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Background: Coronavirus disease 2019 (COVID-19) typically causes breathing issues that can range from flu symptoms to extreme pneumonia, but it can also impair extra respiratory systems and cause multisystemic failure, including neurological complications. Case Presentation: A 55-year-old male with acute progressive symmetrical ascending quadriparesis complaints was admitted to the hospital. Twelve days prior to hospitalization, the patient with taste disruption, myalgia, fever, and polymerase chain reaction with reverse transcription was confirmed to have been positive for COVID-19 infection. The neurophysiological findings were consistent with the diagnosis of Guillain-Barre syndrome (GBS). Conclusion: COVID-19 activates inflammatory cells and creates a number of inflammatory cytokines and eventually produces immune-mediated processes. Both cell and humoral-dependent pathways of GBS pathogenesis are believed to be related. The peripheral nervous system, myelin, axons, and in some cases, both immune-mediated attacks are believed to be the cause of molecular expression. COVID-19 is believed to induce antibody formation against particular gangliosides. Further study is needed to understand the role of GBS caused by infection with COVID-19.

Continuous-flow left ventricular assist devices (LVADs) reduce peak systolic flow, increase diastolic flow, and eliminate pulsatility of circulation. Altered blood flow may lead to a change in end-organ perfusion. Analysis of the flow dynamics of the arteries of end organs, such as the brain, may indicate whether an organ is perfused sufficiently. The aim of this study is to evaluate and identify the flow pattern changes of carotid (CA) and middle cerebral arteries (MCA) in LVAD patients and to compare with heart failure patients and healthy volunteers. Eighty-nine individuals were included in this cross-sectional study. Participants were divided into three groups: LVAD patients (n = 31), heart failure patients (n = 26), and healthy volunteers (n = 27). Carotid and transcranial Doppler ultrasonography were performed for all study groups for peak systolic velocity (PSV), end-diastolic velocity (EDV), pulsatility (PI), and resistive (RI) indices of CA and MCA. Flow dynamics were compared between the groups. Doppler ultrasonographic data were analyzed at a median 12 (3-47) months after LVAD implantation. CA-PSV was lower in LVAD group compared with the other two groups (P

Background Neuroinflammation has an important role in the pathophysiology of migraine, which is a complex neuro-glio-vascular disorder. The main aim of this review is to highlight findings of cortical spreading depolarization (CSD)-induced neuroinflammatory signaling in brain parenchyma from the inflammasome perspective. In addition, we discuss the limited data of the contribution of inflammasomes to other aspects of migraine pathophysiology, foremost the activation of the trigeminovascular system and thereby the generation of migraine pain. Main body Inflammasomes are signaling multiprotein complexes and key components of the innate immune system. Their activation causes the production of inflammatory cytokines that can stimulate trigeminal neurons and are thus relevant to the generation of migraine pain. The contribution of inflammasome activation to pain signaling has attracted considerable attention in recent years. Nucleotide-binding domain (NOD)-like receptor family pyrin domain containing 3 (NLRP3) is the best characterized inflammasome and there is emerging evidence of its role in a variety of inflammatory pain conditions, including migraine. In this review, we discuss, from an inflammasome point of view, cortical spreading depolarization (CSD)-induced neuroinflammatory signaling in brain parenchyma, the connection with genetic factors that make the brain vulnerable to CSD, and the relation of the inflammasome with diseases that are co-morbid with migraine, including stroke, epilepsy, and the possible links with COVID-19 infection. Conclusion Neuroinflammatory pathways, specifically those involving inflammasome proteins, seem promising candidates as treatment targets, and perhaps even biomarkers, in migraine.

Background Acute ischemic stroke (AIS) is characterized as a neurological deficit owing to an acute focal damage to the brain by cerebral infarction. A collateral score is the most significant factor evaluating the prognosis of AIS, its relationship with demographic data, serum biochemical parameters, and clinical disability in this field. Purpose Our research aims to examine the relationship between collateral score (CS) and biochemical parameters and clinical disability in patients with AIS with M1 occlusion in the middle cerebral artery (MCA). Materials and methods We conducted a single-center retrospective study with 100 patients with AIS who are in the first 6 hours of ischemic stroke. Data for consecutive AIS patients were collected from February 2019 to May 2020. The collateral score was assessed by using developed scoring systems defined by Maas et al. The correlations between collateral score and demographic data, biochemical parameters, NIHSS scores (National Institutes of Health Stroke Scale), mRS (modified Rankin scale) scores were recorded. Results The research was performed in 100 patients (median age, 71.55±11.46 years) and there was a statistically significant difference between elevated erythrocyte distribution width (RDW) and Maas collateral score (insular cortex) (p= 0.024) and lymphocyte/monocyte ratio (LMO) and Maas collateral (leptomeningeal) score (p = 0.025). Conclusion In patients with acute MCA M1 occlusion, our analysis found a significant association between elevated RDW, low LMO parameters and collateral score. We assume that in terms of simple and quick usability in acute stroke prognosis, these parameters are useful and functional as a new biomarker.

Background & Objective: Arachidonic acid (ARA) is essential for the fluidity, selective permeability, and flexibility of the cell membrane. It is an important factor for the function of all cells, particularly in the nervous system, immune system, and vascular endothelium. ARA is the second most common polyunsaturated fatty acid in the phospholipids of the nerve cell membrane after docosahexaenoic acid. ARA metabolites have many kinds of physiologic roles. The major action of ARA metabolites is the promotion of the acute inflammatory response, mediated by the production of pro-inflammatory mediators such as PGE2 and PGI2, followed by the formation of lipid mediators, which have pro-resolving effects. Another important action of ARA derivatives, especially COX, is the regulation of vascular reactivity through PGs and TXA2. There is significant involvement of ARA metabolites in neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, and neuropsychiatric disorders. ARA derivatives also make an important contribution to acute stroke, global ischemia, subarachnoid hemorrhage, and anticoagulation-related hemorrhagic transformation. Conclusion: In this review, we have discussed experimental and human study results of neurologic disorders related to ARA and its metabolites in line with treatment options.

Amaç:Serebrovasküler hastalıkların kardiyak fonksiyonlar üzerine negatif etkileriolduğu bilinmektedir. Ek olarak, inme kliniğinde uzun dönem izlemde en sık ve en önemli ölüm nedeni kardiyakdisfonksiyondur. Bu çalışmanın amacı hemorajik inmeli hastalarda kardiyak enzimve inflamasyon belirteçlerinin değişimini incelemek ve bu belirteçlerin inmeprognozuna etklerini araştırmaktır. Materyal ve Metot: Bu çalışmaya intraserebral hematom (ISH) tanısı ile yatırılan ve hastalığın başlangıcının ilk 24 saati içindeolan 53 hasta ile 48 kontrol dahil edildi. Her iki grubun da 1., 5. ve 10. günkreatinin kinaz (CK), kreatinin kinaz myokardiyal band (CKMB), troponin-I(Trop-I), C reaktif protein (CRP) ve lökosit değerleri incelendi.Bulgular: Çalışmaya dâhil edilen 53 hasta ile 48 kontrolgrubu arasında yaş açısından anlamlı farklılık tespit edilmedi (P=0.062). 1.günCK, CRP ve lökosit değerleri ile 5. ve 10 gün CRP ve lökosit değerleri, ISH grubunda kontrol grubuna göre anlamlıolarak daha yüksek idi. Bununla beraber, ISH grubunda CK-MB ve troponindeğerlerinin giderek azaldığı, CRP değerlerinin ise giderek arttığı tespitedildi. 53 kişilik hasta grubunun 13 tanesi takipte exitus oldu. Yaşayanhastalar ile karşılaştırıldığında, exitus olan hastalarda 1. gün CK, CRP velökosit, 5. gün CK, CK-MB ve CRP ile 10. gün CK, CK-MB ve troponin değerlerianlamlı olarak daha yüksek idi.Sonuç:Çalışmamızda, intraserebral hematom tanılı hastalarda CK-MB vetroponin değerlerinde 1.günden itibaren bir azalma olduğunu, CRP değerlerindeise 1. günden itibaren bir artma olduğunu tespit ettik. Ek olarak, exitus olanhastalarda kardiyak enzim ve inflamatuvar belirteçlerin daha yüksek olduğunutespit ettik. Dolayısıyla hemorajikinmeli hastalarda myokardiyal hasarın ve inflamasyonun erken dönemdetanınmasının, morbidite ve mortaliteyi büyük ölçüde azaltabileceğinidüşünüyoruz., Background: Cerebrovascular diseases are known to havenegative effects on cardiac functions. In addition, cardiac dysfunction is themost common and most important cause of death in the stroke clinic duringlong-term follow-up. The aim of this study was to investigate the changes incardiac enzyme and inflammation markers in patients with hemorrhagic stroke andto investigate the effects of these markers on stroke prognosis.Methods: This study included 53 patients and 48 controls who were hospitalizedfor intracerebral hematoma (ICH) and were within the first 24 hours of theonset of the disease.Creatinine kinase (CK), creatinine kinase myocardial band (CKMB), troponin-I(Trop-I), C-reactive protein (CRP) and leukocyte values ​​of both groups were evaluated on the 1st, 5thand 10th days.Results: There were no significant differences in agebetween 53 patients and 48 control groups (68.50 ± 10.22 versus 71.70 ± 6.02, P= 0.062). CK, CRP and leukocyte values ​​on day 1 and CRP and leukocyte values ​​on day 5 and 10 were significantly higher inthe ICH group than in the control group. However, CK-MB and Troponin levelsdecreased and CRP values ​​increased gradually in ICH group. Thirteenpatients of 53 patients died during follow-up. Compared with the survivingpatients, CK, CRP and leukocytes on day 1, CK, CK-MB and CRP on day 5 and CK,CK-MB and Troponin on day 10 were significantly higher in patients with exitus.Conclusions: Inour study, we found a decrease in CK-MB and troponin values ​​from day 1 and an increase in CRP values ​​from day 1 in patients with intracerebralhematoma. In addition, we foundthat cardiac enzymes and inflammatory markers were higher in patients withexitus.Therefore, we believe that early recognition of myocardial damage andinflammation in hemorrhagic stroke patients may significantly reduce morbidityand mortality.

INTRODUCTION Specialized centers, in the management and surgical treatment of medically refractory epilepsy, emphasize the importance of differentiating the varieties of localization related epilepsies. There has been considerable recent interest in temporal and frontal lobe epileptic syndromes and less attention has been paid to parietal and occipital lobe epilepsies. METHODS Here we report the clinical, electroencephalographic and neuroimaging characteristics of 46 patients with medically refractory lesional parietal lobe epilepsy who have been followed up for 1-10 years. RESULTS In this study auras were reported in 78.3% of the patients and included sensory symptoms (72.2%), headache (36.1%), nausea and vomiting (36.1%), psychic symptoms (36.1%) and visual symptoms (16.6%). The most common ictal behavioral changes were paresthesia (69.6%) and focal clonic activity (39.1%). Tonic posture, various automatisms, head deviation, staring, sensation of pain and speech disturbances occurred to a lesser extent. Simple partial seizures were present in 69.6%. Complex partial seizures occurred in 43.5% and secondary generalized tonic clonic seizures were reported in 58.7% of the patients. Interictal routine EEG disclosed abnormal background activity in 1/3 of the patients. Nonlocalising epileptiform abnormalities were found in 34.8% of the patients. EEG findings were normal in 34.8% of the patients. The most common presumed etiologic factors were as follows: posttraumatic encephalomalacia, stroke, tumor, malformation of cortical development, atrophy, and arteriovenous malformation. CONCLUSION Clinical, electrophysiological and neuroimaging features of the lesional symptomatic partial epilepsy patients may help us to localize the seizure focus in some patients with cryptogenic partial epilepsy. So that, the timing decision of the parietal lobe sampling with more invasive techniques like intracranial electrodes prior to epilepsy surgery would be easier.

Introduction: Intracranial mycotic aneurysms (IMA) are rare but serious complications of infective endocarditis (IE). Methods and Results: In this retrospective study (1980-2011) we used original Duke criteria to diagnose IE in 1149 episodes (1081 patients; 81% definite, 80% native valve). Neuro complications occurred in 28%, stroke in 22% (202 infarcts, 53 hemorrhages) and 1% had TIA/TMB. N=33 IMA were detected in 23 (2%) patients. IMA were detected in 8% with focal neuro deficits, 13% with seizures, and 3% with encephalopathy. IMA-related symptoms were present in 22 of 23 cases: headache 48%, seizure 13%, altered sensorium 35% and focal deficits 61%. IMA were detected in 0/885 without stroke, 5.4% with infarcts and 22.6% with hemorrhages (pS.viridians infection (all p Conclusion: IMA are invariably heralded by neurological symptoms and stroke (especially hemorrhages) on brain imaging. Mitral IE has the highest risk for IMA. IMAs can resolve with antibiotics however studies are needed to determine the efficacy of different treatment approaches.

Background: Stroke is a major complication of infective endocarditis (IE). The features and predictors of ischemic and hemorrhagic stroke subtypes are unknown. Methods and Results: In this retrospective study (1980-2011) we used original Duke criteria to diagnose IE in 1149 episodes (1081 patients; 81% definite, 80% native valve). Neuro complications occurred in 28% including infarcts (17.6%), ICH (4.6%), meningitis (4%), brain/spinal abscess (8%), and seizures (2%). Ischemic stroke was the isolated presenting symptom in 11%; its most frequent presentations were total/partial anterior-circulation syndrome (47%) and embolic encephalopathy (21%). On DWI, infarcts were disseminated small (45%) or multi-sized (31%), single (16%), or territorial (9%). Disseminated infarcts were associated with poor outcome (p=0.026). Post-ischemic hemorrhage occurred in 6.4%. Primary ICH was evident on admission in 55% including 36 parenchymal (92% lobar), 13 SAH (69% convexal) and 4 SDH. As compared to the non-stroke group (n=885), the ischemic (n=202) and hemorrhagic stroke (n=53) groups had higher rates of complications (e.g., meningitis, seizures, sepsis, renal failure), longer LOS, higher mortality, and lower rates of home discharge (all pS. viridans and Group B strep infections and more valvular vegetations; the ischemic stroke group had more intracardiac thrombi, and the hemorrhagic stroke group had more mechanical valve infection (all pStaph aureus endocarditis were independent predictors of non-home discharge. Over three decades the LOS decreased, ischemic stroke rates increased, but there was no change in ICH or mortality rates. Conclusion: Endocarditis-related ischemic and hemorrhagic stroke subtypes have overlapping risk factors, predictors, clinical phenotypes, and effects on outcome.

Background Quantitative and qualitative evaluation of middle cerebral artery (MCA) density, together with extent of thrombi, was assessed on plain computerized tomography (CT) to delineate better the prognostic value of the hyperdense MCA sign (HMCAS) in a cohort of patients who underwent intravenous or intra-arterial thrombolysis. Methods Density of MCA was quantified by maximum pixel-sized measurement of Hounsfield unit (HU) in 105 patients with acute MCA proximal segment occlusion, 15 patients with vertebrobasilar circulation stroke (VBS) and 44 nonstroke control subjects. Predictive value of HMCAS, absolute HU value of within MCA, side-to-side HU ratio, and difference along with a newly introduced hyperdense MCA burden score in early dramatic recovery (EDR) and third-month favorable prognosis were determined with multivariate adjustment for age, baseline stroke severity, and thrombus length as measured on CT angiography. Receiver operator characteristics (ROC) curves were used to determine the cutoffs of quantitative indices to determine HMCAS and their prognostic significance. Results Higher HU was present in the ipsilateral MCA of the patients compared with their contralateral side and basilar tip and any MCA of VBS stroke and control subjects (area under the curve [AUC] of ROC curves was .753). Symptomatic-to-asymptomatic HU difference and ratio of MCA stroke were also significantly higher than side-to-side difference calculated in VBS stroke and control groups (AUC of ROC curves: .770 and .764, respectively). Optimal thresholds of absolute HU (44), side-to-side HU difference (2), and ratio (1.0588) showed borderline sensitivity and specificity. HMCAS and its quantitative indices were not significantly associated with EDR and favorable third-month outcome. Furthermore, there was no difference in terms of cardioembolic and atherothrombotic thrombi HU. Conclusions Utility of the HMCAS as a prognostic marker in stroke thrombolysis is not high in the CT angiography era. Previous observation regarding its positive prognostic role can be attributed to its association with proximal location and extent of clot burden, which are detectable reliably with current CT angiography techniques. Neither quantification nor extent of increased density seems to have clinical utility for treatment decision making in MCA strokes and prediction of emboli composition and response to recanalization attempt.

Background The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) is not known. Published cases have documented coexisting cervical artery dissection and unruptured aneurysms, raising the possibility that ultrastructural vessel wall abnormalities underlie the development of vascular lesions as well as RCVS. Methods In this retrospective study we compared the frequency of neurovascular abnormalities in 158 consecutive RCVS patients, 44 patients with primary angiitis of the central nervous system (PACNS, positive controls), and 177 non-stroke patients with acute neurological symptoms (non-arteriopathy controls). Results Coexisting neurovascular abnormalities were significantly higher ( p Conclusion The high prevalence and heterogeneous anatomy of coexisting vascular lesions suggest that subtle ultrastructural arterial wall abnormalities may contribute to their development and also predispose to RCVS.

Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882-894.

IMPORTANCE Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-angiographic syndrome characterized by recurrent thunderclap headaches and reversible segmental multifocal cerebral artery narrowing. More than 30% of patients with RCVS develop subarachnoid hemorrhage (SAH). Patients with RCVS with SAH (RCVS-SAH) are often misdiagnosed as having potentially ominous conditions such as aneurysmal SAH (aSAH) or cryptogenic "angiogram-negative" SAH (cSAH) owing to overlapping clinical and imaging features. OBJECTIVE To identify predictors that can distinguish RCVS-SAH from aSAH and cSAH at the time of clinical presentation. DESIGN Retrospective analysis of 3 patient cohorts: patients with RCVS (1998-2009), patients with aSAH (1995-2003), and patients with cSAH (1995-2003). SETTING Academic hospital and tertiary referral center. PARTICIPANTS Consecutive patients with RCVS-SAH (n = 38), aSAH (n = 515), or cSAH (n = 93) whose conditions were diagnosed using standard criteria. MAIN OUTCOMES AND MEASURES Multivariate logistic regression analysis was used to identify predictors that differentiate RCVS-SAH from aSAH and cSAH. RESULTS Predictors differentiating RCVS-SAH from aSAH were younger age, chronic headache disorder, prior depression, prior chronic obstructive pulmonary disease, lower Hunt-Hess grade, lower Fisher SAH group, higher number of affected arteries, and the presence of bilateral arterial narrowing. Predictors differentiating RCVS-SAH from cSAH were younger age, female sex, prior hypertension, chronic headache disorder, lower Hunt-Hess grade, lower Fisher SAH group, and the presence of bilateral arterial narrowing. CONCLUSION AND RELEVANCE We identified important clinical and imaging differences between RCVS-SAH, aSAH, and cSAH that may be useful for improving diagnostic accuracy, clinical management, and resource utilization.

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a self-limited entity with usually benign outcome. Over 30% RCVS patients develop subarachnoid hemorrhage (SAH). We aimed to identify features that differentiate RCVS-SAH from more ominous causes of SAH, i.e. aneurysmal SAH (aSAH) and cryptogenic ‘angio-negative’ SAH (cSAH). Methods: We compared the clinical-imaging features of 38 consecutive RCVS-SAH patients, to 515 aSAH and 93 cSAH patients consecutively admitted to Massachusetts General Hospital. Results: As compared to aSAH and cSAH, the RCVS-SAH group was significantly younger, more women, and higher frequency of migraine, depression, chronic obstructive pulmonary disease (COPD), alcohol and drug exposure, and prior antidepressant use. The distribution of Hunt-Hess (HH) grade and Fisher group were different between groups, with median values highest in the aSAH group. The RCVS-SAH group had more hypodense lesions on 1st head CT and earlier, more severe and widespread vasoconstriction on cerebral angiography. Discharge mRS scores were lowest in the RCVS-SAH group. To avoid overfitting, multivariate logistic regression (Firth’s method) was performed using separate models for clinical and radiological variables given small "N". Predictors of RCVS-SAH vs. aSAH [model 1]: age (O.R. 0.9, 95% C.I. 0.9-0.96), prior headache disorder (O.R. 9.3, 95% C.I. 3.9-22.4), depression (O.R. 5.6, 95% C.I. 1.8-17.6), and COPD (O.R. 7.6, 95% C.I. 2.9-20.1), and [model 2]: HH grade (O.R. 0.4, 95% C.I. 0.2-0.7), Fisher group (O.R. 0.2, 95% C.I. 0.07-0.4), and the number of constricted arteries (O.R. 1.6, 95% C.I. 1.4-1.9). Predictors of RCVS-SAH vs. cSAH [model 1]: age (O.R. 0.9, 95%C.I. 0.9-0.97), prior headache (O.R. 10.3, 95% C.I. 4.3-24.9), depression (O.R. 6.9, 95% C.I. 2.1-22.4), and alcohol use (O.R. 5.1, 95% C.I. 2.0-12.9), and [model 2]: Fisher group (O.R. 0.01, 95% C.I. 0.0-0.6), vasospasm severity (O.R. 9.1, 95% C.I. 1.4-57.2), and the number of constricted arteries (O.R. 2.0, 95% C.I. 1.2-3.1). Conclusion: Several clinical-imaging features distinguish RCVS-SAH from aSAH and cSAH. These data should prove useful to improve the diagnostic accuracy, management, and resource utilization in patients with SAH.

Background: Stroke is a common, serious complication of infective endocarditis (IE). It is not known how recent advances in management have changed the profile and outcome of IE-related stroke. Methods: We retrospectively reviewed data of all inpatients with ICD-9 diagnostic codes of IE admitted to our tertiary hospital from 1980-2011. Original Duke Criteria were used to confirm the diagnosis. Data was compared over 3 time epochs: 1980-1990 (E1), 1991-2000 (E2) and 2001-2011 (E3). Results: Of 1151 hospitalizations (1083 adults), 277 included IE-related stroke (76% ischemic, 13% hemorrhagic, 10% both). There was an increasing rate of stroke over the 3 epochs (E1 25%, E2 30%, E3 45%; p Conclusion: This large retrospective study spanning 3 decades shows important changes in the profile of IE-related stroke. While patient demographics and organism type appear unchanged, there is a higher rate of stroke detection and lower rates of complications such as brain hemorrhage and death rates, suggesting efficacy of recent diagnostic and management advances.

Background: Stroke is a common, serious complication of infective endocarditis (IE). We investigated risk factors for IE-associated cerebrovascular disease. Methods: We retrospectively reviewed data of all inpatients with discharge ICD-9 codes of IE admitted to our tertiary care hospital from 1980-2011. The diagnosis of IE was confirmed using the original Duke Criteria. Stroke was confirmed on CT or MRI. Results: Of 1151 hospitalizations (1083 adults), 277 (24%) included IE-related stroke (76% ischemic, 13% hemorrhagic, 10% both). Stroke patients had mean age 56 yrs, 63% male, 93% ’Definite IE’ per Duke Criteria, and 83% Native Valve infection. Organism types were Staph 40%, Strep 30% and Other 30%. Trans-esophageal echo was performed in 53% and MRI in 57%. The Stroke and Non-Stroke Groups showed no significant difference in age, sex, traditional cardiovascular risk factors (Htn, DM, lipids, AFib etc), and type of infectious organism. Patients with Stroke had a significantly higher frequency of multiple vegetations (20% vs 8%, p Conclusion: This large 31-year retrospective study shows that potentially treatable conditions such as CHF and sepsis are associated with a higher rate of IE-related stroke.

Background: Increases in oxygen extraction fraction (OEF) are postulated to be a marker of tissue at risk of infarction in acute ischemic stroke patients. R2’, a parameter that can be measured rapidly using an asymmetric spin echo MRI sequence (ASE), can be considered an OEF-weighted MRI metric, being a combination of both OEF and venous cerebral blood volume. We investigated the feasibility and utility of OEF-weighted MRI in acute stroke. Methods: Adult ischemic stroke patients were prospectively enrolled in a 3T MRI study of patients imaged within 48 hours of stroke onset. Only the subset of patients whose MRI included ASE and whose DWI was positive for acute infarction was analyzed. Acquisition time for ASE was 1.5 min. R2’ maps was calculated from ASE data on a voxel-wise basis after motion correction and smoothing. DWI and follow-up images (FU), when available, were co-registered to the ASE. Mean R2’ values and coefficient of variance (mean/SD) within the lesion, ipsilateral DWI-normal (IPS) and contralesional hemisphere (CNL) were calculated and compared. Results: Fifteen subjects met the inclusion criteria with mean±SD age 64±14 years, median admission NIHSS 12 [IQR 7-15] and time from onset to MRI 39±30 h. Eleven patients were imaged after tPA therapy. Both elevated and decreased R2’ were observed within DWI and FU lesions. Mean R2’ values in the DWI lesion (6.0±1.5 Hz) were significantly lower than CNL (7.2±0.6 Hz, P=0.02) and IPS (7.2±0.7 Hz, P=0.01). Coefficient of variance in R2’ values in the DWI lesion were also significantly lower than IPS (P=0.03) or CNL (P=0.05). For the 12 subjects with follow-up imaging, mean R2’ in tissue that eventually infarcted (5.7±1.8 Hz) was significantly lower (P=0.02) than in ipsilateral tissue surrounding the eventually infarcted lesion (7.3±0.6 Hz) but not significantly lower than DWI lesion values (6.1±1.6 Hz). Discussion: In this study using ASE, decreased OEF-weighted MRI values can potentially reflect tissue that is destined for infarction since OEF is proportional to cerebral oxygen metabolic rate. Increased OEF-weighted MRI values may be a sign of tissue that is at risk of infarction. Studies including measures of CBF and CBV will allow for better discrimination of tissue status.

BACKGROUND l-2-Hydroxyglutaric aciduria is a rare progressive neurometabolic disorder of childhood inherited as an autosomal recessive trait. Urine organic-acid screening is necessary for its diagnosis. Although it is a disorder of childhood, recently adult cases have been reported. CASES Here we report 4 adult patients in whom diagnoses were established in adulthood. These patients had some interesting features. First, their diagnoses were delayed until adulthood because of mild clinical symptoms. In such cases, the typical MRI findings are the best diagnostic clue for l-2-Hydroxyglutaric aciduria. Second, there was a correlation between the severity of the clinical course and the extent of MRI findings. The cerebral white-matter lesions were diffuse and confluent on the MRI of 3 of the 4 patients, who also experienced a rapidly progressive clinical decline. Third, there were different clinical presentations even within the same family. CONCLUSIONS For the evaluation of patients with symptoms referable to cerebellar, pyramidal, extrapyramidal, or cognitive impairment as well as seizures associated with subcortical white-matter and symmetrical dentate nuclei and basal ganglia involvement on MRI, urine organic acid analysis should be included in the evaluation, regardless of patient's age.

Creutzfeldt-Jakob disease is the most common disorder among the very rare human transmissible subacute spongiform encephalopathies. Sporadic, familial or iatrogenic forms of the disease can be seen. The common presentations of the disease include rapidly progressive cognitive decline, behavioral changes, visual disturbances and cerebellar dysfunction. Hyperparathyroidism and toxicity of lithium and bismuth have been reported to induce similar symptoms and EEG findings, leading to a Creutzfeldt-Jakob like syndrome. We report a very rare case of Creutzfeldt-Jakob disease presenting with hyperparathyroidism and generalized tonic status epilepticus.