Onset Of Neurological Symptoms Research Articles

Movement Disorder in Wilson Disease: Correlation with MRI and Biomarkers of Cell Injury

To report the phenomenology of movement disorder (MD) in neurological Wilson disease (NWD), and correlate these with MRI, and biomarkers of oxidative stress, excitotoxicity, and inflammation. Eighty-two patients were included, and their phenomenology of MD was categorized. The severity of dystonia was assessed using the Burke-Fahn-Marsden score, and chorea, athetosis, myoclonus, and tremor on a 0-4 scale. The MRI changes were noted. Serum glutamate, cytokines, and oxidative stress markers were measured. Movement disorders were noted in 78/82 (95.1%) patients and included dystonia in 69 (84.1%), chorea in 31 (37.8%), tremor in 24 (29.3%), parkinsonism in 19 (23.2%), athetosis in 13 (15.9%), and myoclonus in 9 (11.0%) patients. Dystonia was more frequently observed in the patients with thalamic (76.8 vs 23.2%), globus pallidus (72.0 vs 28.0%), putamen (69.5 vs 30.5%), caudate (68.3 vs 31.7%) and brainstem (61.0 vs 39.0%) involvement, and tremor with cerebellar involvement (37.5 vs 5.2%). The median age of onset of neurological symptoms was 12 (5-50) years. WD patients had higher levels of malondialdehyde (MDA), glutamate, and cytokines (IL-6, IL-8, IL-10, and TNFα) and lower levels of glutathione and total antioxidant capacity (TAC) compared with the controls. Serum glutamate, IL-6, IL-8, and plasma MDA levels were increased with increasing neurological severity, while glutathione and TAC levels decreased. The severity of dystonia related to the number of MRI lesions. MD is the commonest neurological symptoms in WD. Oxidative stress, glutamate, and cytokine levels are increased in WD and correlate with neurological severity.

Frequency, Onset and Clinical Impact of Neurological Symptoms in COVID-19 Disease: A Cohort Study on 576 Confirmed Cases

Introduction: Coronavirus disease 2019 (Covid-19) might present neurologic symptoms. We aimed to evaluate the frequency and type of neurologic symptoms in patients with Covid-19 at the moment of emergency department (ED) visit, and their impact in the prognosis. Methods: Retrospective cohort study including all consecutive confirmed Covid-19 cases that were hospitalized between March 8th and April 11th, 2020. We compared, in patients with and without neurological symptoms on admission, demographic, clinical presentation, and frequency and type of abnormal laboratory values. We analyzed the variables that were associated with in-hospital all-cause mortality by Cox-regression log rank test. Results: We included 576 patients, 250 (43·3%) female, aged 67·2 years in mean. At the moment of ED visit, 320 (55·6%) patients described neurologic symptoms, including anosmia in 146 (25·3%), myalgia in 139 (24·1%), headache in 137 (23·8%) and altered mental status in 98 (17·0%). In those patients, neurologic symptoms started the first day of symptoms in 198 (54·2%) cases. Patients with neurologic symptoms presented later to the ED (7·9 vs. 6·6 days, p=0·019). In patients with neurologic symptoms, only four (0·6%) cases had not typical Covid-19 general symptoms and only six (1·9%) had a normal laboratory results at the moment of ED presentation, for a sensitivity of 98·7% (95% confidence interval (CI): 96·6-99·6%) and 98·1% (95% CI: 95·7-99·2%), respectively. In the multivariate Cox-regression analysis of mortality predictors, anosmia (HR: 0·358, 95%CI: 0·140-0·916) and altered mental status (HR: 1·867, 95%CI: 1·162-3·001) remained statistically significant. Conclusion: Neurologic symptoms were the most frequent extrapulmonary symptoms. They were present in half of the patients with Covid-19 at the time of the ED visit. In almost all of the cases, typical Covid-19 general symptoms and/or laboratory abnormalities were present. The presence of neurologic symptoms at the moment of ED presentation was an independent predictor of mortality. Funding Statement: The present study did not have any specific source of funding. Declaration of Interests: Authors declare not to have any conflict of interest. Ethics Approval Statement: The study was approved by the local ethics review board (PI-20-1751), which waived the need of written informed consent.

Acute Transverse Myelitis and Acute Disseminated Encephalomyelitis.

1. Ilana Kahn, MD* 1. *Children’s National Health System, George Washington University Medical School, Washington, DC * Abbreviations: ADEM: : acute disseminated encephalomyelitis ADS: : acquired demyelinating syndrome AQP4: : aquaporin-4 ATM: : acute transverse myelitis CNS: : central nervous system CSF: : cerebrospinal fluid IgG: : immunoglobulin G IVIg: : intravenous immunoglobulin MOG: : myelin oligodendrocyte glycoprotein MRI: : magnetic resonance imaging MS: : multiple sclerosis NMO: : neuromyelitis optica NMOSD: : neuromyelitis optica spectrum disorder OCB: : oligoclonal band Most pediatricians report lack of knowledge related to the understanding of the diagnosis and treatment of both acute disseminating encephalomyelitis and acute transvers myelitis. Pediatric providers should understand presenting symptoms, initial diagnostic testing, and acute treatment. Clinicians should know when to refer to a neurologist for evaluation of long-term treatment. After completing this article, readers should be able to: 1. Define and characterize acquired demyelinating syndromes. 2. Identify the prevalence, etiology, and clinical presentations of acute disseminating encephalomyelitis (ADEM) and acute transverse myelitis (ATM). 3. Initiate a diagnostic evaluation, including an evaluation for medical emergencies. 4. Make treatment decisions for acute management. 5. Counsel patients on long-term outcomes after ADEM and ATM. 6. Counsel patients on recurrence risks for multiphasic or chronic demyelinating diseases. Acquired demyelinating syndromes (ADSs) encompass a group of immune-mediated disorders in which there is breakdown of the myelin sheath, the lipid-rich covering around the axon that increases conduction speed and metabolic efficiency of the neuron. ADSs are characterized by a sudden onset of new neurologic symptoms in concurrence with neuroimaging evidence of demyelination. Outcomes vary from full neurologic recovery to long-term severe disability. ADSs are characterized based on location and frequency of the demyelinating events. Location is either focal, limited to 1 specific location, or polyfocal, involving several areas of the central nervous system (CNS). Frequency is classified as monophasic or multiphasic, meaning either a single 1-time event or a recurrent, potentially chronic, disease. It is important to keep at the forefront of your mind, however, that over time what seems to be a monophasic event may, in fact, turn out to be the initial or first attack of a multiphasic disorder. (1) …

The impact of COVID-19 on patients with neuromyelitis optica spectrum disorder; a pilot study

Neuromyelitis optica spectrum disorder (NMOSD) is a CNS neuroinflammatory disorder, mediated by the pathogenic autoantibody aquaporin-4 (AQP4-IgG). Current treatment includes long-term use of immunomodulatory therapies, leading to increased rates of infections among this population. It is of interest therefore, to study how the COVID-19 pandemic affects NMOSD patients in terms of their disease activity. A 15-point questionnaire was administered to 33 participants living in Northern California with NMOSD, MS and other related disorders. Although none of the participants were diagnosed with COVID-19, our results show that 2 participants with NMOSD experienced new onset of neurological symptoms and 2 experienced worsening of previous neurological symptoms – suggesting a possible effect of pandemic-related stress on this CNS autoimmune disorder.

Symptomatic vertebral hemangioma during pregnancy period: A case series and systematic literature review.

Vertebral Hemangioma (VH) is a benign tumor usually symptomless and discovered incidentally. Pregnancy, because of several hormonal and physiologic changes, is a recognized risk factor coinciding with the development of a rapid onset of neurological symptoms in patients affected by VH. In the Literature, sporadic cases of neurological symptoms have been described, which occurred during pregnancy, but only rarely the onset of symptoms was reported after pregnancy and childbirth. Usually surgical treatment is reserved for severe cases with rapid onset of neurological symptoms. However, the use of conservative treatments is still a topic of debate In the present study, we report a series of patients affected by VH become symptomatic during or after pregnancy along with a systematic review of the Literature.

The disease presentation of Moyamoya angiopathy in Eastern India

IntroductionClinical spectrum of Moyamoya angiopathy (MMA) differs across populations with different ethnicity. This study, the largest one done among Indian population was undertaken to assess clinico-radiological profile of MMA patients in eastern India. MethodsA single centre cross-sectional study was undertaken among 76 MMA cases. Each patient was evaluated for epidemiological, clinical and radiological characteristics. SPSS 25 was used for statistical analysis. P < 0.05 was taken as statistically significant. Results36 (47.4%) were children without gender preponderance. There were female predominance among adults (male:female = 1:2.33). Mean age at onset of first neurological symptoms for children was 4.2 ± 2.0years, followed by 34.9 ± 58.2months of latency with final diagnosis at the mean age of 7.4 ± 3.5years. For adults, mean age of onset of first neurological symptoms was 31.5 ± 12.3years, followed by 14.7 ± 41.7months time gap and diagnosed at the mean age of 33.5 ± 12.5years. There was a statistically significant difference between child and adult regarding the diagnostic latency (p = 0.035). Fixed motor weakness (FMW) was the predominant symptom across the whole disease course. Among children predominant first neurological symptom was fixed motor weakness (FMW) (52.8%), followed by seizures (22.2%). FMW was predominant (55%) first neurological complaint, followed by headache (22.5%) among adults. Seizure was more prevalent among children both as first (p = 0.002) and presenting symptom at the time of diagnosis (p = 0.048). Over the course of the disease seizure was more common among children (p = 0.001), while headache was more common among adults (p = 0.017). Recurrence of symptoms was more common among children (p = 0.059). Infarcts were more common among children (91.7%) than adults (72.5%), while hemorrhage was seen only among adults (25%) (p = 0.004). Isolated cerebral cortex was involved more commonly among children (59.4%) than adults (36.1%), while isolated subcortical involvement was seen only among adults (19.4%) (p = 0.016). Majority of the MMA cases were of Suzuki stage 4 (39.5%) and 5 (27.6%). Brain atrophy was associated with diagnostic latency (p = 0.009). ConclusionIndian Moyamoya presents similar to disease presentation in Caucasian and Japanese patients. It is a frequently overlooked cause of stroke in young, often with various non-motor presentations, failure to recognize which leads to delay in diagnosis. Radiological burden disproportionate to number of acute vascular events, with subtle neurological manifestations like headache or seizure, often with cognitive decline, should raise suspicion of MMA.

NUBPL mitochondrial disease: new patients and review of the genetic and clinical spectrum

BackgroundThe nucleotide binding protein-like (NUBPL) gene was first reported as a cause of mitochondrial complex I deficiency (MIM 613621, 618242) in 2010. To date, only eight patients have been reported...

Mental disorders in neurological diseases. Can symptoms of bipolar disorder be the first manifestation of X-linked adenoleukodystrophy? A case report

Abstract Background: X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disease which causes demyelination of the white matter of the brain. The symptoms include mental impairment, progressive paresis, impaired motor coordination, and epileptic seizures. Diagnosis is established mainly by genetic testing. Currently, the recommended treatment is haematopoietic stem cell transplantation (HSCT). Goal: The aim of the study was to present the case of a patient suffering from X-ALD, who developed symptoms of bipolar disorder in the initial phase of the disease prior to the onset of characteristic neurological symptoms. Case presentation: In 2015, a 33-year-old patient was admitted to a psychiatric department due to aggressive behaviour he showed towards his wife and other family members. He had been treated for a depressive episode in 2005, and for a manic episode without psychotic symptoms earlier in 2015. During the successive psychiatric hospitalizations, in addition to psychopathological symptoms, the patient had been observed to have neurological symptoms, which included progressive paraparesis and ataxia. In 2018, based on imaging and genetic tests, the patient was diagnosed with X-ALD. The patient’s condition gradually deteriorated; with time, he was unable to move on his own. During a hospital stay in 2019, he was transferred to an internal medicine department due to a progressive urinary tract infection, which, however, could not be controlled, and the patient died. Conclusions: 1. X-ALD is a rare metabolic illness. In the early stages of the disease, various psychopathological symptoms, including affective disorders, are observed. 2. Early initiation of adequate treatment increases the chances of extending the patient’s life. 3. In the present case, the patient did not die due to the underlying disease, but due to causes typical of bed-bound patients, i.e. complications of progressing infection.

Imaging of Neurologic Disease in Hospitalized Patients with COVID-19: An Italian Multicenter Retrospective Observational Study.

Of 725 consecutive hospitalized patients with coronavirus disease 2019, 108 (15%) had acute neurologic symptoms necessitating neurologic imaging.

Choroid plexus changes on magnetic resonance imaging in multiple sclerosis and neuromyelitis optica spectrum disorder

ObjectiveTo investigate alterations in the choroid plexus in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) using brain magnetic resonance imaging (MRI). MethodsWe prospectively recruited consecutive patients with MS or NMOSD from July 2018 to February 2019. The inclusion criterion was brain MRI within three months from onset of acute neurological symptoms. The thickness and enhancement ratio of the choroid plexus on gadolinium-enhanced T1-weighted images of patients with MS (n = 51), patients with NMOSD (n = 32), and healthy controls (HCs, n = 28) were compared. ResultsMRI in patients with MS or NMOSD showed a comparably thick but more enhanced choroid plexus compared with that of HCs. In the axial view, enhancement ratios of the lateral ventricle of MS and NMOSD patients and HCs were 1.64 ± 0.34, 1.65 ± 0.25, and 1.39 ± 0.17, respectively (P > .999 for MS vs. NMOSD; P = .001 for MS vs. HCs; P = .001 for NMOSD vs. HCs). ConclusionsThe choroid plexus was significantly more enhanced on brain MRI of patients with MS or NMOSD than on that of HCs, suggesting the involvement of the choroid plexus in the autoimmune inflammatory processes in MS and NMOSD.

Treatment outcomes following continuous miglustat therapy in patients with Niemann-Pick disease Type C: a final report of the NPC Registry

BackgroundNiemann-Pick disease Type C (NP-C) is a rare, progressive neurodegenerative disorder characterized by progressive neurodegeneration and premature death. We report data at closure of the NPC Registry that describes the natural history, disease course and treatment experience of NP-C patients in a real-world setting.MethodsThe NPC Registry was a prospective observational cohort study that ran between September 2009 and October 2017. Patients with a confirmed diagnosis of NP-C were enrolled regardless of treatment status. All patients underwent clinical assessments and medical care as determined by their physicians; data were collected through a secure internet-based portal.ResultsAt closure on October 19, 2017, 472 patients from 22 countries were enrolled in the NPC Registry. Mean (standard deviation) age at enrollment was 21.2 (15.0) years, and 51.9% of patients were male. First neurological symptom onset occurred during the early-infantile (< 2 years), late-infantile (2 to < 6 years), juvenile (6 to < 15 years), or adolescent/adult (≥ 15 years) periods in 13.5, 25.6, 31.8, and 29.1% of cases, respectively. The most frequent neurological manifestations prior to enrollment included ataxia (67.9%), vertical supranuclear gaze palsy (67.4%), dysarthria (64.7%), cognitive impairment (62.7%), dysphagia (49.1%), and dystonia (40.2%). During infancy, splenomegaly and hepatomegaly were frequent (n = 199/398 [50%] and n = 147/397 [37.0%], respectively) and persisted in most affected patients. Of the 472 enrolled patients, 241 were continuously treated with miglustat during the NPC Registry observation period, of whom 172 of these 241 patients were treated continuously for ≥12 months. A composite disability score that assesses impairment of ambulation, manipulation, language, and swallowing was highest in the early-infantile population and lowest in the adolescent/adult population. Among the continuous miglustat therapy population, 70.5% of patients had improved or had stable disease (at least 3 of the 4 domains having a decreased or unchanged score between enrollment and last follow-up). The NPC Registry did not identify any new safety signals associated with miglustat therapy.ConclusionsThe profiles of clinical manifestations in the final NPC Registry dataset agreed with previous clinical descriptions. Miglustat therapy was associated with a stabilization of neurological manifestations in most patients. The safety and tolerability of miglustat therapy was consistent with previous reports.

Acute spontaneous epidural hematoma of cervical spine presenting as quadriparesis in a young patient: a case report

Introduction: Spontaneous spinal epidural hematoma (SSEH) is a relatively uncommon pathology of the spine. The incidence of SSEH is approximately 0.1 – 1 per 100,000 individuals. SSEH can cause acute spinal cord compression and rapid onset of neurologic deficits. Although rare, SSEH must be considered in the differential diagnosis of patients presenting with sudden onset of neurologic symptoms without a history of trauma.Case presentation: We report a case of a 19-year-old female patient who presented with sudden onset of weakness of all four limbs with pain in the upper cervical region. She has no history of trauma. Magnetic Resonance Imaging (MRI) of the spine was done, which showed an epidural mass in the cervical region, causing severe cord compression. Emergency cervical laminectomy and evacuation of epidural hematoma has was done with excellent post-operative recovery.Conclusion: Spontaneous spinal epidural hematoma is a relatively rare entity of the spine. The signs and symptoms of this rare condition are non-specific. In patients presenting with a sudden onset of neurologic deficits, the condition should be one of the differential diagnoses. MRI is the investigation of choice in patients suspected to have SSEH. Early surgical intervention results in a better outcome. The level of preoperative neurologic deficit, the severity of the condition, and operative interval are important factors affecting the postoperative outcome.Keywords: cervical spine, laminectomy, spontaneous spinal epidural hematoma

Possible clinical role of MOG antibody testing in children presenting with acute neurological symptoms.

The differential diagnosis between acquired inflammatory demyelinating syndromes of the central nervous system (CNS), such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and acute disseminated encephalomyelitis (ADEM) can be very challenging at onset. Apart from cerebrospinal fluid oligoclonal bands and anti-aquaporin-4 antibodies (AQP4-Ab), definite diagnostic biomarkers are lacking. Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) have been increasingly described in children with AQP4-seronegative NMOSD, ADEM and other inflammatory demyelinating CND syndromes; despite partial overlaps with AQP4-Ab disease, a novel "MOG-Ab-disorder" phenotype has been suggested. In this study, we tested the presence of MOG-Ab and AQP4-Ab in 57 children at first onset of acute neurological symptoms; three clinical subgroups were identified: 12 patients had acquired inflammatory demyelinating CNS syndromes, 11 had other autoimmune/immune-mediated disorders of the central and peripheral nervous system and 34 had non-immune-mediated CNS disorders. MOG-Abs were found positive only in a subset of cases in the subgroup with acquired inflammatory demyelinating CNS syndromes (in 2/12 patients, both with non-MS phenotype) and in none of the patients with other autoimmune and immune-mediated disorders of the central and peripheral nervous system or with non-immune-mediated disorders of the CNS.Data from the literature review support clinical and analytical observations.

Alterations of lymphocyte count and platelet volume precede cerebrovascular lesions in stroke-prone spontaneously hypertensive rats

Background: Cerebral small vessel disease (CSVD) is associated with future stroke. Although pathological alteration in small vessels of patients with CSVD can be detected by neuroimaging, diagnosis of CSVD is delayed because it is an asymptomatic disease. The stroke-prone spontaneously hypertensive rat (SHRSP) show similar pathological features to human CSVD and develop stroke-related symptoms with advancing age.Objective: We investigated the time course of haematological parameters in Wistar rats and SHRSP.Material and Methods: Blood cells were analysed using an automated haematological analyser.Results: SHRSP develop stroke-related symptoms including onset of neurological symptoms, decreased body weight and blood brain barrier leakage between 12 and 14 weeks of age. Lymphocyte counts were gradually decreased at 3 weeks before development of stoke-related symptoms and then were further decreased after the development of stroke-related symptoms. The both mean platelet volume and large platelet ratio gradually increased at 3 weeks before the development of stoke-related symptoms. However, although SHRSP showed more microcytic red cells than Wistar rats, the trajectories of change in erythrocyte-related parameters were similar between Wistar rats and SHRSP.Conclusion: Our pilot study suggests that alterations of lymphocyte count and platelet volume predictive indicators for asymptomatic CSVD and symptomatic stroke in SHRSP.

Screening for Platelet Dysfunction and Use of Prophylactic Tranexamic Acid in Patients Undergoing Deep Brain Stimulation: A Retrospective Analysis of Incidence and Outcome of Intracranial Hemorrhage

Introduction: The rate of intracranial hemorrhage (ICH) after deep brain stimulation (DBS) is between 1.5 and 6.1%, with prolonged deficits occurring in 0.4–2.5% of the patients. This retrospective study investigates whether the prophylactic administration of tranexamic acid (TA) to patients with abnormal platelet function detected preoperatively by platelet function analyzer (PFA) lowered the risk for an ICH event. Methods: We performed a systematic review of the medical records of 485 consecutively admitted patients who underwent bilateral DBS surgery in a single-center university hospital setting between 2009 and 2018. The cohort was split into two groups. In one group, preoperative PFA screening was performed (n = 156, patients recruited from 2014 to 2018), and TA was administered if platelet function was abnormal. No preoperative PFA was performed in the second group (n = 359, patients recruited from 2009 to 2013). Both cohorts were analyzed for the occurrence of ICH, defined by (i) detection of ICH in routine postoperative magnetic resonance/computed tomography imaging or (ii) in non-routine imaging for the onset of new neurological symptoms. Results: Fourteen of the 156 screened patients (9%) showed reproducible PFA-100 closure abnormalities (3 with von Willebrand disease, 11 with no identifiable cause of platelet dysfunction). Two of the 156 patients (1.3%) in this cohort revealed an ICH on imaging, 1 of whom (0.6%) exhibited a prolonged neurological deficit as a result of ICH. In the cohort without platelet testing, 11 of the 329 patients (3.3%) demonstrated ICH on imaging, of whom 5 (1.5%) suffered from a prolonged neurological deficit. Conclusion: In this retrospective study, the screening and the administration of TA appeared to lower the risk of an ICH by 1.8%. One patient with von Willebrand disease suffered an ICH despite TA treatment. A prospective study is needed to clarify the impact of platelet testing and TA administration on the of incidence ICH.

Safety of urgent carotid endarterectomy following thrombolysis.

Acute ischemic stroke is a leading cause of morbidity and mortality worldwide, and the incidence of ischemic stroke is predicted to increase in coming years. Carotid atherosclerotic occlusive disease accounts for up to 20% of all ischemic strokes, and mounting evidence suggests that, in the setting of an acute ischemic stroke due to carotid disease, earlier treatment with carotid intervention results in better outcomes. In patients with acute ischemic stroke, systemic or intravenous thrombolysis (IVT) has revolutionized ischemic stroke therapy, and intravenous tissue plasminogen activator (tPA) has become the principal treatment for acute ischemic stroke when administered within 3 to 4.5 hours of neurologic symptom onset. Given these trends in acute ischemic stroke therapy, vascular specialists are increasingly asked to perform carotid intervention following IVT, but reports in the literature examining outcomes in this circumstance are scarce, and the data regarding the appropriate interval from IVT to carotid endarterectomy (CEA) remains controversial. Literature searches were performed in PubMed (MEDLINE) and Ovid examining journal articles published between January 1st, 1998 and September 30th, 2019. The search terms used were: "urgent carotid endarterectomy," "carotid endarterectomy" AND "thrombolysis," "acute stroke and thrombolysis," "timing of carotid endarterectomy," and various combinations of these terms. A total of 21 published reports detailing outcomes in 1165 patients have been published to date, with an average interval from IVT to CEA of 7.1 days, a cumulative 30-day stroke and death rate of 4.1% (0-18%) and a mean frequency of intracranial hemorrhage of 2.6% (0-18%). The aggregate data from the 21 reported series suggest that CEA can be performed safely within the first 14 days after the onset of neurologic symptoms in patients receiving antecedent IVT, however, data regarding the safety of urgent CEA within 48 to 72 hours of thrombolysis is conflicting, with some series reporting excellent results and others showing an increased risk of ICH, stroke, and/or death in these select patients. Given the trend toward expedited treatment of acute ischemic stroke with subsequent transfer to regional referral centers, vascular specialists will be confronted with an increasing number of patients who may require urgent CEA after antecedent IVT. Further study is warranted to clearly delineate the appropriate interval from IVT to CEA and, specifically, to establish the safety of CEA with 72 hours of tPA administration.

Predictors of acute metabolic decompensation in children with maple syrup urine disease at the emergency department.

Acute metabolic decompensation (AMD) of maple syrup urine disease (MSUD) must be promptly recognized and treated. In this study, we aimed to identify simple variables associated with AMD in children with MSUD for use in emergency settings. Data were collected retrospectively from 115 emergency visits of 29 children with MSUD over a 4-year period in a major referral hospital. Variables in visits with and without AMD were compared using t test, Mann-Whitney U test, and chi-square test. Logistic regression was used to identify independent variables associated with decompensations. Cut-off values of laboratory variables were determined with receiver operating characteristic curves and correlations with Spearman's rank correlation. Most important variables independently associated with AMD were poor feeding, malaise, anion gap, and especially uric acid, which correlated with leucine levels. Vomiting, dehydration, neurological signs, ketonuria, and ketoaciduria were also associated with AMD. Although sodium, chloride, and glucose were lower in AMD, they had little diagnostic value.Conclusion: In children with MSUD, uric acid and anion gap are key markers for AMD. Poor feeding and malaise are clues before the onset of neurological symptoms. These simple parameters can help determine the presence of AMD in emergency settings.What is Known:• In maple syrup urine disease, acute metabolic decompensations are characterized by gastrointestinal and neurological findings.• Diagnosis requires detection of significantly elevated leucine, which may take a long time or not be available.What is New:• Poor feeding, malaise, hyperuricemia, and high anion gap are parameters that can help diagnose acute decompensations in children with maple syrup urine disease at emergency departments.• Uric acid may be a biomarker for acute decompensations because of its high sensitivity, specificity, and its strong correlation with leucine.

Abstract TP84: Prognostic Factor of Intracranial Vertebral Artery Dissection: High Resolution Vessel Wall Magnetic Resonance Imaging Study

Background: Vertebral artery dissection (VAD) is well recognized cause of stroke in young and middle aged individuals. But, prognostic factor of posterior circulation ischemic stroke or transient ischemic attack (TIA) caused by intracranial VAD has been rarely discussed. Our aim was to evaluate the predictors of poor outcomes in posterior circulation ischemic stroke caused by intracranial VAD. Methods: We retrospectively reviewed patients with posterior circulation ischemic stroke or TIA caused by intracranial VAD using high-resolution vessel wall MRI who were recruited from Samsung Medical Center Stroke Registry (between January 1, 2011 and April 30, 2019). Poor outcomes were defined as a 3-months modified Rankin scale (mRS) score ≥ 2. Results: We registered 96 patients (74 males; mean age, 58.9±14.2 years) with acute posterior circulation ischemic stroke or TIA caused by intracranial VAD. Trauma history associated with VAD was presented in 23 (24%) of patients. Headache and neck pain around neurological symptom onset were presented in 41 (42.7%) and 19 (19.8%) of patients, respectively. Dissecting aneurysm, bilateral vertebral artery involvement, basilar artery involvement and wall hematoma with dissection were presented in 26 (27.1%), 26 (27.1%), 12 (12.5%) and 66 (68.8%) of patients, respectively. Of the 96 VADs, 26 (27.1%) presented with focal stenosis, 21 (21.9%) with multifocal stenosis, and 57 (59.4%) with occlusion. Lateral medulla involvement and multiple territory involvement were presented in 35 (36.5%) and 31 (32.3%) of patients. Recurrence rate of ischemic stroke or TIA within 90 days of symptom onset was 6.25%. Twenty-nine patients (30.2%) had poor outcomes at 3 months. Lateral medulla involvement was an independent predictor for poor outcome (odds ratio=3.293, 95% confidence interval=1.301-8.333, p=0.012). Conclusion: Posterior circulation ischemic stroke or TIA caused by intracranial VAD is associated with relatively benign clinical course. But the presence of lateral medulla involvement is independent predictor for poor outcome. Patients presenting lateral medullary infarction caused by intracranial VAD should be monitored closely.

Abstract WP383: Intraoperative Strokes in Patients Who Underwent Transcatheter and Surgical Aortic Valve Replacement

Introduction: Perioperative ischemic stroke is a complication of transcatheter and surgical aortic valve replacement (TAVR/SAVR). We compared characteristics of perioperative stroke in TAVR and SAVR. Methods: Between January 2012 to December 2017, 9069 patients underwent TAVR or SAVR at single tertiary center. ECMO patients, with ventricular assist devices, or those who underwent concurrent cardiac procedures other than coronary revascularization were excluded. A patient was presumed to have an “intraoperative” stroke if last known normal (LKN) was immediately before surgery. If LKN was after surgery, then the stroke was considered be “postoperative”. Time of stroke discovery was defined as time of neurologic symptom onset. If symptom onset time was unknown, then time of initial CT brain was used. Results: One hundred seven patients with perioperative strokes met inclusion for analysis. Twenty-eight TAVR patients developed a perioperative stroke, compared to 79 SAVR patients. Strokes were intraoperative in 48 (44.9%) and postoperative in 59. No difference was found in the occurrence of intraoperative strokes between TAVR (15/28, 31.3%) and SAVR (33/79, 41.8%, p=0.28). Time from LKN to discovery of intraoperative stroke was median 606 minutes in TAVR and 1359 minutes in SAVR (p=0.049). Median time from LKN to discovery of a postoperative stroke was no different between TAVR and SAVR groups (median 120 minutes TAVR, 173.5 minutes SAVR p=0.57). Among 48 patients who had an intraoperative stroke, only TAVR patients (n=2) received acute reperfusion therapy. Conclusion: Intraoperative strokes were discovered sooner in patients who had undergone TAVR than SAVR. Early detection of perioperative strokes may allow for more opportunities for reperfusion therapy.

Results of endovascular treatment of acute tandem occlusion of internal carotid and middle cerebral arteries

To evaluate the results of endovascular treatment of patients with ischemic stroke caused by acute tandem occlusion of the internal carotid and middle cerebral arteries (ICA and MCA). Endovascular intervention was performed in 8 patients with tandem occlusion of ICA and MCA. The neurological deficit on admission was 17.4±5.6 NIHSS points and the average ASPECTS score was 9.5±0.8. The time from the onset of neurological symptoms to reperfusion was 224±68 min. Thrombolytic therapy was initiated before brain revascularization and continued intraoperatively in 4 out of 8 patients. ICA stenting after aspiration thrombectomy was required in 6 patients. Both antegrade (n=4) and retrograde (n=2) methods of brain revascularization were used. The rate of detection of tandem lesion in patients with ischemic stroke who underwent endovascular treatment was 5.4%. Technical success in restoring blood flow in the ICA basin at extra-and intra-cranial levels (mTICI 2b/3) was achieved in 7 (87.5%) patients. Intracranial hemorrhagic complications that affected the outcome of the disease were clinically significant in 25% of patients (n=2). The mortality rate was 37.5% (n=3). A satisfactory clinical result (a deficit of 0-2 mRS scores) was achieved at discharge in one of 5 surviving patients. Further studies of choosing the optimal surgical tactics for tandem occlusion of ICA and MCA, taking into account its effectiveness and safety, are need.