High-dose diazepam controls severe dyskinesia in Anti-NMDA receptor encephalitis

2020 ◽  
pp. 10.1212/CPJ.0000000000001001
Author(s):  
Hye-Rim Shin ◽  
Yoonhyuk Jang ◽  
Yong-Won Shin ◽  
Kon Chu ◽  
Sang Kun Lee ◽  
...  

ObjectiveSince there is no standard treatment to control dyskinesia in anti-NMDA receptor (NMDAR) encephalitis, we analyzed therapeutic efficacy of high-dose diazepam in dyskinesia associated with NMDAR encephalitis.MethodsWe reviewed NMDAR encephalitis patients with dyskinesia, who were admitted to Seoul National University Hospital between November 2012 and July 2018. High-dose diazepam was administered orally or via a nasogastric tube, 3–6 times a day. We assessed the treatment effect by comparing dyskinesia severity on the first day when the diazepam treatment reached the highest dose, with after 1 week of highest dose of diazepam treatment.ResultsAmong 68 NMDAR encephalitis patients during study period, 33 patients were treated with enteral diazepam (ranging from 6 mg to 180 mg) to control dyskinesia, along with immunotherapy. The severity of dyskinesia improved from average grade 2.4 ± 0.6 to 1.1 ± 0.7, after 1 week of the highest dose of diazepam (mean severity change −1.4 ± 0.6, 95% confidence interval −1.2 to −1.6; p < 0.001). No patients had serious adverse events except mild sedation.ConclusionsDyskinesia in NMDAR encephalitis improved after treatment with enteral diazepam without significant side effects. This study suggests enteral diazepam could be a treatment option for control dyskinesia in NMDAR encephalitis.Classification of evidenceThis study provides Class IV evidence that for patients with dyskinesias associated with NMDAR encephalitis, enteral diazepam is effective and safe in dyskinesia control.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5084-5084
Author(s):  
Hee Won Moon ◽  
Tae Young Kim ◽  
Seong- Ho Kang ◽  
Hyun-Sook Chi ◽  
Eul Zu Seo ◽  
...  

Abstract Recent studies proposed the classification of multiple myeloma (MM) by the pathways involved in the early pathogenesis; nonhyperdiploid variants with a high incidence of IgH translocations and hyperdiploid variants associated with no IgH translocation. Most studies applied cytogenetic study or flow cytometry to define the ploidy. In this study, we combined the cytogenetic results and fluorescent in situ hybridization results to define the ploidy and investigated IgH tranlocation and 13q deletion in relation to the ploidy level on Korean patients with MM. A total of 135 cases diagnosed as MM between 1997 and 2003 from Seoul National University Hospital and the Asan Medical center were enrolled in this study. Conventional cytogenetic studies and FISH studies with different probes specific for the regions containing the genes or chromosomes (RB1, D13S319, D13S25, IgH/FGFR3, IgH/BCL2, IGH dual color, break apart rearrangement probe, IgH/CCND1, 1q, p53, p16, MLL, CEP 7, 11, 12) were performed. Of 135 patients with MM, 62 (45.9%) patients had hyperdiploid karyotype by cytogenetics and FISH. IgH translocations were observed in 37.4% of Korean patients with MM and were more frequent (54.7%) in hyperdiploid variants than in nonhyperdiploid variants (17.4%). Incidence of deletion 13q was 34.7% and also more frequent in hyperdiploid variants (54.2%) than in nonhyperdiploid variants (16.1%). In conclusion, IgH translocations and 13q deletions were not associated with nonhyperdiploid MM and appeared more frequently in hyperdiploid variant in Korean patients with MM.


Biomolecules ◽  
2019 ◽  
Vol 9 (10) ◽  
pp. 572 ◽  
Author(s):  
Wang

MicroRNA (miRNA) is a small non-coding RNA that functions in the epigenetics control of gene expression, which can be used as a useful biomarker for diseases. Anti-NMDA receptor (anti-NMDAR) encephalitis is an acute autoimmune disorder. Some patients have been found to have tumors, specifically teratomas. This disease occurs more often in females than in males. Most of them have a significant recovery after tumor resection, which shows that the tumor may induce anti-NMDAR encephalitis. In this study, I review microRNA (miRNA) biomarkers that are associated with anti-NMDAR encephalitis and related tumors, respectively. To the best of my knowledge, there has not been any research in the literature investigating the relationship between anti-NMDAR encephalitis and tumors through their miRNA biomarkers. I adopt a phylogenetic analysis to plot the phylogenetic trees of their miRNA biomarkers. From the analyzed results, it may be concluded that (i) there is a relationship between these tumors and anti-NMDAR encephalitis, and (ii) this disease occurs more often in females than in males. This sheds light on this issue through miRNA intervention.


2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).


1991 ◽  
Vol 38 (2) ◽  
pp. 119-127
Author(s):  
Yong Chol Han ◽  
Chul Gyu Yoo ◽  
Young Whan Kim ◽  
Sung Koo Han ◽  
Young Soo Shim ◽  
...  

Stroke ◽  
2016 ◽  
Vol 47 (suppl_1) ◽  
Author(s):  
Han-Gil Jeong ◽  
Beom-Joon Kim ◽  
Chi Kyung Kim ◽  
Jun Yup Kim ◽  
Dong-Wan Kang ◽  
...  

Background: Red thrombi, composed of fibrin and trapped erythrocytes, have magnetic susceptibility effect. Susceptibility vessel sign (SVS) is visualized more sensitively using susceptibility weighted imaging (SWI) than T2*-weighted imaging. Bright vessel appearance (BVA) on arterial spin labeling (ASL) imaging can visualize occluded arterial segment by arterial transit artifact, more sensitively in small and peripheral branches. We investigated the usefulness of SWI-SVS with BVA to visualize different thrombus and predict stroke mechanisms. Methods: From a total of 564 stroke cases who admitted to Seoul National University Hospital in 2014, the authors collected eligible cases with the following inclusion criteria; (1) Lesion-documented ischemic stroke (N=425); (2) SWI and ASL MRI performed (N=407); (3) Symptomatic arterial occlusion with BVA (N=141). All images were analyzed for the presence and location of SWI-SVS and BVA. The location of SWI-SVS and BVA were classified into (1) proximal, large arteries; distal ICA, M1/2, A1, P1, basilar artery, V4 and (2) peripheral, small arteries; M3/4, P2/3, A2/3, lenticulostriate arteries, three cerebellar arteries. The relationships between SWI-SVS in the presence of BVA and stroke etiologies are explored. Results: Male was 58.2% (n=82) and mean age was 65.7±14.3. Thirty-four percent (n=48/141) of BVA and 30.3% (n=30/99) of SVS was located within small, peripheral arteries. SWI-SVS was more commonly associated with other determined etiology (20.2% vs. 4.8%) and cardioembolism (39.4% vs. 14.3%), but less with large artery atherosclerosis (26.3% vs. 69.0%, P <0.01) compared to the patients without SWI-SVS. Cancer-related hypercoagulability (60%, n=12/20) was most common in other determined cases with SWI-SVS. Multivariate analysis showed that SWI-SVS was an independent predictor of other determined etiology (adjusted OR, 7.20; 95% CI, 1.48-34.99) and cardioembolism (adjusted OR, 5.76; 95% CI, 1.27-26.02) Conclusions: SWI-SVS with BVA may predict ischemic stroke of cardioembolism and other determined etiology. Occlusions of small, peripheral arteries are well visualized with BVA and composition of thrombus can be identified by SWI-SVS.


Neurology ◽  
2019 ◽  
Vol 94 (9) ◽  
pp. e942-e949 ◽  
Author(s):  
Hyo-Jung Kim ◽  
Jeong-Mi Song ◽  
Liqun Zhong ◽  
Xu Yang ◽  
Ji-Soo Kim

ObjectivesTo develop a simple questionnaire for self-diagnosis of benign paroxysmal positional vertigo (BPPV).MethodsWe developed a questionnaire that consisted of 6 questions, the first 3 to diagnose BPPV and the next 3 to determine the involved canal and type of BPPV. From 2016 to 2017, 578 patients with dizziness completed the questionnaire before the positional tests, a gold standard for diagnosis of BPPV, at the Dizziness Clinic of Seoul National University Bundang Hospital.ResultsOf the 578 patients, 200 were screened to have BPPV and 378 were screened to have dizziness/vertigo due to disorders other than BPPV. Of the 200 patients with a questionnaire-based diagnosis of BPPV, 160 (80%) were confirmed to have BPPV with positional tests. Of the 378 patients with a questionnaire-based diagnosis of non-BPPV, 24 (6.3%) were found to have BPPV with positional tests. Thus, the sensitivity, specificity, and precision of the questionnaires for the diagnosis of BPPV were 87.0%, 89.8%, and 80.0% (121 of 161, 95% confidence interval 74.5%–85.5%). Of the 200 patients with a questionnaire-based diagnosis of BPPV, 30 failed to respond to the questions 4 through 6 to determine the involved canal and type of BPPV. The questionnaire and positional tests showed the same results for the subtype and affected side of BPPV in 121 patients (121 of 170, 71.2%).ConclusionThe accuracy of questionnaire-based diagnosis of BPPV is acceptable.Classification of evidenceThis study provides Class III evidence that, in patients with dizziness, a questionnaire can diagnose BPPV with a sensitivity of 87.0% and a specificity of 89.8%.


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