772-775
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• 2003

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries, but the etiology remains unclear. Angiographic characteristics include bilateral stenosis or occlusion of the terminal portions of the intracranial internal carotid arteries and bilateral development of fine collateral vessels at the base of the brain known as ‘Moyamoya vessels’. Cardiac surgery using cardiopulmonary bypass due to coronary artery disease and others among patients with moyamoya disease is very rare, and cardiac surgery for such patients has a potential risk of intraoperative and perioperative brain ischemia. We successfully treated a patient who underwent artrial septal defect closure and coronary artery bypass graft using the cardiopulmonary bypass, so we report this case with a brief literature review.

• Journal of Korean Neurosurgical Society
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• v.54 no.4
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• pp.280-288
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• 2013

Objective : Perfusion computed tomography (PCT) has the ability to measure quantitative value and produce maps of mean transit time (MTT), cerebral blood flow (CBF), and cerebral blood volume (CBV). We assessed cerebral hemodynamics by using these parameters and acetazolamide (ACZ) challenge for pre- and post-procedural evaluation in patients with unilateral cerebrovascular stenotic disease. Methods : Thirty patients underwent pre-procedural PCT with ACZ challenge, and 24 patients (80%) was conducted follow up PCT after angioplasty with same protocol. The mean MTT, CBF, and CBV were measured and compared in both middle cerebral arterial (MCA) territories before and after ACZ challenge. Hemispheric ratio and percent change after ACZ challenge were calculated before and after angioplasty. Results : The mean stenosis rate was 76.6%. Significant increases in MTT (32.6%, p=0.000) and significant decreases in CBF (-14.2%, p=0.000) were found in stenotic side MCA territories. After ACZ challenge, there were significant changes in MTT (37.4%, p=0.000), CBF (-13.1%, p=0.000), and CBV (-10.5%, p=0.001) in pre-procedural perfusion study. However, no significant increases were found in MTT, or decreases in CBF and CBV in post-procedural study. There were no significant changes after ACZ challenge also. In addition, the degrees of these changes (before and after ACZ challenge) were highly correlated with the stenotic degrees in pre-procedural perfusion study. Conclusion : PCT with ACZ challenge appears to be a useful tool to assess the cerebral perfusion status especially in patients with unilateral symptomatic stenotic disease.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• Journal of Korean Neurosurgical Society
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• v.67 no.2
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• pp.158-165
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• 2024

Objective : Superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis is conducted for flow augmentation. In this study, we measured the STA cut flow of a Korean population and evaluated the relationship between STA cut flow and long-term patency of the bypass. Methods : A retrospective study was conducted. Intraoperative measurement of STA flow was conducted using a microvascular flow meter on patients who underwent STA-MCA. After cutting the distal end, the STA flow rate was measured with no resistance and recorded. After finishing anastomosis, STA flow was measured and recorded. The cut flow index was calculated by dividing post anastomosis flow by cut flow in intracranial atherosclerotic stenosis patients. Results : The median STA cut flow was 35.0 mL/min and the post anastomosis flow was 24.0 mL/min. The cut flow of STA decreased with aging (p=0.027) and increased with diameter (p=0.004). The cut flow showed no correlation with history of hypertension or diabetes mellitus (p=0.713 and p=0.786), but did correlate a positively with history of hyperlipidemia (p=0.004). There were no statistical differences in cut flow, STA diameter, and post anastomosis flow between the frontal and parietal branches (p=0.081, p=0.853, and p=0.990, respectively). Conclusion : The median STA cut flow of a Korean population was 35 mL/min. Upon reviewing previous articles, it appears that there are differences in the STA cut flow between Western and Asian patients.

• Journal of Korean Neurosurgical Society
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• v.30 no.6
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• pp.805-812
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• 2001

Objectives : The goal of the surgical management of large and giant aneurysm is complete extirpation of the aneurysms with preservation or reconstruction of the parent artery. To improve the surgical management results of those aneurysms in the future, we review our experience and discuss technical maneuvers and strategies used to avoid potential complications of those aneurysm surgery. Material and Methods : During the past 12 years, thirty six cases of large and giant aneurysms(diameter>19mm) were managed by surgery. The clinical characteristics, treatment methods, surgical complications and outcome of those cases were analyzed and, based on the review of the literatures, the preventive methods of surgical complication related to the clipping of those aneurysms were discussed. Results : The locations of those aneurysms were anterior circulation in 34 cases and posterior circulation in 2 cases. The most frequent site of aneurysmal location was a paraclinoidal region of the anterior circulation. The aneurysms were managed surgically by direct clipping of aneurysmal neck in 31 cases, aneurysmal trapping followed by extracranial-intracranial bypass in 2 cases, proximal clipping of parent artery, aneurysmorrhaphy, and excision of aneurysm followed by end to end anastomosis of parent artery in each one case. Surgical complications occurred in 13 cases. A parent vessel occlusion by thrombus formation and parent vessel stenosis after clipping of aneurysm were the main complications. We obtained good outcome in 27, fair 5, poor 1 and dead in 3 case(s). Conclusion : We conclude that selection of suitable management method for each case, high quality of surgical technique and prevention of complication during operation are important key points for the successful treatment of large and giant aneurysm. The heparinization prior to application of temporary clip on parent vessels, aneurysmal decompression during dissection and clipping of aneurysm, complete closing of the aneurysmal neck and avoiding the narrowing of parent vessel after clipping of aneurysm were the main technical maneuvers used to avoid complications of those aneurysm surgery.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.97-103
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• 2005

Hypereosinophilic syndrome (HES) is characterized by a sustained eosinophilia of $1,500/mm^3$ or more in the absence of any known causes or the signs and symptoms of organ involvement. We report a 64-year-old man with HES initially presenting with involvement of the liver and bone marrow. Despite controlling the eosinophilia by corticosteroid, he developed a cerebral infarction and later progressive interstitial pneumonia. Brain angiography revealed a severe stenosis of the proximal right internal carotid artery (ICA) and a complete obstruction of the intracranial ICA. An open lung biopsy revealed fibrosis and lymphoplasma cell infiltration without eosinophils, which were consistent with nonspecific interstitial pneumonia.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.91-94
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• 2003

Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial calcifications in the particularly periventricular area. It undergoes an autosomal recessive inheritance. Twelve cases of Raine syndrome have been reported in the literature. However, a case of Raine syndrome in Korea has not been reported yet. Therefore, we describe a female newborn with Raine syndrome with a brief review of the literatures.