Improving the Prognosis of Patients With Acute Ischemic Stroke Treated in the Late Time Window After the Introduction of Advanced Imaging Software: Benefits From Thrombectomy in the Extended Time Window

Background@#Mechanical thrombectomy (MT) of ischemic stroke was recommended as a clinical guideline in 2015, and the indication for time was expanded in 2018 based on two clinical studies. We aimed to compare and analyze the prognosis of patients treated under the extended time indication before and after the introduction of advanced software. @*Methods@#We obtained data from medical records between 2016 to 2020. From 2016 to 2017, patients who did not receive MT who visited the hospital within 24 hours from the last normal time (LNT) were classified as standard medical treatment (SMT) group. Among patients who underwent MT between 2019 and 2020, patients who visited the hospital between 6-24 hours from the LNT were classified into the extended MT (EMT) group. Good outcome was defined as 3-months modified rankin scale (mRS) ≤ 2, and a poor outcome as mRS ≥ 4. @*Results@#From 2016 to 2017, 1,058 patients were hospitalized for ischemic stroke, of which 60 (5.7%) received MT, and 27 patients were classified into the SMT group. Among 1,019 patients between 2019 and 2020, 85 (8.3%) received MT, and 24 patients were in the EMT group. Among the SMT group, only 3 had a good prognosis, and 24 (88.9%) had a poor prognosis. However, in the EMT group, 10 (41.7%) had a good prognosis, and 9 (37.5%) had a poor prognosis. The SMT group had a 49.1 times higher risk of poor prognosis compared to the EMT group (P = 0.008). @*Conclusion@#The number of patients with ischemic stroke who receive MT has increased by using advanced imaging software. It was confirmed that patients treated based on the extended time indication also had a good prognosis.

Global Impact of the COVID-19 Pandemic on Cerebral Venous Thrombosis and Mortality / 대한뇌졸중학회지

Background@#and Purpose Recent studies suggested an increased incidence of cerebral venous thrombosis (CVT) during the coronavirus disease 2019 (COVID-19) pandemic. We evaluated the volume of CVT hospitalization and in-hospital mortality during the 1st year of the COVID-19 pandemic compared to the preceding year. @*Methods@#We conducted a cross-sectional retrospective study of 171 stroke centers from 49 countries. We recorded COVID-19 admission volumes, CVT hospitalization, and CVT in-hospital mortality from January 1, 2019, to May 31, 2021. CVT diagnoses were identified by International Classification of Disease-10 (ICD-10) codes or stroke databases. We additionally sought to compare the same metrics in the first 5 months of 2021 compared to the corresponding months in 2019 and 2020 (ClinicalTrials.gov Identifier: NCT04934020). @*Results@#There were 2,313 CVT admissions across the 1-year pre-pandemic (2019) and pandemic year (2020); no differences in CVT volume or CVT mortality were observed. During the first 5 months of 2021, there was an increase in CVT volumes compared to 2019 (27.5%; 95% confidence interval [CI], 24.2 to 32.0; P<0.0001) and 2020 (41.4%; 95% CI, 37.0 to 46.0; P<0.0001). A COVID-19 diagnosis was present in 7.6% (132/1,738) of CVT hospitalizations. CVT was present in 0.04% (103/292,080) of COVID-19 hospitalizations. During the first pandemic year, CVT mortality was higher in patients who were COVID positive compared to COVID negative patients (8/53 [15.0%] vs. 41/910 [4.5%], P=0.004). There was an increase in CVT mortality during the first 5 months of pandemic years 2020 and 2021 compared to the first 5 months of the pre-pandemic year 2019 (2019 vs. 2020: 2.26% vs. 4.74%, P=0.05; 2019 vs. 2021: 2.26% vs. 4.99%, P=0.03). In the first 5 months of 2021, there were 26 cases of vaccine-induced immune thrombotic thrombocytopenia (VITT), resulting in six deaths. @*Conclusions@#During the 1st year of the COVID-19 pandemic, CVT hospitalization volume and CVT in-hospital mortality did not change compared to the prior year. COVID-19 diagnosis was associated with higher CVT in-hospital mortality. During the first 5 months of 2021, there was an increase in CVT hospitalization volume and increase in CVT-related mortality, partially attributable to VITT.

Care Process of Recanalization Therapy for Acute Stroke during the COVID-19 Outbreak in South Korea

Background@#and Purpose We aimed to determine whether the care process and outcomes in patients with acute stroke who received recanalization therapy changed during the outbreak of coronavirus disease 2019 (COVID-19) in South Korea. @*Methods@#We used data from a prospective multicenter reperfusion therapy registry to compare the care process including the time from symptom onset to treatment, number of treated patients, and discharge disposition and treatment outcomes between before and during the COVID-19 outbreak in South Korea. @*Results@#Upon the COVID-19 outbreak in South Korea, the number of patients receiving endovascular treatment to decrease temporarily but considerably. The use of emergency medical services by stroke patients increased from 91.5% before to 100.0% during the COVID-19 outbreak (p=0.025), as did the median time from symptom onset to hospital visit [median (interquartile range), 91.0 minutes (39.8–277.0) vs. 176.0 minutes (56.0–391.5), p=0.029]. Furthermore, more functionally dependent patients with disabilities were discharged home (59.5% vs. 26.1%, p=0.020) rather than staying in a regional or rehabilitation hospital. In contrast, there were no COVID-19-related changes in the times from the hospital visit to brain imaging and treatment or in the functional outcome, successful recanalization rate, or rate of symptomatic intracerebral hemorrhage. @*Conclusions@#These findings suggest that a prehospital delay occurred during the COVID-19 outbreak, and that patients with acute stroke might have been reluctant to visit and stay in hospitals. Our findings indicate that attention should be paid to prehospital care and the behavior of patients with acute stroke during the COVID-19 outbreak.

Prediction of Early Recanalization after Intravenous Thrombolysis in Patients with Large-Vessel Occlusion / 대한뇌졸중학회지

Background@#and Purpose We aimed to develop a model predicting early recanalization after intravenous tissue plasminogen activator (t-PA) treatment in large-vessel occlusion. @*Methods@#Using data from two different multicenter prospective cohorts, we determined the factors associated with early recanalization immediately after t-PA in stroke patients with large-vessel occlusion, and developed and validated a prediction model for early recanalization. Clot volume was semiautomatically measured on thin-section computed tomography using software, and the degree of collaterals was determined using the Tan score. Follow-up angiographic studies were performed immediately after t-PA treatment to assess early recanalization. @*Results@#Early recanalization, assessed 61.0±44.7 minutes after t-PA bolus, was achieved in 15.5% (15/97) in the derivation cohort and in 10.5% (8/76) in the validation cohort. Clot volume (odds ratio [OR], 0.979; 95% confidence interval [CI], 0.961 to 0.997; P=0.020) and good collaterals (OR, 6.129; 95% CI, 1.592 to 23.594; P=0.008) were significant factors associated with early recanalization. The area under the curve (AUC) of the model including clot volume was 0.819 (95% CI, 0.720 to 0.917) and 0.842 (95% CI, 0.746 to 0.938) in the derivation and validation cohorts, respectively. The AUC improved when good collaterals were added (derivation cohort: AUC, 0.876; 95% CI, 0.802 to 0.950; P=0.164; validation cohort: AUC, 0.949; 95% CI, 0.886 to 1.000; P=0.036). The integrated discrimination improvement also showed significantly improved prediction (0.097; 95% CI, 0.009 to 0.185; P=0.032). @*Conclusions@#The model using clot volume and collaterals predicted early recanalization after intravenous t-PA and had a high performance. This model may aid in determining the recanalization treatment strategy in stroke patients with large-vessel occlusion.

Prediction of Early Recanalization after Intravenous Thrombolysis in Patients with Large-Vessel Occlusion / 대한뇌졸중학회지

Background@#and Purpose We aimed to develop a model predicting early recanalization after intravenous tissue plasminogen activator (t-PA) treatment in large-vessel occlusion. @*Methods@#Using data from two different multicenter prospective cohorts, we determined the factors associated with early recanalization immediately after t-PA in stroke patients with large-vessel occlusion, and developed and validated a prediction model for early recanalization. Clot volume was semiautomatically measured on thin-section computed tomography using software, and the degree of collaterals was determined using the Tan score. Follow-up angiographic studies were performed immediately after t-PA treatment to assess early recanalization. @*Results@#Early recanalization, assessed 61.0±44.7 minutes after t-PA bolus, was achieved in 15.5% (15/97) in the derivation cohort and in 10.5% (8/76) in the validation cohort. Clot volume (odds ratio [OR], 0.979; 95% confidence interval [CI], 0.961 to 0.997; P=0.020) and good collaterals (OR, 6.129; 95% CI, 1.592 to 23.594; P=0.008) were significant factors associated with early recanalization. The area under the curve (AUC) of the model including clot volume was 0.819 (95% CI, 0.720 to 0.917) and 0.842 (95% CI, 0.746 to 0.938) in the derivation and validation cohorts, respectively. The AUC improved when good collaterals were added (derivation cohort: AUC, 0.876; 95% CI, 0.802 to 0.950; P=0.164; validation cohort: AUC, 0.949; 95% CI, 0.886 to 1.000; P=0.036). The integrated discrimination improvement also showed significantly improved prediction (0.097; 95% CI, 0.009 to 0.185; P=0.032). @*Conclusions@#The model using clot volume and collaterals predicted early recanalization after intravenous t-PA and had a high performance. This model may aid in determining the recanalization treatment strategy in stroke patients with large-vessel occlusion.

Comparison of Long Term Prognosis between Carotid Endarterectomy versus Stenting; A Korean Population-Based Study Using National Insurance Data / 신경중재치료의학

PURPOSE: Although carotid endarterectomy (CEA) is recommended as a treatment for carotid stenosis rather than carotid artery stenting (CAS), CAS has been preferred in Korea. The aim of this study was to analyze long-term outcomes after CAS compared with CEA using Korean nationwide insurance data. MATERIALS AND METHODS: We obtained all data from the nationwide database of the Health Insurance Review & Assessment Service (HIRA) during the study period using several codes regarding the procedure or operation. We included the HIRA data, which included at least one-year follow-up after the procedures. The outcomes associated with both procedures were death, recurrence of ischemic stroke, and admission for cerebral hemorrhage. RESULTS: A total of 16,065 eligible patients who were treated with CAS or CEA between 1 January 2007 and 31 December 2016 were analyzed. The number of patients with CAS and CEA was 12,173 (75.8%) and 3,892 (24.2%), respectively. 8,976 patients (55.9%) were classified as symptomatic patients. CAS was associated with a higher risk of all-cause mortality (adjusted hazard ratio [HR], 1.282; 95% confidence interval [CI], 1.173–1.400). The adjusted rates for recurrent ischemic stroke and cerebral hemorrhage between CAS versus CEA were 24.9% versus 15.9% (HR, 1.474; 95% CI, 1.325–1.639) and 1.5% versus 0.9% (HR, 2.026; 95% CI, 1.322–3.106), respectively. In young symptomatic patients, there was no statistically significant difference in all-cause mortality and cardiovascular death between CAS and CEA. CONCLUSION: Our study using Korean nationwide insurance data demonstrated similar results to previous studies. Until further evidence of CAS is established through prospective studies, CAS should be performed in selected patients according to current guidelines.

Endovascular Treatment in Acute Ischemic Stroke: A Nationwide Survey in Korea / 신경중재치료의학

PURPOSE: As endovascular therapy (EVT) has been approved as a treatment guideline for acute ischemic stroke (AIS), it has been increasing in Korea. We conducted a nationwide survey to evaluate the current status of EVT for patients with AIS in Korea. MATERIALS AND METHODS: An electronic survey was sent to a representative clinician at each hospital where EVT was available in Korea. A Google survey was used to distribute the questionnaires and receive responses from October 2017 to December 2017. RESULTS: Among 120 hospitals in our society, 76 participated in this survey. In 26% of the hospitals, more than 50 annual cases of EVT were performed, and 25–50 annual cases in 37%. Fifty-six hospitals (73.7%) achieved successful recanalization (thrombolysis in cerebral infarction ≥2b) in more than 80% of patients. Computed tomography (CT) angiography was the most common imaging modality for AIS treatment, and magnetic resonance (MR) diffusion, MR-perfusion/diffusion, and perfusion CT were frequently used in order. Non-eligibility criteria for EVT included a National Institutes of Health Stroke Scale < 4 (64.5%), the absence of MR perfusion/diffusion mismatch (52.6%), and a low Alberta Stroke Program Early CT Score (42.1%). For anterior circulation stroke, 60% of Korean hospitals adopted a wider time range of “8 hours” from symptom onset, while 70% of hospitals had a time limitation of 12–24 hours for posterior circulation stroke. The most preferred EVT device was a stentriever (89.4%). In the failed cases due to underlying stenosis, 79% of Korean hospitals performed angioplasty or stenting for revascularization. CONCLUSION: This first nationwide survey showed that most Korean hospitals conducted EVT for AIS patients according to the present guideline in the era of mechanical thrombectomy by integrating the clinical experiences of many medical institutions and specialists.

Association of Alzheimer's Disease with the Risk of Developing Epilepsy: a 10-Year Nationwide Cohort Study

BACKGROUND AND PURPOSE: Previous studies have reported conflicting results about the prevalence of seizures in Alzheimer's disease (AD). There are few epidemiological studies on this topic in Asia. Thus, the objective of this study was to examine demographic and clinical characteristics as well as incidence for seizures in AD patients compared to non-AD patients in a prospective, longitudinal, community-based cohort with a long follow-up. METHODS: Data were collected from National Health Insurance Service-National Elderly Cohort (NHIS-elderly) Database to define patients with AD from 2004–2006 using Korean Classification Diseases codes G30 and F00. We performed a 1:5 case-control propensity score matching based on age, sex, and household income. We conducted Cox proportional hazards regression analysis to estimate the risk of epilepsy in AD patients. RESULTS: In the cohort study, patients with AD had higher risk for epilepsy than those without AD, with hazard ratio of 2.773 (95% confidence interval [CI], 2.515–3.057). This study also showed that male gender and comorbidities such as hypertension, hyperlipidemia, diabetes, and chronic kidney disease increased the risk of developing epilepsy. Patients with AD had 1.527 (95% CI, 1.375–1.695) times higher mortality rate than those in the control group. CONCLUSIONS: AD patients have significantly higher risk of developing epilepsy than non-AD patients.

Secondary Degeneration of the Ipsilateral Thalamus and Substantia Nigra Following Cerebral Infarction in the Striatum

Secondary degeneration after ischemic stroke has been demonstrated by computed tomography and magnetic resonance imaging. We report a 77-year-old man with striatal infarction followed by multifocal degeneration that developed in a stepwise manner at the ipsilateral substantia nigra and thalamus on diffusion-weighted images obtained at 4 weeks, 6 weeks and 20 weeks after onset. We also review the underlying pathophysiology and its clinical meanings.

Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease: Correlation with Clinical Severity and Old Brain Lesions

PURPOSE: Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. MATERIALS AND METHODS: FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. RESULTS: TIS was significantly different as follows: 4.86+/-2.55 in patients with nonspecific symptoms, 5.89+/-3.10 in patients with single TIA, 9.60+/-3.98 in patients with recurrent TIA and 8.37+/-3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35+/-4.22 vs. 7.49+/-3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). CONCLUSION: Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.

A Protocol-Based Decision for Choosing a Proper Surgical Treatment Option for Carotid Artery Stenosis

OBJECTIVE: There are two established surgical treatment options for carotid artery stenosis. Carotid endarterectomy (CEA) has been accepted as a gold standard for surgical treatment while carotid artery stenting (CAS) has recently become an alternative option. Each treatment option has advantages and disadvantages for the treatment outcomes. We propose a protocol for selection of a proper surgical treatment option for carotid artery stenosis. MATERIALS AND METHODS: A total of 192 published articles on management of carotid artery stenosis were reviewed. Preoperatively considerable factors which had been repeatedly noted in those articles for the risk/benefits of CEA or CAS were selected. According to those factors, a protocol with four categories was established. RESULTS: CEA or CAS is indicated when the patient has a symptomatic stenosis > or = 50%, or when the patient has an asymptomatic stenosis > or = 80%. Each treatment option has absolute indications and favorable indications. Each absolute indication is scored with three points, and each favorable indication, one point. Based on the highest scores, a proper treatment option (CEA or CAS) is selected. CONCLUSION: We have been treating patients according to this protocol and evaluating the outcomes of our protocol-based decision because this protocol might be helpful in assessment of risk/benefit for selection of a proper surgical treatment option in patients with carotid artery stenosis.

Delayed Cerebral Infarction due to Stent Folding Deformation Following Carotid Artery Stenting

We report a case of delayed cerebral infarction due to stent longitudinal folding deformation following carotid artery stenting using a self-expandable stent with an open-cell design. The stented segment of the left common carotid artery was divided into two different lumens by this folding deformation, and the separated lumens became restricted with in-stent thrombosis. Although no established method of managing this rare complication exists, a conservative approach was taken with administration of anticoagulant and dual antiplatelet therapy. No neurological symptoms were observed during several months of clinical follow-up after discharge.

Antiplatelet Effect of Clopidogrel Can Be Reduced by Calcium-Channel Blockers

PURPOSE: Clopidogrel is metabolized by the hepatic cytochrome P450 (CYP) system into its active thiol metabolite. CYP3A4 is involved in the metabolism of both clopidogrel and dihydropyridine calcium channel blockers (CCBs). A few reports have suggested an inhibitory interaction between CCBs and clopidogrel. Accordingly, the aim of this study was to determine the effect of CCBs on the antiplatelet activity of clopidogrel by serial P2Y12 reaction unit (PRU) measurements. MATERIALS AND METHODS: We assessed changes in antiplatelet activity in patients receiving both clopidogrel and CCBs for at least 2 months prior to enrollment in the study. The antiplatelet activity of clopidogrel was measured by VerifyNow P2Y12 assay in the same patient while medicated with CCBs and at 8 weeks after discontinuation of CCBs. After discontinuation of the CCBs, angiotensin receptor blockers were newly administered to the patients or dosed up for control of blood pressure. RESULTS: Thirty patients finished this study. PRU significantly decreased after discontinuation of CCBs (238.1+/-74.1 vs. 215.0+/-69.3; p=0.001). Of the 11 patients with high post-treatment platelet reactivity to clopidogrel (PRU> or =275), PRU decreased in nine patients, decreasing below the cut-off value in seven of these nine patients after 8 weeks. Decrease in PRU was not related to CYP2C19 genotype. CONCLUSION: CCBs inhibit the antiplatelet activity of clopidogrel.

Serial Changes of Fluid-Attenuated Inversion Recovery Hyperintense Vessels in a Patient With Middle Cerebral Artery Stenosis

No abstract available.

Recanalization of Acute Intracranial Artery Occlusion Using Temporary Endovascular Bypass Technique / 신경중재치료의학

PURPOSE: The purpose of this study is to present our preliminary experience of the temporary endovascular bypass (TEB) technique using an Enterprise stent for recanalization of acute intracranial artery (IA) occlusion. MATERIALS AND METHODS: Patients treated by TEB were enrolled in this retrospective study from January 2009 to May 2010. All the procedures consist of temporary partial deployment and subsequent retrieval of Enterprise stent, supplemented by intra-arterial infusion of urokinase (UK) and/or tirofiban. According to the thrombolysis in cerebral infarction (TICI) classification, recanalization was evaluated with initial and postprocedural angiography. Safety was evaluated related to the procedure and clinical outcomes were assessed by National Institute of Health Stroke Scale (NIHSS) score at discharge and modified Rankin scale (mRS) score at 3 months. RESULTS: Eleven patients (median NIHSS 12.8, mean age 61.6 years, male: female = 8:3) with acute IA occlusion were treated with TEB. All the patients presented with TICI 0, and the occluded vessel was the middle cerebral artery (n=7), the basilar artery (n=1), and the distal ICA occlusion (n = 3). IV infusion of tissue plasminogen activator (tPA) was done in 4 patients and mechanical thrombolysis with intra-arterial UK was performed in 9. Recanalization was achieved in 73% (8 patients; TICI > or = 2). There were no procedure-related complications except for two asymptomatic intracranial hemorrhages. Improvement (> or = 4 points on the NIHSS) and good outcome (mRS < or =2) after 90 days was shown in six patients (55%). One patient died 6 days after procedure. CONCLUSION: TEB may be a valuable treatment option in acute thromboembolic IA occlusion without stent implantation.

Stroke Update 2011: New Antithrombotics / 대한뇌졸중학회지

Several new antithrombotic drugs have been developed and approved to use in clinical practice recently. Dabigatran, a direct thrombin inhibitor, and rivaroxaban, a factor Xa inhibitor, have been approved in many countries including Korea to prevent stroke in patient with atrial fibrillation. Apixaban, another factor Xa inhibitor, showed good results in clinical trial and is waiting for approval for clinical use. New antiplatelet agent, terutroban, selective thromboxane A2 receptor inhibitor, failed to prove the efficacy over the aspirin in secondary stroke prevention. Vorapaxar, a new antiplatelet agent that inhibits thrombin through PAR-1 antagonism, showed a high incidence of intracranial hemorrhage in patient with a history of stroke.

The Assessment of Routine Electroencephalography in Patients with Altered Mental Status

PURPOSE: Changes in electroencephalography (EEG) patterns may offer a clue to the cause of altered mental status and suggest the prognoses of patients with such mental status. We aimed to identify the EEG patterns in patients with altered mental status and to correlate EEG findings with clinical prognoses. MATERIALS AND METHODS: We included 105 patients with altered mental status who underwent EEG. EEG and clinical chart reviews with ongoing patient follow-ups were performed to determine the clinical prognosis of the patients. Clinical data were sorted using the Glasgow Coma Scale (GCS) and Glasgow Outcome Scale (GOS). EEG findings were classified according to a method suggested by Scollo-Lavizzari. The EEGs were analyzed to find out whether any correlation existed with the prognoses of patients. RESULTS: Nonconvulsive status epilepticus (NCSE) was detected in only three patients (2.9%). Specific EEG patterns were observed in 28 patients. Twenty-nine (27.6%) patients expired, and 45 (42.9%) patients were in a vegetative state. EEG grade and GCS significantly correlated with GOS. EEG grade alone had a correlation with GCS. Patients with a severe EEG finding had a poor prognosis. CONCLUSION: EEG findings reflect the mental status of patients, and EEG grades are correlated with the clinical prognosis of patients. Although EEG is not frequently performed on patients with altered mental state, it can play a supplemental role in establishing a prognosis. Thus, the use of EEG should be emphasized in clinical setting.

Clinical Features and Genetic Analysis of Fascioscapulohumeral Muscular Dystrophy

BACKGROUND: Facioscapulohumeral muscular dystrophy (FSHD) is associated with contractions of the polymorphic D4Z4-repeat array in 4q35 and has the distinctive clinical presentation of an initial involvement of the facial, shoulder-girdle, and upper-arm muscles. The aim of the present study was to determine clinical characteristics in Korean patients with FSHD and potential relationships between contracted D4Z4-repeat size and the FSHD phenotype. METHODS: We studied 34 genetically confirmed patients who had repeat sizes less than 38 kb, and analyzed their clinical manifestations with a structured protocol. The expressed phenotypes were scored according to the Clinical Severity Score formulated by Ricci and van Overveld. RESULTS: The clinical spectrum ranged widely, from asymptomatic individuals with minimal signs to wheelchair- bound patients. The initial affects were mainly in the facial muscles (68.8%), followed by the shoulder-girdle muscle (28.1%). Asymmetric features of the face and shoulder girdle were also important findings (71.9% and 90.0%, respectively). Winging scapular (87.5%), transverse smile (84.4%), Beevor's sign (68.8%), and sleeping with eyes opened (59.4%) were clinically important signs. There was a significant negative correlation between repeat size and clinical severity (r=-0.38, p=0.03). CONCLUSIONS: Distinctive clinical characteristics of FSHD are descending progression and asymmetric distribution of the muscle weakness. Our results also confirmed that the severity of FSHD increases with decreasing D4Z4-repeat size.

Three Patients With Classic and Atypical Neurodegeneration With Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is a disorder characterized by various mixtures of extrapyramidal, pyramidal or psychiatric abnormalities associated with iron accumulation in the basal ganglia. The mutations in the pantothenate kinase gene (PANK2) were found in approximately two thirds of the patients with NBIA. We report three patients wtih NBIA, and two of them showed mutations in the PANK2 gene.

Rescue Use of Tirofiban for Acute Carotid In-Stent Thrombosis

Carotid artery stenting is widely performed for extracranial carotid artery stenosis. In-stent thrombosis is a rare but potentially devastating complication. We present a case of acute in-stent thrombosis immediately following stent insertion and post-balloon dilatation in a 64-year-old male. Thrombosis was successfully treated by intravenous tirofiban, a glycoprotein IIb/IIIa receptor inhibitor.