Acta neurologica Taiwanica最新文献

Unilateral Cortical Ribboning and Corticomedullary Lesions in a Rare Case of Coexisting Anti-N-methyl-D-aspartate Receptor Encephalitis and Neuronal Intranuclear Inclusion Disease. 罕见的抗n -甲基- d -天冬氨酸受体脑炎和神经元核内包涵病并发的单侧皮质带状和皮质髓质病变

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-25-00037

Chen-Chang Shih, Kuo-Hsuan Chang

{"title":"Unilateral Cortical Ribboning and Corticomedullary Lesions in a Rare Case of Coexisting Anti-N-methyl-D-aspartate Receptor Encephalitis and Neuronal Intranuclear Inclusion Disease.","authors":"Chen-Chang Shih, Kuo-Hsuan Chang","doi":"10.4103/ant.ANT-D-25-00037","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-25-00037","url":null,"abstract":"Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder characterized by psychiatric manifestations, seizures, and dyskinesias. Neuronal intranuclear inclusion disease (NIID) presents heterogeneously with dementia, neuropathy, and episodic encephalitis-like events, typically showing bilateral corticomedullary hyperintensities on diffusion-weighted imaging (DWI). The simultaneous occurrence of these conditions is extremely uncommon, complicating diagnosis due to overlapping clinical and radiological features. We report the case of a 68-year-old female with a history of atrial fibrillation and hyperthyroidism who presented with urinary tract infection, followed by acute consciousness disturbance, left arm paresis, and pyrexia. Initial antibiotic therapy resolved symptoms, but recurrent neurological deficits ensued. Diagnostic evaluation confirmed the presence of both anti-NMDAR encephalitis, evidenced by positive serum antibodies, and NIID, supported by abnormal GGC repeat expansions in the NOTCH2NLC gene and intranuclear inclusions on skin biopsy. Brain magnetic resonance imaging demonstrated unusual unilateral cortical ribboning and corticomedullary lesions on DWI. Therapy with corticosteroids and intravenous immunoglobulin resulted in significant improvement; however, subsequent workup revealed colorectal adenocarcinoma. This case highlights the diagnostic complexity and management challenges by the rare concurrence of anti-NMDAR encephalitis and NIID.","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"35 2","pages":"108-111"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791909","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

Taiwan Clinical Practice Guidelines for Myasthenia Gravis. 台湾重症肌无力临床实践指南。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-25-00063

Hsu-Ling Yeh, Che-Cheng Chang, An-Bang Liu, Meng-Han Tsai, Long-Sun Ro, Yi-Chen Chang, Tung-Yu Tiong, Jen-Jen Su, Yi-Chung Lee, Yu-Ning Huang, Shu-Lang Liao, Thy-Sheng Lin, Yi-Chu Liao, Chen-Chih Chung, Kuo-Hsuan Chang, Shu-Ping Chao, Yuan-Ting Sun, Yuh-Cherng Guo, Chin-Chang Huang, Wei-Ting Chiu, Chiung-Mei Chen, Ming-Hsing Chang, Chi-Chao Chao, Cheng-Che Chou, I-Jun Chou, Yung-Ting Kuo, Anne Chang, Chen-Shu Chang, Mei-Yun Cheng, Kuan-Yu Lin, Kon-Ping Lin, Wei-Hung Chen, Jung-Lung Hsu, Chih-Chao Yang, Nai-Wen Tsai, Jiann-Horng Yeh, Hou-Chang Chiu

{"title":"Taiwan Clinical Practice Guidelines for Myasthenia Gravis.","authors":"Hsu-Ling Yeh, Che-Cheng Chang, An-Bang Liu, Meng-Han Tsai, Long-Sun Ro, Yi-Chen Chang, Tung-Yu Tiong, Jen-Jen Su, Yi-Chung Lee, Yu-Ning Huang, Shu-Lang Liao, Thy-Sheng Lin, Yi-Chu Liao, Chen-Chih Chung, Kuo-Hsuan Chang, Shu-Ping Chao, Yuan-Ting Sun, Yuh-Cherng Guo, Chin-Chang Huang, Wei-Ting Chiu, Chiung-Mei Chen, Ming-Hsing Chang, Chi-Chao Chao, Cheng-Che Chou, I-Jun Chou, Yung-Ting Kuo, Anne Chang, Chen-Shu Chang, Mei-Yun Cheng, Kuan-Yu Lin, Kon-Ping Lin, Wei-Hung Chen, Jung-Lung Hsu, Chih-Chao Yang, Nai-Wen Tsai, Jiann-Horng Yeh, Hou-Chang Chiu","doi":"10.4103/ant.ANT-D-25-00063","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-25-00063","url":null,"abstract":"Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by muscle weakness and fatigue due to autoantibodies impairing neuromuscular transmission. With a global prevalence of 40-180 per million, Taiwan has seen a rise in MG cases from 4476 in 2013 to 5752 in 2019, with generalized MG (GMG) prevalence increasing significantly, whereas GMG patients incur substantially higher medical costs and resource utilization compared to non-MG patients. To standardize MG management, the Society formed an expert committee of 37 MG specialists and a patient representative, tasked with developing evidence-based guidelines. The committee, organized into 11 thematic subgroups, conducted a systematic review of literature from 2015 to 2024 and employed a Modified Delphi method to achieve consensus on clinical guidance statements. Recommendations cover diagnosis (e.g., antibody testing and electrophysiological tests), treatment (e.g. thymectomy, steroids, and novel biologics), and management of specific MG subtypes, including ocular, early- and late-onset, muscle-specific tyrosine kinase-related, and refractory MG, as well as myasthenic crisis and neonatal MG. The guidelines emphasize early diagnosis, integrated management of comorbidities, and long-term care strategies, including patient support and tailored exercise programs. These consensus statements aim to enhance care quality, improve patient outcomes, and guide future research and pharmaceutical development in Taiwan's medical community.","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"35 2","pages":"59-69"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791823","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

Prolonged Conscious Disturbance in a Patient with Neuronal Intranuclear Inclusion Disease Masquerading as Hashimoto Encephalopathy: A Case Report. 伪装为桥本脑病的神经元核内包涵病患者的长时间意识障碍1例报告。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-24-00034

Chien-Ya Hsu, Chun-Wei Chang

引用次数: 0

Taiwan Poststroke Disability Study: A Study Protocol. 台湾脑卒中后残疾研究：研究方案。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-25-00022

Chih-Hao Chen, Li-Kai Tsai, Ting-Yu Chang, Hui-Chen Su, Pi-Shan Sung, Chung-Hsing Chou, Hsin Tung, Yi-Chen Hsieh, Chih-Ping Chung, Lung Chan, Hung-Yi Chiou, Li-Ming Lien, Jiann-Shing Jeng

{"title":"Taiwan Poststroke Disability Study: A Study Protocol.","authors":"Chih-Hao Chen, Li-Kai Tsai, Ting-Yu Chang, Hui-Chen Su, Pi-Shan Sung, Chung-Hsing Chou, Hsin Tung, Yi-Chen Hsieh, Chih-Ping Chung, Lung Chan, Hung-Yi Chiou, Li-Ming Lien, Jiann-Shing Jeng","doi":"10.4103/ant.ANT-D-25-00022","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-25-00022","url":null,"abstract":"Background: Stroke is a leading cause of mortality and disability in Taiwan, necessitating a comprehensive understanding of its long-term impacts.Objectives: This study aims to investigate the prevalence, risk factors, and progression of poststroke disabilities across motor, swallowing, cognitive, emotional, epilepsy, and quality-of-life domains.Materials and methods: This multicenter, prospective cohort study will recruit 2000 acute ischemic and hemorrhagic stroke patients from 14 major hospitals in Taiwan. Assessments will be conducted at baseline, 3 months, and 12 months poststroke using validated tools, questionnaires, imaging, and blood biomarkers (ClinicalTrials registered no: NCT06772194).Results: Key outcomes include motor function recovery, prevalence of dysphagia, cognitive impairment, poststroke depression, and epilepsy, as well as quality-of-life measures. Advanced imaging techniques and biomarker analyses, including genome-wide association studies, will explore predictors of disability and recovery.Conclusions: This study seeks to address gaps in stroke recovery research by integrating clinical and biological data to enhance personalized stroke management. T-PODS is expected to inform strategies for reducing disability and improving long-term outcomes for stroke survivors in Taiwan, contributing to the global effort to optimize stroke care and rehabilitation.","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"35 2","pages":"79-87"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791919","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

Landau-Kleffner Syndrome with Adult-onset Epilepsy: A Case Report. Landau-Kleffner综合征伴成人癫痫1例报告。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-24-00039

Chien-Ya Hsu, Mei-Yun Cheng

引用次数: 0

Pituitary Apoplexy with the Initial Presentations Similar to Trigeminal Autonomic Cephalalgia: A Case Report. 以三叉神经自主神经性头痛为首发表现的垂体性中风1例报告。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-24-00033

Chien-Ya Hsu, Chih-Hong Lee

引用次数: 0

Uncommon Stroke Mimics: Racemose Neurocysticercosis. 罕见的中风模拟：总状体神经囊虫病。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-24-00038

Ilse Murrieta-Hernandez, Juan-Carlos Lopez-Hernandez, Enrique Piña-Rosales, Brenda Dzul-Garcia, Raul Medina-Rioja

引用次数: 0

Cold Climate and Alzheimer's Disease: A Narrative Review of Biomedical and Environmental Mechanisms. 寒冷气候与阿尔茨海默病：生物医学和环境机制的叙述性回顾。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-25-00060

Khaled A Abdel-Sater

{"title":"Cold Climate and Alzheimer's Disease: A Narrative Review of Biomedical and Environmental Mechanisms.","authors":"Khaled A Abdel-Sater","doi":"10.4103/ant.ANT-D-25-00060","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-25-00060","url":null,"abstract":"Emerging evidence suggests that exposure to cold environments may influence Alzheimer's disease (AD) pathogenesis through interconnected vascular, inflammatory, metabolic, and thermoregulatory mechanisms. Aging impairs physiological resilience to cold stress, which may exacerbate neurodegenerative processes in vulnerable individuals. This narrative review summarizes findings from epidemiological, mechanistic, and interventional studies on the relationship between cold exposure and AD. This article is a narrative review informed by a structured literature search conducted in PubMed and Scopus databases up to July 2025. Boolean operators such as (\"cold exposure\" OR \"cold climate\") AND (\"Alzheimer's disease\" OR \"neurodegeneration\") were applied. While not a systematic review, a PRISMA-style flowchart was solely for transparency, illustrating how the literature was screened. No meta-analytic methods or formal bias assessment tools were applied. Cold exposure is associated with cerebral vasoconstriction, glucose hypometabolism, sleep disruption, mitochondrial stress, and increased permeability of the blood-brain barrier. These changes promote neuroinflammation and oxidative stress, which are key contributors to the pathology of AD. Reduced brown adipose tissue with aging makes people more sensitive to cold. Apolipoprotein E (APOE) ε4 allele carriers seem to be more susceptible to these stresses. Epidemiological data support a higher dementia risk in colder climates, with seasonal and regional variations persisting after adjustment for confounders. Exposure to cold environments may act as a modifiable risk factor for AD, particularly in aging populations with impaired thermoregulation. While evidence points to plausible mechanistic links, further longitudinal and experimental studies are required. Integrating environmental variables into neurodegenerative risk models may enhance prevention strategies and support personalized interventions.","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"35 2","pages":"70-78"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791765","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

Deficiency of Adenosine Deaminase 2: A Rare Cause of Stroke-related Dystonia in Young Adults - A Case Report. 腺苷脱氨酶2缺乏：年轻人中风相关肌张力障碍的一个罕见原因-一个病例报告。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ant.ANT-D-25-00039

Thien Thien Lim, Chia Yin Por, Chee Ming Teh, Beng Siang Koay

引用次数: 0

Abnormal Movement-related Cortical Potentials in Patients with Holmes Tremor and Cerebellar Degeneration. 霍尔姆斯震颤和小脑变性患者运动相关皮层电位异常。

Acta neurologica Taiwanica Pub Date : 2026-04-01 Epub Date: 2026-04-24 DOI: 10.4103/ANT.ANT_113_0056

Jui-Te Fu, Chon-Haw Tsai, Ming-Kuei Lu

{"title":"Abnormal Movement-related Cortical Potentials in Patients with Holmes Tremor and Cerebellar Degeneration.","authors":"Jui-Te Fu, Chon-Haw Tsai, Ming-Kuei Lu","doi":"10.4103/ANT.ANT_113_0056","DOIUrl":"https://doi.org/10.4103/ANT.ANT_113_0056","url":null,"abstract":"Background: Movement-related cortical potential (MRCP) represents function of the cerebello-thalamo-cortical circuit.Objectives: To elucidate the functional role of the olivodentatorubral pathway and the cerebellum on the motor cortex (M1), we analyzed MRCP in patients with Holmes' tremor, cerebellar degeneration, and healthy controls.Materials and methods: Three patients with Holmes' tremor in their right arms, four patients with cerebellar degeneration, and eight healthy subjects were studied. MRCP was recorded from 26 scalp Ag/AgCl cup electrodes when a self-paced, brisk voluntary extension of the wrist with a rate of every 7 s was performed. The MRCP components, including negative slope (NS') and frontal peak of the motor potential (fpMP), were analyzed. Nonparametric statistics (Mann-Whitney) were used to calculate the amplitude difference between the groups.Results: Compared with the healthy control group, the Holmes' tremor group revealed a significant reduction of the fpMP amplitude in the frontal midline electrode FCZ. The reduction of the mean amplitude of NS' was found in the posterior-scalp region for right-hand movement. The cerebellum degeneration group showed a significant prolongation of the fpMP latency and a significant decrease of the fpMP amplitude. In addition, a reduced mean amplitude of the NS' on the midline and the central region contralateral to the hand movement was noted.Conclusions: Findings suggest that the cerebellum plays a pivotal role in compositing both the negative shifting before the movement onset and the motor potential (MP) after the movement onset. Its associated circuits mediating Holmes' tremor might influence only the amplitude of the MP after the movement onset.","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"35 2","pages":"88-95"},"PeriodicalIF":0.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147791825","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