Acta neurologica Taiwanica最新文献_第7页

Macrophage Activation Syndrome in a Case of Myasthenia Gravis with Concurrent Cytomegalovirus Infection. 巨噬细胞激活综合征并发巨细胞病毒感染重症肌无力1例。

Acta neurologica Taiwanica Pub Date : 2020-12-01

Nai-Hsin Huang, Li-Ming Lien, Wen-Hung Chen

{"title":"Macrophage Activation Syndrome in a Case of Myasthenia Gravis with Concurrent Cytomegalovirus Infection.","authors":"Nai-Hsin Huang, Li-Ming Lien, Wen-Hung Chen","doi":"","DOIUrl":"","url":null,"abstract":"Objective: Macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) refers to a set of clinical symptoms caused by the excessive activation and proliferation of macrophages. It was linked with autoimmune disease such as systemic-onset juvenile rheumatoid arthritis, systemic lupus erythematosus, rheumatoid arthritis, and dermatomyositis, etc. Herein we report a case of myasthenia gravis (MG) with concurrent cytomegalovirus (CMV) infection developed MAS.Case report: A 31-year-old female with history of MG for 2 years under stable control with azathioprine and prednisolone. She presented with persistent high fever for 2 weeks after an upper respiratory infection. Lab data revealed pancytopenia, elevated triglyceride, ferritin and C-reactive protein (CRP). A bone marrow aspiration confirmed hemophagocytosis. Investigation for occult infection revealed her plasma was positive for CMV IgG and IgM, and high for CMV viral load. She was then treated with 5 sessions of plasmapheresis and pulse steroid. Azathioprine was discontinued and replaced with cyclosporine. Gancylovir was given for her concurrent CMV infection. After 2 weeks of treatment, her fever gradually subsided, and her blood cell count, hepatobiliary enzymes, ferritin and CRP have returned to normal range. She was discharged in good recovery.Conclusion: MAS is a rare complication of systemic autoimmune disease with poor prognosis, which may be precipitated by concurrent infection. Early recognition of this syndrome and prompt immune modulation therapy is crucial for successful treatment.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(4) ","pages":"114-118"},"PeriodicalIF":0.0,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39017270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Significant Destructive Interaction of BDNF Val>Met Polymorphism with Stroke Severity and Family History of Dementia for Cognitive Impairments. BDNF Val>Met多态性与脑卒中严重程度和认知障碍痴呆家族史的显著破坏性相互作用

Acta neurologica Taiwanica Pub Date : 2020-09-30

Sajjad Rezaei, Karim Asgari Mobarake, Alia Saberi, Parvaneh Keshavarz

{"title":"Significant Destructive Interaction of BDNF Val>Met Polymorphism with Stroke Severity and Family History of Dementia for Cognitive Impairments.","authors":"Sajjad Rezaei, Karim Asgari Mobarake, Alia Saberi, Parvaneh Keshavarz","doi":"","DOIUrl":"","url":null,"abstract":"Purpose: The patients with more severe stroke, have more chance to develop higher levels of cognitive impairments; and family history of dementia as a genetic background, can give rise to an increased risk of the severity of cognitive deterioration. In this study, we sought to investigate whether the risk alleles of Val66Met of brain-derived neurotrophic factor (BDNF) polymorphism, has a destructive interaction with the stroke severity (SS) and family history of dementia (FHD) for cognitive impairments?Method: In a case-control study, the carriers of at least one Val allele (n=56) were compared to the carriers of Met/Met homozygotes (n=156) in terms of FHD and SS (through National Institutes of Health Stroke Scale) on the north of Iran. To determine the cognitive functions, the third version of Addenbrooke's Cognitive Examination (ACE-III) was used.Result: The mean age of patients was 64.52±11.71, and in average 202 day had passed from their stroke. The interactive effects of genotypes Val66Met BDNF with SS[F=8.95, ή2=0.04, P=0.003] and FHD[F=4.59, ή2=0.02, P=0.03] were significant for total score of ACE-III. It means that the Met/ Met homozygosity, modulated the effect of risk factors of SS and FHD on the cognitive function. Such homozygosity protects the attentional function and language abilities against the SS and FHD(P≤0.05).Conclusion: It can be speculated that presence of Val/Met heterozygosity has a destructive interaction with the SS and FHD for decreasing the cognitive function, particularly in attention and language domains. Our findings suggested that the inhibition of signaling and trafficking of Val/Met heterozygosity is possibly a practical strategy in reducing the cognitive impairments following the stroke.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(3) ","pages":"67-78"},"PeriodicalIF":0.0,"publicationDate":"2020-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38437097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Wernekink Commissure Syndrome: A Rare Cause of Bilateral Cerebellar Syndrome. Wernekink连接综合征:双侧小脑综合征的罕见病因。

Acta neurologica Taiwanica Pub Date : 2020-09-01

Kefah Alhayek, Belal Aldabbour, Haneen Al Refa'I Refa'I, Maram Alanbar

{"title":"Wernekink Commissure Syndrome: A Rare Cause of Bilateral Cerebellar Syndrome.","authors":"Kefah Alhayek, Belal Aldabbour, Haneen Al Refa'I Refa'I, Maram Alanbar","doi":"","DOIUrl":"","url":null,"abstract":"Purpose: Focal signs are a big deal in neurology and are among the most important clues leading to diagnosis and localization. Wernekink commissure syndrome is due to lesions in the caudal paramedian midbrain involving the entire decussation of the superior cerebellar peduncles, resulting in the clinical hallmark of a bilateral cerebellar syndrome.Case report: A 79-year-old man presented with sudden, severe unsteadiness associated with slurring of speech, binocular double vision, and bilateral hand tremor. Examination showed right INO, moderately severe dysarthria, bilateral dysmetria and dysdiadochokinesia, with severe truncal ataxia and bilateral upper and lower limb ataxia. Also, bilateral coarse tremor was noted in both hands which was present at rest, action and on reaching for objects. Brain MRI revealed an acute infarction involving the Wernekinck decussation in the right caudal midbrain and mesencephalo-pontine junction.Conclusion: The differential of Wernekink Commissure Syndrome is complex, and localization and lateralization are extremely difficult owing to prominent bilateral cerebellar symptoms. The finding of an associated unilateral INO in some cases makes it possible to confidently narrow the list of differentials and localize the lesion to the paramedian tegmentum ipsilateral to the non-adducting eye.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(3) ","pages":"86-89"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38437099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Severe Necrosis and Cellulitis Complicating Treatment with Interferon β-1a. 干扰素β-1a治疗严重坏死和蜂窝织炎。

Acta neurologica Taiwanica Pub Date : 2020-09-01

Chun-Chen Chang, Chien-Hui Lin, Jen-Jen Su

引用次数: 0

Mathematical Framework of Deconvolution Algorithms for Quantification of Perfusion Parameters. 灌注参数定量反卷积算法的数学框架。

Acta neurologica Taiwanica Pub Date : 2020-09-01

Fanpei Yang, Sukhdeep Singh Bal Bal, Yueh-Feng Sung, Giia-Sheun Peng

{"title":"Mathematical Framework of Deconvolution Algorithms for Quantification of Perfusion Parameters.","authors":"Fanpei Yang, Sukhdeep Singh Bal Bal, Yueh-Feng Sung, Giia-Sheun Peng","doi":"","DOIUrl":"","url":null,"abstract":"Purpose: MR perfusion weighted imaging (PWI) has been used as sensitive indicator of tissue at risk for infarction. Quantitative perfusion parameters such as cerebral blood flow (CBF), mean transit time (MTT) and cerebral blood volume (CBV) can be obtained from post processing of PWI data using standard singular value decomposition algorithm (SVD). Assumption regarding absence of arterial - tissue delay (ATD) used in SVD algorithm results in underestimation of perfusion parameters. To estimate accurate values for perfusion parameters it is important to understand the mathematical framework behind SVD and improved SVD algorithms (bSVD and rSVD).Method: This study explains the mathematical framework of SVD and improved SVD algorithms and uses computational techniques that use bSVD algorithm to obtain perfusion parameters maps of CBF, CBV and MTT for acute stroke patient.Result: Computational techniques based on mathematical deconvolution algorithms are used to post process CBV, CBF and MTT maps where decrease in CBF and CBV were seen in left hemisphere.Conclusion: The bSVD algorithm is found to be sensitive to ATD and provides more accurate estimates of perfusion parameters than the SVD algorithm, however CBF estimates from bSVD and rSVD still remain influenced by other artifacts Keywords: PWI = perfusion weighted imaging, CBF= cerebral blood flow, MTT = mean transit time, CBV= cerebral blood volume, SVD = singular value decomposition algorithm.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(3) ","pages":"79-85"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38437098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Atypical Spinal Cord Infarction: A Prolonged and Stuttering Course for Six Days. 非典型脊髓梗塞:一个延长和口吃的过程，为期6天。

Acta neurologica Taiwanica Pub Date : 2020-09-01

Meng-Chi Chiu, Chi-Yuan Liang, Yung-Sung Huang, Wen-Long Tsao

引用次数: 0

Study of the Demographic and Clinical Profile in a Neurocutaneous Rare Disease: A Cross-Sectional Study. 一种神经皮肤罕见疾病的人口学和临床特征研究:一项横断面研究。

Acta neurologica Taiwanica Pub Date : 2020-08-01 DOI: 10.21203/rs.3.rs-45681/v1

L. Jouybari, S. Foji, A. Sanagoo, Morteza oladenabidozin, Alireza Yazdani

{"title":"Study of the Demographic and Clinical Profile in a Neurocutaneous Rare Disease: A Cross-Sectional Study.","authors":"L. Jouybari, S. Foji, A. Sanagoo, Morteza oladenabidozin, Alireza Yazdani","doi":"10.21203/rs.3.rs-45681/v1","DOIUrl":"https://doi.org/10.21203/rs.3.rs-45681/v1","url":null,"abstract":"OBJECTIVE\u0000Neurofibromatosis is one of the most common dominantly inherited genetic disorders. This study aimed to study the demographic and clinical profile of neurofibromatosis patients.\u0000\u0000\u0000METHODS\u0000This study is cross-sectional conducted in 2020 on the population of patients with neurofibromatosis. Patients who are members of the Neurofibromatosis Association answered the online demographic and clinical information questionnaire.\u0000\u0000\u0000RESULTS\u0000446 patients with neurofibromatosis participated in this study with a mean age of 33.39 plus or minus 12.87 years. 297 patients (66.6%) were women and 378 (84.8%) patients had type 1 neurofibromatosis. The disease visibility was reported to be moderate in 254 patients (54.9%) and the severity of the disease was mild in 238 (53.4%) patients. The type of neurofibromatosis was not significantly related to gender, age groups, parental education, and ethnicity. The relationship between severity and age (p is equal to less than 0.001) and gender (p is equal to 0.042) was significant and the relationship between visibility and age (p is equal to less than 0.001) was significant but despite the fact that the disease was more visible in men than women, it was not significantly related to gender.\u0000\u0000\u0000CONCLUSIONS\u0000The study results showed that the most common complication in the study population was Cafe au lait spot. In addition, visibility and severity of the disease were mild and moderate, respectively. Keyword: Neurofibromatosis, Demographic information, Clinical Information.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"70 1","pages":"15-23"},"PeriodicalIF":0.0,"publicationDate":"2020-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85554537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Apilarnil: A Novel Neuroprotective Candidate. 阿非拉尼尔:一种新的神经保护候选者。

Acta neurologica Taiwanica Pub Date : 2020-06-30

Mehmet Hamamci, Zuleyha Doganyigit, Sibel Silici, Aslı Okan, Emin Kaymak, Seher Yilmaz, Adem Tokpinar, Levent Ertuğrul Inan

{"title":"Apilarnil: A Novel Neuroprotective Candidate.","authors":"Mehmet Hamamci, Zuleyha Doganyigit, Sibel Silici, Aslı Okan, Emin Kaymak, Seher Yilmaz, Adem Tokpinar, Levent Ertuğrul Inan","doi":"","DOIUrl":"","url":null,"abstract":"Purpose: This study was designed to investigate the effect of apilarnil on neuronal damage and related mechanisms in a sepsis model in order to demonstrate whether or not apilarnil has neuroprotective effect.Methods: In this study, 64 adult male Sprague-Dawley species rats were randomly divided into eight groups. The rats were administered apilarnil and/or lipopolysaccharide (LPS). Superoxide dismutase (SOD), catalase (CAT), malondialdehyde (MDA), xanthine oxidase (XOD) and testican-1 levels were measured in the brain tissue. Proinflammatory cytokines (tumor necrosis factor alpha [TNF-α], interleukin 1 beta [IL-1β], interleukin 6 [IL-6]) were measured in brain tissue. Histological examinations were performed on hippocampus and cortex tissues in all groups. Apoptotic cell count was estimated using the Tunel method to observe the apilarnil's effect on apoptosis. Purkinje cells were counted in the hippocampus to measure the protective effect of apilarnil on the hippocampus.Results: Apilarnil reduced the decrease in SOD and CAT levels in the brain developing sepsis. Apilarnil reduced the increase in MDA, XOD, and testican-1 levels in the septic brain. It was observed that the number of degenerated neurons due to sepsis decreased as apilarnil dose increased. Apilarnil reduced the elevated levels of proinflammatory cytokines (IL-6, TNF-α, IL-1β) induced by sepsis. Apilarnil prevented sepsis-related apoptosis in the brain.Conclusion: The neuroprotective potential of apilarnil against brain damage in the sepsis model was demonstrated and suggested that it has the potential to contribute to new therapeutic targets against various neurological disorders.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(2) ","pages":"33-45"},"PeriodicalIF":0.0,"publicationDate":"2020-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37959035","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Does Early Recognition of Treatment Failure and Changing Anti-Epilepsy Medication Regimen Improve Short-Term Seizure Remission Rates In Childhood Absence Epilepsy? 早期识别治疗失败和改变抗癫痫药物治疗方案能提高儿童缺席癫痫的短期发作缓解率吗?

Acta neurologica Taiwanica Pub Date : 2020-06-01

Suresh Gurbani, Sirichai Chayasirisobhon, Aditya Gurbani, Noriko McCall

{"title":"Does Early Recognition of Treatment Failure and Changing Anti-Epilepsy Medication Regimen Improve Short-Term Seizure Remission Rates In Childhood Absence Epilepsy?","authors":"Suresh Gurbani, Sirichai Chayasirisobhon, Aditya Gurbani, Noriko McCall","doi":"","DOIUrl":"","url":null,"abstract":"Purpose: To study the role of early serial EEGs in improving seizure freedom rates after initiation of ethosuximide or valproic acid for childhood absence epilepsy.Methods: Retrospective data analysis study of AED naive patients with childhood absence epilepsy undergoing treatment at the community-based epilepsy clinic. Due to small sample size Fisher's exact test was used to determine two-tailed p value at less than 0.05 statistical significance..Results: At 2-month study period 71.4% patients in the ethosuximide and 87.5% in the valproic acid group achieved seizure freedom, with EEG normalization in 21.4% and 50% respectively. At 6-month study period, in patients continuing ethosuximide, seizure freedom and EEG normalization rates were 89.5% and 52.6% respectively; while in patients continuing valproic acid, results were 100% and 78.6% respectively. Both at 2-month and 6-month study periods, a trend towards higher seizure freedom was noted in patients with typical versus non-typical epileptiform discharges at baseline with the valproic acid group showing a superior response.Conclusion: Although no statistically significant difference in response rates was noted, 1) a shift towards higher seizure freedom with valproic acid; 2) improved response after switching to valproic acid at 2 months, if warranted; and 3) superior response rate in patients with typical EEG epileptiform discharges at baseline were observed. A larger study is needed to define the role of early serial EEGs to delineate higher drug failure probability; and to determine the importance of non-typical EEG characteristics at baseline in relation to the choice of AED and long-term outcome Keywords: childhood absence epilepsy, ethosuximide, valproic acid, non-typical epileptiform discharges.","PeriodicalId":7102,"journal":{"name":"Acta neurologica Taiwanica","volume":"29(2) ","pages":"46-53"},"PeriodicalIF":0.0,"publicationDate":"2020-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37959036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Facial Artery Arteriovenous Malformation Endovascular and Surgical Treatment: Liquid Embolic Device an Advantage for Both Technique. 面动脉动静脉畸形血管内及手术治疗:液体栓塞装置是两种技术的优势。

Acta neurologica Taiwanica Pub Date : 2020-06-01

Umberto G Rossi, Giuseppe Verrina, Anna Maria Ieradi, Mariangela Rutigliani, Maurizio Cariati

引用次数: 0