We read with great interest the study by Lim et al, 1 which aimed to develop an easily applicable scale to grade the severity of autoimmune encephalitis (AE). Nine key clinical features were identified and included in the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). CASE showed good interobserver and intraobserver reliability, and internal consistency, as well as a high correlation with the modified Rankin Scale (mRS).We applied CASE retrospectively to a cohort of 60 patients with AE, admitted to our institution between 2012 and 2018, and prospectively in 3 additional patients. Median age at onset was 55 years (range = 3 months-88 years), in 13 of 63 (21%) onset was before 18 years of age, and 34 of 63 (54%) were female. All patients fulfilled the clinical diagnostic criteria for AE 2 : definite AE was diagnosed in 16 of 63 patients (25%), definite anti-NMDA receptor encephalitis in 11 of 63 (17%), definite autoimmune limbic encephalitis in 6 of 63 (10%), antibody-negative probable AE in 4 of 63 (6 %), possible AE in 25 of 63 (40%), and Hashimoto encephalopathy in 1 of 63 (2%). At the disease nadir, median mRS score was 4 (range = 3-5). We applied CASE at maximum clinical severity (median CASE score = 9, range = 3-24). In our cohort, CASE significantly correlated with mRS (p < 0.0001, r 2 = 0.5025; Fig), although the correlation strength was weaker than that reported by Lim et al. However, we encountered several issues when CASE was applied to our patients that need to be clarified. Severe clinical conditions, such as coma and status epilepticus, make other items hardly assessable (eg, gait instability, language). In such cases, it is not specified what score should be given to items that are not directly assessable (we gave the maximum score). Postictal state after seizures also interferes with the evaluation of most items. The authors should clarify how to apply the scale in these situations and whether this score is meant to be used only in clinically stable patients. Furthermore, when applying CASE to a pediatric population, some items, such as memory and language, were not easily assessable. We think that the scale is more suitable for adult patients. Moreover, it would be appropriate to develop a different scale for patients with altered consciousness.CASE is potentially a useful tool and addresses the unmet need of clinical scales to grade AE severity, as mRS is quite coarse in evaluating this condition. However, CASE needs further improvement and validation to be employed in clinical trials.

Objectives: The purpose of this study was to determine the relative accuracies of mammography, sonography, MRI and clinical examination in predicting residual tumour size and pathological response after neoadjuvant chemotherapy for locally advanced or inflammatory breast cancer. Each prediction method was compared with the gold standard of surgical pathology. Methods: 43 patients (age range, 25-62 years; mean age, 42.7 years) with locally advanced or inflammatory breast cancer who had been treated by neoadjuvant chemotherapy were enrolled prospectively. We compared the predicted residual tumour size and the predicted response on imaging and clinical examination with residual tumour size and response on pathology. Statistical analysis was performed using weighted kappa statistics and intraclass correlation coefficients (ICC). Conclusion:Predictions of response and residual tumour size made on MRI were better correlated with the assessments of response and residual tumour size made upon pathology than were predictions made on the basis of clinical examination, mammography or sonography. Thus, the evaluation of predicted response using MRI could provide a relatively sensitive early assessment of chemotherapy efficacy.

Purpose To evaluate the relationship between penetrating arterial pulsation and the progression of white matter hyperintensities (WMHs) by using the sonographic resistance index (RI) along the M1 segment of the middle cerebral artery (MCA). Materials and Methods The study design was approved by the institutional review board of Seoul National University Hospital. The study included 450 individuals who had undergone initial transcranial Doppler (TCD) sonography and magnetic resonance imaging, with follow-up imaging performed within 34-45 months, and who had no stenosis of 30% or more in the internal carotid artery or MCA or a history of stroke other than an old lacunar infarction. MRIR was defined as distal RI divided by proximal RI, where the distance between proximal MI and distal M1 was approximately 20 mm based on TCD evaluation. WMH progression was quantitatively evaluated by subtracting WMH volume at baseline from WMH volume at follow-up. Results At baseline, mean MRIR was 0.974 ± 0.045 (standard deviation), and mean WMH volume was 9.66 mL ± 14.54. After a mean of 38.3 months ± 3.4, the WMH volume change was 4.06 mL ± 7.35. WMH volume change was linearly associated with MRIR (r = 0.328, P < .001), along with the baseline WMH volume (r = 0.433, P < .001) and mean MCA pulsatility index (r = 0.275, P = .037). MRIR values greater than or equal to 1.000 were associated with a greater increase in WMH volume (P < .001). Conclusion MRIR might reflect the pulsation of penetrating arteries and is independently associated with WMH progression. RSNA, 2017 Online supplemental material is available for this article.

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Cancer related stroke may have different phenotypes from non-cancer stroke, especially in terms of stroke progression and recurrence. We performed a case-control study to identify their incidences and risk factors in cancer related stroke. Between January 2001 and December 2009, we conducted a retrospective review of acute ischemic stroke patients with cancer who were admitted to Seoul National University Hospital, Seoul, Korea. The stroke patients without cancer served as control. We collected demographic variables, vascular risk factors, stroke phenotype, clinical course, and cancer information including diagnosis, stage, and treatment status. Among cancer stroke patients, the potential risk factor of stroke recurrence was evaluated. The mean age of the 102 cancer patients was 66.4 ± 10.8 years, and 64.7 % were men. The mean time interval from cancer diagnosis to stroke onset was 39.7 ± 60.9 months. The principal lesion pattern of cancer stroke was multiple dots extending single vascular territory (39.2 %), and they were associated with low hemoglobin and high fibrinogen levels. Stroke progression and recurrence were noted in 9.8 and 27.5 % of cancer stroke patients, and in 9.3 and 12.7 % of control patients, respectively. The stroke subtype was independently associated with recurrence of cancer stroke after multiple logistic regression (odds ratio = 3.165, 95 % confidence interval = 1.080-9.277, p = 0.036). Cancer related stroke has a distinct phenotype in terms of infarction pattern and laboratory findings. Stroke recurrence is frequently observed among cancer stroke patients, and its risk is related with stroke subtype.

Inhibitory synaptic receptors are dysfunctional in epileptic brains, and agents that selectively target these receptors may be effective for the treatment of epilepsy. MicroRNAs interfere with the translation of target genes, including various synaptic proteins. Here, we show that miR-203 regulates glycine receptor-β (Glrb) in epilepsy models. miR-203 is upregulated in the hippocampus of epileptic mice and human epileptic brains and is predicted to target inhibitory synaptic receptors, including Glrb. In vitro transfection, target gene luciferase assays, and analysis of human samples confirmed the direct inhibition of GLRB by miR-203, and AM203, an antagomir targeting miR-203, reversed the effect of miR-203. When intranasal AM203 was administered, AM203 reached the brain and restored hippocampal GLRB levels in epileptic mice. Finally, intranasal AM203 reduced the epileptic seizure frequency of mice. Overall, this study suggests that GLRB expression in the epileptic brain is controlled by miR-203, and intranasal delivery of AM203 showed therapeutic effects in chronic epilepsy mice.

IMPORTANCEThe cerebral white matter hyperintensity (WMH) is frequently noted in patients with chronic heart disease. Long-term alteration of cardiac hemodynamics might have an influence on the mechanism of cerebral WMH.OBJECTIVE To investigate the association between chronically altered cardiac hemodynamics and severity of cerebral WMH in patients with chronic valvular heart disease. DESIGN, SETTING, AND PARTICIPANTSThis cross-sectional analysis identified 303 consecutive patients at a tertiary referral center between 2008 and 2016 who were 50 years or older, and diagnosed with severe chronic valvular heart disease and underwent cardiac catherization, echocardiography, and received brain magnetic resonance imaging. Among these patients, 71 with other demonstrated cardiac disease, central nervous system disease, and/or without sufficient catheterization data were excluded, and the remaining 232 patients were included in further analyses.EXPOSURES The site and mechanism of valve diseases, as well as clinical and medication profiles, were reviewed. Cardiac catheterization parameters such as right atrial (RA) mean pressure, right ventricular pressure, and aortic mean pressure were obtained. Comprehensive echocardiographic hemodynamic markers such as left ventricular (LV) ejection fraction, LV mass index, LV end diastolic volume, cardiac index, and E/e′ ratio were also obtained. MAIN OUTCOMES AND MEASURESWhite matter hyperintensity volume was quantitatively evaluated using volumetric analysis. RESULTSThis study included 232 patients (103 men [44.4%] and 129 women [55.6%]; mean [SD] (range) age, 65.6 [8.8] (51-88) years) in the final analysis. The mean (SD) WMH volume was 5. 93 (7.14) mL (median [interquartile range], 4.33 [1.33-8.62] mL), and mean (SD) RA pressure was 10.0 (4.7) mm Hg. From the catheterization data, 147 patients (63.4%) were classified as having a disease involving the mitral valve; 93 (40.1%), aortic valve; 37 (15.9%), tricuspid valve; and 4 (1.7%), pulmonary valve. In multivariate linear regression analysis, adjusting the type and mechanism of valve disease and clinical, echocardiographic, and/or other catheterization parameters, WMH volume was linearly associated with mean RA pressure (B coefficient, 0.702; 95% CI, 0.373-1.031; P = .001), along with age (B coefficient, 0.145; 95% CI, 0.029-0.261; P = .01) and mean aortic pressure (B coefficient, 0.112; 95% CI, 0.034-0.190; P = .005).CONCLUSIONS AND RELEVANCE Mean RA pressure was independently associated with the WMH volume in chronic valvular heart disease. Chronically altered RA hemodynamics might have a distinct influence on the pathomechanism underlying the development of WMH.

Background and Purpose-The high prevalence of intracranial aneurysms (IAs) in patients with a bicuspid aortic valve or coarctation of the aorta suggests a link between IA and aortic pathology. However, studies reporting this link do not sufficiently address the heterogeneity of IAs arising from different anatomic locations. This study aimed to explore whether a location-specific relationship exists between the 2 kinds of aneurysms. Methods-Retrospective institutional analysis of patients aged ≥18 years with both IA and an aortic aneurysm (AA) was performed from 2005 to 2014. IAs were categorized based on their locations: internal carotid artery, other anterior circulation, and posterior arteries. AAs were classified as ascending, descending, infrarenal, or multiple. We analyzed the clinical characteristics and the distribution of IA in each AA group. Results-Of 2375 patients, 660 with available intracranial angiography were screened for IA. We identified 71 patients with 97 IAs. The frequency of both anterior circulation-IAs and internal carotid artery-IAs differed significantly among the AA groups (P=0.001 and P=0.01, respectively). Anterior circulation-IAs were most frequently observed in ascending AA group and least frequently in infrarenal AA group. In contrast, internal carotid artery-IAs were found mostly in infrarenal AA group, least in ascending AA group. Proportions of patients having anterior circulation-IA and internal carotid artery-IA were also highest in ascending AA group and infrarenal AA group, respectively. The number of posterior arteries-IAs was too small to characterize. Conclusions-The differing distribution patterns of IA among AA groups suggest a site-specific sharing of pathomechanism between the 2 types of aneurysms.