A better understanding of moyamoya disease (MMD), such as natural clinical course, surgical outcomes and research, has been obtained. This review article focuses on an giving an update for adult MMD in the Korean population. In this paper, we mainly discuss the results of our domestic investigations including meta-analysis, and related subjects from other countries.
Adult* ; Hemorrhage ; Humans ; Ischemia ; Korea ; Moyamoya Disease*

PURPOSE: To assess clinical and angiographic outcomes after endovascular treatment (EVT) in ischemic stroke patients according to anesthesia types (general anesthesia vs. conscious sedation). MATERIALS AND METHODS: A systematic literature review through an online data base between January 1990 and September 2017 was performed. A fixed effect model was used in cases of <50% heterogeneity. The primary outcomes were good clinical outcome at the 3-month follow-up and successful recanalization. A meta-regression analysis was done to estimate primary outcomes of log odds ratio (OR) on onset-to-puncture time (OTP) differences. Publication bias was determined using Begg’s funnel plot and additional the Trim and Fill method. RESULTS: Sixteen articles including 2,662 patients (general anesthesia, n=1,275; conscious sedation, n=1,387) were included. General anesthesia significantly decreased good outcomes than conscious sedation (OR, 0.564; 95% confidence interval [CI], 0.354–0.899). However, outcomes did not differ significantly in randomized controlled trials (RCTs; OR, 1.101; 95% CI, 0.395–3.071). Anesthesia type was not associated with successful recanalization (OR, 0.985; 95% CI, 0.787–1.233). General anesthesia increased the risk of mortality (OR, 1.532; 95% CI, 1.187–1.976) and pneumonia (OR, 1.613; 95% CI, 1.172–2.221), but not symptomatic intracranial hemorrhage (OR, 1.125; 95% CI, 0.767–1.652). The meta-regression analysis showed no linear relationship between OTP differences and log OR of good outcome (coefficient, 0.0004; P=0.95) or successful recanalization (coefficient, 0.0005; P=0.94), respectively. CONCLUSION: General anesthesia seemed to be associated with adverse clinical outcome after EVT. However, its efficacy was not demonstrated in RCTs. Successful recanalization did not differ according to anesthesia type. Studies using individual patient data based on further RCTs are necessary to elucidate anesthesia effect on procedural and clinical outcomes.
Anesthesia ; Anesthesia, General ; Conscious Sedation ; Follow-Up Studies ; Humans ; Intracranial Hemorrhages ; Methods ; Mortality ; Odds Ratio ; Pneumonia ; Population Characteristics ; Publication Bias ; Stroke

OBJECTIVE: Endovascular treatment (EVT) outcomes for acute M2 segment of middle cerebral artery occlusion remains unclear because most results are obtained from patients with large artery occlusion in the anterior circulation. The objective of this study was to assess procedural outcomes for acute M2 occlusion and compare outcomes according to thrombus location (M1 vs. M2).METHODS: A systematic review was performed for online literature published from January 2004 to December 2016. Primary outcome was successful recanalization rate and symptomatic intracranial hemorrhage (S-ICH) after the procedure. A fixed effect model was used if heterogeneity was less than 50%.RESULTS: Eight articles were included. EVT showed successful recanalization rate of 69.1% (95% confidence interval [CI], 54.9–80.4%) and S-ICH rate of 6.1% (95% CI, 4.5–8.3%). The rates of good clinical outcome at 3 months and mortality were 59.4% (95% CI, 49.9–68.2%) and 14.9% (95% CI, 11.4–19.3%), respectively. According to thrombus location (M1 vs. M2), successful recanalization (odds ratio [OR], 1.539; 95% CI, 0.293–8.092; p=0.610) and S-ICH (OR, 1.313; 95% CI, 0.603–2.861; p=0.493) did not differ significantly. Good clinical outcome was more evident in M2 occlusion after EVT than that in M1 occlusion (OR, 1.639; 95% CI, 1.135–2.368; p=0.008). However, mortality did not differ significantly according to thrombus location (OR, 0.788; 95% CI, 0.486–1.276; p=0.332).CONCLUSION: EVT seems to be technically feasible for acute M2 occlusion. Direct comparative studies between EVT and medical treatment are needed further to find specific beneficiary group after EVT in patient with M2 occlusion.
Arteries ; Humans ; Infarction ; Infarction, Middle Cerebral Artery ; Intracranial Hemorrhages ; Middle Cerebral Artery ; Mortality ; Population Characteristics ; Stroke ; Thrombectomy ; Thrombosis

No abstract available.
Aspergillus* ; Sinusitis*

The close vicinity of the optic nerve to the sphenoid sinus may cause visual loss in the sphenoid fungus ball. We present a case of blindness secondary to sphenoid fungus ball without any evidence of orbital invasion in imaging studies. A 61-year-old man, suffering from uncomplicated diabetes, was referred for right visual loss that developed 1 day ago. He perceived hand motion on the right. CT and MRI revealed a fungus ball in the right sphenoid sinus. However, there was no evidence of orbital invasion. Endoscopic sphenoethmoidectomy was performed to remove the fungus ball. Systemic mega-dose steroid and amphotericin B were started because he lost the light perception 3 days after surgery. Biopsy revealed aspergillus fungus ball and no evidence of mucosal invasion. However, blindness was not reversed. Evidence of orbital invasion in imaging diagnosis is elusive in sphenoid fungus ball; therefore, systemic antifungal treatment should be initiated and early endoscopic sphenoidotomy should be performed in case of rapidly progressing visual loss, especially in diabetic or immunocompromised patients. Mega-dose steroid therapy for optic neuropathy should be selective because it may aggravate underlying systemic diseases to cause early termination of systemic antifungal treatment.
Amphotericin B ; Aspergillus ; Biopsy ; Blindness ; Fungi ; Hand ; Humans ; Immunocompromised Host ; Light ; Middle Aged ; Optic Nerve ; Optic Nerve Diseases ; Orbit ; Sphenoid Sinus ; Stress, Psychological

Simultaneous or subsequent bilateral thalamic hemorrhagic events have ranged from 12 to 19 in prior reports, with a time lag between bilateral thalamic hemorrhage of up to two days. Herein, we report the first case of delayed (17 days) consecutive contralateral thalamic hemorrhage after spontaneous first thalamic hemorrhage. A 65-year-old female initially presented with a drowsy mentality with a left-side motor weakness (grade II/III). Brain computed tomography (CT) demonstrated right side thalamic and intraventricular hemorrhage. She regained alertness with mild residual motor weakness (grade III/IV) under medical management. Seventeen days later, a sudden and generalized tonic-clonic seizure developed. Brain CT scans revealed a new contralateral thalamic hemorrhage coincident with microbleeds. Neurologic status remained unchanged, consisting of a stuporous mentality with quadriparesis of grade II/II. We report the first case of delayed consecutive contralateral thalamic hemorrhage up to 17 days after first thalamic hemorrhage. The case highlights the need for close monitoring of patients with thalamic hemorrhage who experience microbleeds on the contralateral side, due to the possibility of delayed hemorrhage.
Aged ; Brain ; Female ; Hemorrhage* ; Humans ; Hypertension ; Intracranial Hemorrhages ; Quadriplegia ; Seizures ; Stupor ; Thalamus ; Tomography, X-Ray Computed

Simultaneous or subsequent bilateral thalamic hemorrhagic events have ranged from 12 to 19 in prior reports, with a time lag between bilateral thalamic hemorrhage of up to two days. Herein, we report the first case of delayed (17 days) consecutive contralateral thalamic hemorrhage after spontaneous first thalamic hemorrhage. A 65-year-old female initially presented with a drowsy mentality with a left-side motor weakness (grade II/III). Brain computed tomography (CT) demonstrated right side thalamic and intraventricular hemorrhage. She regained alertness with mild residual motor weakness (grade III/IV) under medical management. Seventeen days later, a sudden and generalized tonic-clonic seizure developed. Brain CT scans revealed a new contralateral thalamic hemorrhage coincident with microbleeds. Neurologic status remained unchanged, consisting of a stuporous mentality with quadriparesis of grade II/II. We report the first case of delayed consecutive contralateral thalamic hemorrhage up to 17 days after first thalamic hemorrhage. The case highlights the need for close monitoring of patients with thalamic hemorrhage who experience microbleeds on the contralateral side, due to the possibility of delayed hemorrhage.
Aged ; Brain ; Female ; Hemorrhage* ; Humans ; Hypertension ; Intracranial Hemorrhages ; Quadriplegia ; Seizures ; Stupor ; Thalamus ; Tomography, X-Ray Computed

BACKGROUND AND OBJECTIVES Vestibular neuritis (VN) is one of critical life events that can affect physical, emotional, and function aspects of quality of life. Most patients recover well from VN within 6 months following the onset of the disease. However, they can still interfere with their daily lives in some patient with VN. Dizziness Handicap Inventory (DHI) was developed to assess the self-perceived handicapping effects imposed by vestibular system diseases. The aim of study was to evaluate subjective symptoms among the pre-VN, at the time of onset of VN, and post-VN using DHI questionnaire, respectively. MATERIALS AND METHODS Twenty patients with VN were asked to complete the DHI by mailed survey. Each of the DHI was scored. We also evaluated the relationship between the DHI scores and degree of the canal paresis on the caloric test. RESULTS Almost every patients had substantially improved its subjective symptoms in 6 months after VN. There was no correlation between the canal paresis in the lesion ear and DHI scores at the time of VN. CONCLUSION The results of this study suggest that most patients recovery well from VN without any handicap, but the emotional support in combination with physical and/or functional rehabilitation should be required to provide early resumption of normal activity
Dizziness ; Ear ; Humans ; Paresis ; Postal Service ; Quality of Life ; Surveys and Questionnaires ; Vertigo ; Vestibular Neuronitis

Objective: Conflicting results regarding SOX17 genes and the risk of intracranial aneurysms (IA) exist in the Korean population, although significant positive correlations were noted in genome-wide association studies in European and Japanese populations. Therefore, we aimed to investigate an association between SOX17 gene variants and IA using exome sequencing data. Methods: This study included 26 age-gender matched IA patients and 26 control subjects. The SOX17 gene variants identified from whole-exome sequencing data were examined. Genetic associations to estimate odds ratio (OR) and 95% confidence interval (CI) were performed using the software EPACTS. Results: The mean age of the IA and control groups were 51.0±9.3 years and 49.4±14.3 years, respectively (p=0.623). Seven variants of SOX17, including six single nucleotide polymorphisms and one insertion and deletion, were observed. Among these variants, rs12544958 (A>G) showed the most association with IA, but the association was not statistically significant (OR, 1.97; 95% CI, 0.81-4.74; p=0.125). Minor allele frequencies of the IA patients and controls were 0.788 and 0.653, respectively. None of the remaining variants were significantly associated with IA formation. Conclusion No significant association between SOX17 gene variants and IA were noted in the Korean population. A large-scale exome sequencing study is necessary to investigate any Korean-specific genetic susceptibility to IA.

OBJECTIVE: Elevated cellular retinoic acid binding protein-I (CRABP-I) is thought to be related to the abnormal proliferation and migration of smooth muscle cells (SMCs). Accordingly, a higher CRABP-I level could cause disorganized vessel walls by causing immature SMC phenotypes and altering extracellular matrix proteins which could result in vulnerable arterial walls with inadequate responses to hemodynamic stress. We hypothesized that elevated CRABP-I level in the cerebrospinal fluid (CSF) could be related to subarachnoid hemorrhage (SAH). Moreover, we also extended this hypothesis in patients with vascular malformation according to the presence of hemorrhage. METHODS: We investigated the CSF of 26 patients : SAH, n=7; unruptured intracranial aneurysm (UIA), n=7; arteriovenous malformation (AVM), n=4; cavernous malformation (CM), n=3; control group, n=5. The optical density of CRABP-I was confirmed by Western blotting and presented as mean+/-standard error of the measurement. RESULTS: CRABP-I in SAH (0.33+/-0.09) was significantly higher than that in the UIA (0.12+/-0.01, p=0.033) or control group (0.10+/-0.01, p=0.012). Hemorrhage presenting AVM (mean 0.45, ranged 0.30-0.59) had a higher CRABP-I level than that in AVM without hemorrhage presentation (mean 0.16, ranged 0.14-0.17). The CRABP-I intensity in CM with hemorrhage was 0.21 and 0.31, and for CM without hemorrhage 0.14. Overall, the hemorrhage presenting group (n=11, 0.34+/-0.06) showed a significantly higher CRABP-I intensity than that of the non-hemorrhage presenting group (n=10, 0.13+/-0.01, p=0.001). CONCLUSION: The results suggest that elevated CRABP-I in the CSF could be related with aneurysm rupture. Additionally, a higher CRABP-I level seems to be associated with hemorrhage development in vascular malformation.
Aneurysm ; Arteriovenous Malformations ; Blotting, Western ; Cerebrospinal Fluid* ; Extracellular Matrix Proteins ; Hemodynamics ; Hemorrhage ; Humans ; Intracranial Aneurysm ; Myocytes, Smooth Muscle ; Phenotype ; Rupture ; Subarachnoid Hemorrhage ; Tretinoin* ; Vascular Malformations