Extensive Leukoencephalopathy in Spastic Paraplegia Type 4: Possible Role of Cerebral Autosomal Arteriopathy With Subcortical Infarcts and Leukoencephelopathy

Despite recent advances in next-generation sequencing, the underlying etiology of adult-onset leukoencephalopathy has been difficult to elucidate. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a representative hereditary adult-onset leukoencephalopathy associated with vasculopathy. Leukoencephalopathy in spastic paraplegia type 4 (SPG4) is known to be rare, but it might be underestimated because most spastic paraplegia with leukoencephalopathy is rarely considered. We report a case of co-occurring SPG4 and CADASIL. A 61-year-old male presented with sudden visual impairment after a headache. He showed a spastic gait and had a family history with similar symptoms. An SPG4 gene mutation and a pathogenic variant in the NOTCH3 gene were found. This case shows that the diverse and complex clinical manifestations of patients with extensive leukoencephalopathy are related to more than one gene mutation. We also suggest the necessity for relevant genetic tests in the diagnosis of adult-onset leukoencephalopathy.

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.

Executive Summary of Stroke Statistics in Korea 2018: A Report from the Epidemiology Research Council of the Korean Stroke Society / 대한뇌졸중학회지

Despite the great socioeconomic burden of stroke, there have been few reports of stroke statistics in Korea. In this scenario, the Epidemiologic Research Council of the Korean Stroke Society launched the “Stroke Statistics in Korea” project, aimed at writing a contemporary, comprehensive, and representative report on stroke epidemiology in Korea. This report contains general statistics of stroke, prevalence of behavioral and vascular risk factors, stroke characteristics, pre-hospital system of care, hospital management, quality of stroke care, and outcomes. In this report, we analyzed the most up-to-date and nationally representative databases, rather than performing a systematic review of existing evidence. In summary, one in 40 adults are patients with stroke and 232 subjects per 100,000 experience a stroke event every year. Among the 100 patients with stroke in 2014, 76 had ischemic stroke, 15 had intracerebral hemorrhage, and nine had subarachnoid hemorrhage. Stroke mortality is gradually declining, but it remains as high as 30 deaths per 100,000 individuals, with regional disparities. As for stroke risk factors, the prevalence of smoking is decreasing in men but not in women, and the prevalence of alcohol drinking is increasing in women but not in men. Population-attributable risk factors vary with age. Smoking plays a role in young-aged individuals, hypertension and diabetes in middle-aged individuals, and atrial fibrillation in the elderly. About four out of 10 hospitalized patients with stroke are visiting an emergency room within 3 hours of symptom onset, and only half use an ambulance. Regarding acute management, the proportion of patients with ischemic stroke receiving intravenous thrombolysis and endovascular treatment was 10.7% and 3.6%, respectively. Decompressive surgery was performed in 1.4% of patients with ischemic stroke and in 28.1% of those with intracerebral hemorrhage. The cumulative incidence of bleeding and fracture at 1 year after stroke was 8.9% and 4.7%, respectively. The direct costs of stroke were about ₩1.68 trillion (KRW), of which ₩1.11 trillion were for ischemic stroke and ₩540 billion for hemorrhagic stroke. The great burden of stroke in Korea can be reduced through more concentrated efforts to control major attributable risk factors for age and sex, reorganize emergency medical service systems to give patients with stroke more opportunities for reperfusion therapy, disseminate stroke unit care, and reduce regional disparities. We hope that this report can contribute to achieving these tasks.

Isolated infraspinatus atrophy due to suprascapular neuropathy presenting as abnormal shoulder posture

@#Isolated infraspinatus atrophy is rare and difficult to confirm based on physical examination, although external rotation lag signs may provide a clue to the diagnosis. We present a case of isolated infraspinatus weakness caused by suprascapular neuropathy presenting as abnormal shoulder posture.

The factors affecting the level of urgency in the frequent users of the emergency department

OBJECTIVE@#study was conducted to identify patients who actually require medical treatment in the frequent users of the emergency department (ED) and evaluate the factors affecting the level of urgency by Korean Triage and Acuity Scale.@*METHODS@#We retrospectively reviewed the medical records of frequent users who used more than four times a year to the ED in 2015. They were triaged on every use of ED and divided into non-urgent group and urgent group based on an average triage scale of three. The characteristics were compared between both groups.@*RESULTS@#The total 443 patients were frequent users and they used the ED 2,944 times. The urgent group included 92 patients, and their median number of ED uses were 4 times. The urgent group was older and higher rate of male than the non-urgent group. The more patients suffered from medical diseases such as diabetes, cerebrovascular disease, ischemic heart disease, other heart disease, lung disease, and kidney disease in the urgent group. There was no difference in education, and socioeconomic status, and ratio uses with same symptoms in both groups. At the end of the study two years later, 55% of the urgent group died.@*CONCLUSION@#The urgent group consists of 92 patients (21%) of the frequent emergent department users. The factors affecting the level of urgency are male sex, cerebrovascular disease, other heart disease, lung disease, and kidney disease as medical history. On the other hand, the psychiatric history and other minor diseases are factors affecting reversely the level of urgency.

Advantages and disadvantages of the ENVOY 6F distal access guiding catheter in endovascular coiling for anterior circulation aneurysms

OBJECTIVE: Selecting an appropriate guiding catheter to provide both sufficient supportability for working devices and sufficient distal navigability is essential for ensuring the success of a procedure. This study aimed to evaluate the advantages and disadvantages of using the ENVOY 6F distal access (DA) guiding catheter in coil embolization of anterior circulation cerebral aneurysms.METHODS: We included 98 patients (72 [73.5%] women, median age: 63 [range: 25–84] years) who underwent endovascular coiling with the ENVOY 6F DA guiding catheter from May to November 2016. We analyzed data on patient demographics and the number of co-axial techniques to position the guiding catheter, initial and final location of the catheter, and complications related to the catheter.RESULTS: The co-axial technique was used to position the ENVOY 6F DA guiding catheter in the internal carotid artery (ICA) in 20 cases (20.41%). The initial position of the ENVOY 6F DA guiding catheter involved the cervical ICA (79.6%), horizontal petrous ICA (17.3%), and vertical petrous ICA (3.1%). Final control angiograms after endovascular coiling showed proximal change in the final, compared to the initial, position of the ENVOY 6F DA guiding catheter in 25 cases (25.51%). Procedure-related complications were observed in nine patients (9.18%), involving vasospasm in all cases; however, there was no symptomatic case.CONCLUSION: The ENVOY 6F DA guiding catheter had relatively sufficient distal navigability without symptomatic procedural complications. However, the change in the catheter position after endovascular coiling denoted insufficient supportability.

Brain Plasticity and Neurorestoration by Environmental Enrichment

In the adult mammalian brain, neural-lineage cells are continuously generated in the subventricular zone (SVZ) and dentate gyrus of the hippocampus. These cells in vivo arising from the adult SVZ may be regulated by environmental enrichment (EE). EE is a method of raising animals in a huge cage containing novel objects, running wheels and social interaction with a complex combination of physical, cognitive, and social stimulations. EE can affect neural plasticity via overexpression of growth factors such as brain-derived neurotrophic factor (BDNF), vascular endothelial growth factor (VEGF), insulin-like growth factor-1 (IGF-1), fibroblast growth factor-2 (FGF-2), and synaptic activity-regulating genes. EE also have advanced effects on brain functions including the enhancement of motor and cognitive functions in normal and pathological states. Additionally, behavioral changes by EE are related with molecular changes including neurogenesis, gliogenesis, angiogenesis, axonal sprouting, and dendritic branching in the adult brain. In this review, we focus on brain plasticity and neurorestoration associated with molecular changes of neurotrophic growth factors such as BDNF, VEGF, IGF-1, FGF-2 and synaptic activity-regulating genes that occurs in interaction to EE.

Retrospective Cohort Study on the Administration of Sedative for Delirium in Terminally Ill Cancer Patients and Survival Time / 한국호스피스완화의료학회지

PURPOSE: This study analyzed the difference in survival time of patients with delirium according to sedative medication. METHODS: From January 2012 through December 2013, a retrospective cohort study was performed using the electronic medical records (EMR) of Pusan National University Hospital. Among 900 patients who died from cancer, we selected 240 who suffered delirium based on the EMR. The Nu-DESC delirium screening test was used to diagnose delirium. RESULTS: The median length of delirium period was five days. Delirium characteristics were dominated by inappropriate behaviors (35.0%). Sedatives were administered in 72.1% of the cases. The most frequently used sedative was haloperidol which was used in 59.6% of cases. The delirium period significantly differed by patients' age (F=3.96, P=0.021), cancer type (F=3.31, P=0.010), chemotherapy (t=−3.44 P=0.001). The average survival time was 16.85 days for the sedative medication group and 9.37 days for the non-medication group, which, however, was not significant (t=1.766, P=0.079). CONCLUSION: In this study, the use of sedatives did not affect patients' survival time. Thus, appropriate sedative medication can be positively recommended to comfort terminal cancer patients and their families.

Recurrent Carotid Cavernous Fistula Originating from a Giant Cerebral Aneurysm after Placement of a Covered Stent

We report the case of a recurrent carotid cavernous fistula (CCF) originating from a giant cerebral aneurysm (GCA) after placement of a covered stent. A 47-year-old woman presented with sudden onset of severe headache, and left-sided exophthalmos and ptosis. Cerebral angiography revealed a CCF caused by rupture of a GCA in the cavernous segment of the left internal carotid artery. Two covered stents were placed at the neck of the aneurysm. The neurological symptoms improved at first, but were aggravated in the 6 months following the treatment. Contrast agent endoleak was seen in the distal area of the stent. Even though additional treatments were attempted via an endovascular approach, the CCF could not be cured. However, after trapping the aneurysm using coils and performing superficial temporal artery-middle cerebral artery bypass, the neurological symptoms improved. In cases of recurrent CCF originating from a GCA after placement of a covered stent, it is possible to treat the CCF by endovascular trapping and surgical bypass.

Adjuvant Tirofiban Injection Through Deployed Solitaire Stent As a Rescue Technique After failed Mechanical Thrombectomy in Acute Stroke / 신경중재치료의학

PURPOSE: We present our experiences of intra-arterial tirofiban injection through a deployed Solitaire stent as a rescue therapy after failed mechanical thrombectomy in patients with acute ischemic stroke. MATERIALS AND METHODS: Data on 18 patients treated with adjunctive tirofiban injection through a temporarily deployed Solitaire stent after failed mechanical thrombectomy were retrospectively reviewed. Solitaire stent was used as a primary thrombectomy device in 16 of 18 patients. Two patients received manual aspiration thrombectomy initially. If initial mechanical thrombectomy failed, tirofiban was injected intra-arterially through the deployed Solitaire stent and then subsequent Solitaire thrombectomy was performed. RESULTS: Fourteen patients had occlusions in the middle cerebral artery, 2 in the distal internal carotid artery, and 2 in the basilar artery. Successful recanalization was achieved in 14 patients (77.7%) after intra-arterial injection of tirofiban and subsequent Solitaire thrombectomy. Three patients without successful recanalization after rescue method received angioplasty with stenting. Overall, successful recanalization (TICI grades 2b and 3) was achieved in 17 (94.4%) of 18 patients. Periprocedural complications occurred in 5 patients: distal migration of emboli in 5 patients and vessel perforation in 1. Three patients died. Good functional outcome (mRS < or = 2) was achieved in 9 patients (50.0%) at 3 months. CONCLUSION: Rescue intra-arterial injection of tirofiban through a temporarily deployed Solitaire stent may facilitate further recanalization in cases of failed mechanical thrombectomy in patients with acute ischemic stroke.

Tsutsugamushi Meningitis with Parotitis Mimicking Mumps Meningitis

No abstract available.

Ultrasonographic features of vascular closure devices: initial and 6-month follow-up results

PURPOSE: This study aimed to evaluate the ultrasonographic findings for various types of vascular closure devices (VCDs) immediately after the angiographic procedure and at 6-month follow-up. METHODS: We included 18 VCDs including Angio-Seal (n=4), FemoSeal (n=8), ExoSeal (n=3), Perclose (n=2), and StarClose (n=1) in this study. Four patients were implanted with 2 VCDs at the each side of bilateral femoral arteries, while the remaining 8 patients were inserted 1 VCD at the right femoral artery. Ultrasonography was performed within 10 days and at approximately 6 months after the angiographic procedure. Ultrasonographic morphology of the attached VCD and its relationship with the arterial wall were analyzed. RESULTS: Initial ultrasonography revealed the attached VCD as the relevant unique structure with successful deployment and hemostasis. Follow-up ultrasonography demonstrated partial absorption of hemostatic materials in cases of Angio-Seal (n=3), FemoSeal (n=5), and ExoSeal (n=3), changes in the soft tissue surrounding the femoral artery in case of Angio-Seal (n=1), arterial intimal hyperplasia in cases of FemoSeal (n=3), and no gross changes as compared with the initial ultrasonographic findings in cases of Perclose (n=2) and StarClose (n=1). CONCLUSION: Initial ultrasonographic evaluation reflected the unique structure of each VCD, with most of them being easily distinguishable. Follow-up ultrasonography revealed various changes in the affected vessels.

Clinical Practice Guideline for the Management of Intracranial Aneurysms / 신경중재치료의학

PURPOSE: An intracranial aneurysm, with or without subarachnoid hemorrhage (SAH), is a relevant health problem. The rupture of an intracranial aneurysm is a critical concern for individual health; even an unruptured intracranial aneurysm is an anxious condition for the individual. The aim of this guideline is to present current and comprehensive recommendations for the management of intracranial aneurysms, with or without rupture. MATERIALS AND METHODS: We performed an extensive literature search, using Medline. We met in person to discuss recommendations. This document is reviewed by the Task Force Team of the Korean Society of Interventional Neuroradiology (KSIN). RESULTS: We divided the current guideline for ruptured intracranial aneurysms (RIAs) and unruptured intracranial aneurysms (UIAs). The guideline for RIAs focuses on diagnosis and treatment. And the guideline for UIAs focuses on the definition of a high-risk patient, screening, principle for treatment and selection of treatment method. CONCLUSION: This guideline provides practical, evidence-based advice for the management of patients with an intracranial aneurysm, with or without rupture.

Angiographic and Clinical Result of Endovascular Treatment in Paraclinoid Aneurysms / 신경중재치료의학

PURPOSE: The purpose of this study was to analyze the results of an immediate and mid-term angiographic and clinical follow-up of endovascular treatment for paraclinoid aneurysms. MATERIALS AND METHODS: From January 2002 to December 2012, a total of 113 consecutive patients (mean age: 56.2 years) with 116 paraclinoid saccular aneurysms (ruptured or unruptured) were treated with endovascular coiling procedures. Clinical and angiographic outcomes were retrospectively evaluated. RESULTS: Ninety-three patients (82.3%) were female. The mean size of the aneurysm was 5.5 mm, and 101 aneurysms (87.1%) had a wide neck. Immediate catheter angiography showed complete occlusion in 40 aneurysms (34.5%), remnant sac in 51 (43.9%), and remnant neck in 25 (21.6%). Follow-up angiographic studies were performed on 80 aneurysms (69%) at a mean period of 20.4 months. Compared with immediate angiographic results, follow-up angiograms showed no change in 38 aneurysms, improvement in 37 (Fig. 2), and recanalization in 5. There were 6 procedure-related complications (5.2%), with permanent morbidity in one patient. CONCLUSION: Out study suggests that properly selected patients with paraclinoid aneurysms can be successfully treated by endovascular means.

Unexpected Detachment of Solitaire Stents during Mechanical Thrombectomy

OBJECTIVE: Unexpected Solitaire stent detachment can occur during mechanical Solitaire thrombectomy. The purpose of this study was to retrospectively evaluate the influencing factors causing unexpected Solitaire stent detachment and the clinical outcomes. METHODS: Between October 2011 to December 2013, 232 cases of mechanical Solitaire thrombectomy for acute ischemic stroke were performed in 3 stroke centers. During this period, we encountered unexpected Solitaire stent detachments during mechanical Solitaire thrombectomies in 9 cases. RESULTS: Solitaire stents unexpectedly detached in 9 cases (3.9%) during the retrieval of Solitaire stents. The median patient age was 76 years. The occlusion sites of the unexpected stent detachment were the proximal middle cerebral artery (MCA) in 7 cases and the internal carotid artery in 2 cases. The sizes of the stents that unexpectedly detached were 6x30 mm in 7 cases, 5x30 mm in 1 case, and 4x20 mm in 1 case. Four patients had unexpected detachment at the first retrieval, 1 patient at the second, 3 patients at the third, and 1 patient at the fifth. In all of the cases of unexpected detachment at the first retrieval, the stent deployment site was the proximal MCA. After detachment, a proximal marker of the Solitaire stent was observed in 3 patients. However, no marker was visible in the remaining 6 patients. CONCLUSION: Unexpected Solitaire stent detachment should be considered in the first instance of stent retrieval for a relatively large-diameter stent, especially in elderly patients with MCA occlusions.

Recurrent Atopic Myelitis Presenting as an Isolated Lhermitte's Sign / 대한임상신경생리학회지

Atopic myelitis (AM) is a relatively mild form of myelitis associated with allergic diathesis, and present with predominant sensory manifestations. Lhermitte's sign has been considered as a relatively non-specific clinical sign suggesting demyelinating lesion in cervical cord. Here we report a patient with recurrent AM who presented with isolated Lhermitte's sign, both in first and second attacks. This report suggests that either the diagnosis or recurrence of AM can be frequently underdiagnosed because of its predominant sensory manifestations.

Acute Exacerbation of Interstitial Lung Disease in Newly Diagnosed Probable Dermatomyositis / 대한임상신경생리학회지

No abstract available.

Neurorestoration Induced by Mesenchymal Stem Cells: Potential Therapeutic Mechanisms for Clinical Trials

Stem cells are emerging as therapeutic candidates in a variety of diseases because of their multipotent capacities. Among these, mesenchymal stem cells (MSCs) derived from bone marrow, umbilical cord blood or adipose tissue, comprise a population of cells that exhibit extensive proliferative potential and retain the ability to differentiate into multiple tissue-specific lineage cells including osteoblasts, chondrocytes, and adipocytes. MSCs have also been shown to enhance neurological recovery, although the therapeutic effects seem to be derived from an indirect paracrine effect rather than direct cell replacement. MSCs secrete neurotrophic factors, promote endogenous neurogenesis and angiogenesis, encourage synaptic connection and remyelination of damaged axons, decrease apoptosis, and regulate inflammation primarily through paracrine actions. Accordingly, MSCs may prevail as a promising cell source for cell-based therapy in neurological diseases.

Three Cases of Anti-GQ1b Antibody Syndrome with Internal Ophthalmoplegia

The anti-GQ1b IgG antibody is associated with Guillain-Barre syndrome (GBS) with ophthalmoplegia, Bickerstaff's brainstem encephalitis, acute ophthalmoparesis without ataxia, and ataxic GBS without ophthalmoplegia as well as Miller Fisher syndrome. Only limited numbers of patients with acute ophthalmoparesis without ataxia are known to show internal ophthalmoplegia. We report three patients with internal ophthalmoplegia from anti-GQ1b antibody syndrome.

A Case of Chrug-Strauss Syndrome Complicated with Intestinal Perforation

Gastrointestinal symptoms of Churg-Strauss syndrome (CSS) are common, but intestinal perforation is very unusual and occurred during steroid therapy in the previous case. We report a CSS showing intestinal perforation, which was caused by a complication of vasculitis.