Acta neurologica Taiwanica最新文献

{"title":"Dravet Syndrome: Past, Present, and Future.","authors":"Yi-Hsuan Liu, Cheng-Yen Kuo, I-Ching Chou, Ting-Rong Hsu, Kuang-Lin Lin","doi":"10.4103/ant.ANT-D-25-00005","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-25-00005","url":null,"abstract":"<p><p>Dravet syndrome (DS) is a severe and rare developmental epileptic encephalopathy and genetic epilepsy characterized by the onset of seizures before 1 year of age, typically prolonged generalized tonic-clonic or hemiclonic seizures, specific triggers such as vaccination, high body temperature, or photic stimulation, and normal development before seizure onset. It was proposed in 1989 and confirmed as a genetic epilepsy in 2001 due to SCN1A gene mutations. The seizures are pharmacoresistant, and the prognosis is poor. Comorbidities include movement disorders, intellectual disabilities, behavioral and psychiatric issues, and a high risk of sudden unexpected death in epilepsy (SUDEP). Despite significant advances in understanding the natural course of DS and its pathophysiological mechanisms, management remains difficult. Current treatments, including various antiseizure medications and neuromodulation therapies, rarely achieve complete seizure freedom and fail to prevent cognitive decline. The review aims to summarize the advancements in diagnosis, optimal management, and the prognosis of DS into adulthood while discussing the ongoing challenges, unresolved needs, and potential areas for future research. In addition, it highlights the importance of early intervention, prognostic factors, and the critical need for effective management strategies beyond seizure control, encompassing cognitive preservation, SUDEP prevention, and addressing sleep problems.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"125-132"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187882","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}

{"title":"Harlequin Syndrome in a Post-COVID-19 Infection Elderly: A Case Report.","authors":"Yuda Turana, Robert Shen, Octavianus Darmawan, Jimmy Fransisco Abadinta Barus","doi":"10.4103/ANT.ANT_113_0023","DOIUrl":"10.4103/ANT.ANT_113_0023","url":null,"abstract":"<p><p>Harlequin syndrome (HS) is a rare autonomic nervous system disorder caused by blockage of the unilateral upper-thoracic sympathetic pathway that provides vasomotor and sudomotor supply to the face. Infection and the subsequent immune response cascade are proposed as triggers for arterial occlusion due to microvascular ischemia, underlying the pathophysiology of HS in adult cases. We report this unusual case to inform readers about the possibility of causation and pathophysiological pathway of HS in post-COVID-19 infection. An 82-year-old male patient developing HS post-COVID-19 infection, presented with sudden left-hemifacial redness 4 days before admission, with similar recurrent complaints ten times since his resolved symptomatic COVID-19. Other physical and neurological examinations are regular. Laboratory results showed mild microcytic hypochromic anemia with D-dimer five-folds higher than normal limits. D-dimer fluctuated elevation during HS attacks is a sign of inflammatory process and increased thromboembolic risk in a long-COVID. The patient's history of chronic hypercholesterolemia with old lacunar infarction in the brain's frontal lobes suggested worsening microvascular disturbance. The patient was diagnosed as HS in post-COVID-19. To the author's knowledge, this is the first reported HS case in Indonesia and the second HS case post-COVID-19. Moreover, we also provide a schematic approach to the possible pathological process of HS in post-COVID-19. Post-COVID-19 infection is highly possible causing HS because of its persistent pathological inflammation leading to dysautonomic sympathetic fibers. However, its involvement in a specific pathophysiological pathway that disturbs the cervical sympathetic chain and causes HS, without presenting as systemic dysautonomia, should be further investigated.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":" ","pages":"176-180"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577180","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}

{"title":"Communicating Neurological Bad News: An Essential Skill for Neurologists and a Fundamental Medical Education Core Competency.","authors":"Hussein Algahtani, Bader Shirah, Omar M Alfaifi, Yahia A Alghtanie, Hussam A Jamaluddin","doi":"10.4103/ANT.ANT_113_0052","DOIUrl":"10.4103/ANT.ANT_113_0052","url":null,"abstract":"<p><p>The effective communication of bad news in neurology is a critical and delicate process that profoundly influences patient experiences and outcomes. This comprehensive discussion emphasizes the importance of thorough preparation, appropriate settings, trust-building, clear language, active listening, and provision of support. Recognizing the impact on psychological well-being, the process extends beyond information delivery to demonstrating empathy and commitment to holistic care. The evolution of communication training, now integral to medical education, is highlighted. The call for universal implementation underscores the need for ongoing training and development, emphasizing cultural sensitivity and interdisciplinary collaboration. Integrating bad news delivery programs into educational curricula is proposed, ensuring healthcare professionals are adept at navigating these conversations with empathy. Effective communication of bad news contributes to patient-centered care, fostering a compassionate healthcare environment that prioritizes patient' and families' well-being.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"139-143"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187903","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}

{"title":"Isolated Intracranial Hypertension Following Coronavirus Disease in 2019 AstraZeneca Vaccine: Report of 2 Consecutive Cases.","authors":"Mohammad-Amin Farzi, Mohsen Hadavi Bavil, Mohamad Hussein Assi","doi":"10.4103/ANT.ANT_113_0040","DOIUrl":"10.4103/ANT.ANT_113_0040","url":null,"abstract":"<p><p>Herein reported are two patients that developed symptoms of isolated intracranial hypertension (IIH) following vaccination with AstraZeneca ChAdOx1-S vaccine. Patient identified during daily practice and selected for further evaluation for headache based on clinical findings. Both patients underwent diagnostic lumbar puncture, and after detection of high cerebrospinal fluid (CSF) opening pressure, therapeutic CSF extraction was performed. Although causative relationship couldnt be inferred between AstraZeneca ChAdOx1-S vaccine and IIH, solely based on these two cases, all clinicians should be vigilant about IIH in recipients of this vaccine.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"157-160"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187912","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}

{"title":"Subtle Manifestation of Lower Cavernous Sinus Syndrome as the Initial and Sole Presentation of Pterygopalatine Squamous Cell Carcinoma Invasion from the Skull Base: A Case Report.","authors":"Brian Po-Sheng Wu, Poyin Huang","doi":"10.4103/ANT.ANT_112_0109","DOIUrl":"https://doi.org/10.4103/ANT.ANT_112_0109","url":null,"abstract":"<p><p>We present a case highlighting the critical role of early recognition of partial cavernous sinus syndrome (CSS), as diagnostic delay can lead to irreversible tumor progression. A 50-year-old woman presented with subtle binocular diplopia and facial tingling sensation for 1 month. Neurological examination revealed isolated right abducens nerve (VI) palsy and paresthesia at territories of right ophthalmic (V1) and maxillary (V2) nerves. Partial CSS was suspected. Brain magnetic resonance image (MRI) revealed a contrast-enhancing mass involving the pterygopalatine fossa, sphenoid, and maxillary sinuses extending into the cavernous sinus. Fiberscope biopsy confirmed nonkeratinizing squamous cell carcinoma (SCC). After consultation with otolaryngologist, concurrent chemoradiation as the initial treatment modality was suggested. We describe an uncommon etiology of CSS by pterygopalatine SCC invasion, presenting initially as subtle cranial nerve palsies. Involvement of lower anatomical structures in cavernous sinus and \"mandibular sparing\" can be a hint of invasive lesion from pterygopalatine fossa. This case emphasizes the importance of high clinical suspicion for partial CSS, as early recognition can have great impact on patient's prognosis.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"173-175"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187861","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}

{"title":"Postirradiation Carotid Stenosis: Pathophysiology, Diagnostic Approaches, Endovascular Treatment, and Outcomes.","authors":"Chia-Hung Wu, Chung-Han Yang, Kai-Wei Yu, Wei-An Tai, Fu-Sheng Hsueh, Hsin-Wei Wu, Chao-Bao Luo, Te-Ming Lin, Feng-Chi Chang","doi":"10.4103/ant.ANT-D-24-00047","DOIUrl":"https://doi.org/10.4103/ant.ANT-D-24-00047","url":null,"abstract":"<p><p>Postirradiation carotid stenosis (PIRCS) has emerged as a unique and challenging subtype of carotid stenosis (CS), which threatens patients with significantly worse outcomes even after aggressive intervention. Compared with nonirradiated CS patients, PIRCS patients face a shorter in-stent restenosis-free survival time after percutaneous transluminal angioplasty and stenting. This distinct behavior of PIRCS likely stems from radiation-induced endothelial changes, accelerated atherosclerosis, and potentially increased inflammatory responses. Furthermore, atypical lesions in the carotid arteries, which often occur in tandem and unusual locations, can complicate the diagnosis and treatment planning. Consequently, understanding the unique pathophysiology and clinical presentation of PIRCS is crucial for choosing optimal treatments and improving patient outcomes in this vulnerable population. In this review, we describe the basic features and unique characteristics of PIRCS. Some potentially novel treatment approaches are also discussed.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"133-138"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187880","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}

{"title":"Assessment of Cognitive Functions and Event Related Potentials in Female Patients with Migraine without Aura: A Cross-sectional Study.","authors":"Shereen Fathi, Ahmed Abdelalim, AyatAllah Farouk Hussein, Reham Shamloul, Sara Refaat Shazly, Ghada Hatem","doi":"10.4103/ANT.ANT_112_0098","DOIUrl":"https://doi.org/10.4103/ANT.ANT_112_0098","url":null,"abstract":"<p><strong>Background: </strong>Migraine is associated with cognitive symptoms during its all phases. Both migraine and cognitive impairment are more common in females.</p><p><strong>Objectives: </strong>This study aimed to assess cognitive functions in females with migraine without aura using cognitive tests and event related potential (ERP) (P 300).</p><p><strong>Materials and methods: </strong>Forty-one female patients diagnosed with migraine without aura according to the International Classification of Headache Disorders, 3rd edition, and 40 healthy controls matched for age, sex, and education were included, and all participants performed the Arabic version of the Addenbrooke's Cognitive Examination (ACE-III), the Brief Visuospatial Memory Test-Revised, the Symbol Digit Modalities Test (SDMT), and underwent ERP-P300 using the auditory oddball paradigm.</p><p><strong>Results: </strong>Patients showed better total score of ACE-III and visuospatial score than healthy control group (P = 0.027, P < 0.0001, respectively), no significant difference between both groups regarding attention, memory, language, and processing speed (P > 0.05). A negative correlation between P 300 latency and SDMT and a positive correlation between SDMT, attention and amplitude not reaching statistical significance were found (P > 0.05).</p><p><strong>Conclusions: </strong>Migraine patients showed a better performance in some cognitive functions compared to heathy control. P 300 could be used as a tool for detecting early and subtle cognitive impairment.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"144-149"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187887","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}

{"title":"Clinical Features and Outcome in Myasthenia Gravis Patients with Antimuscle-specific Tyrosine Kinase Antibody: A Retrospective Study.","authors":"Yi-Chun Chung, Jiann-Horng Yeh, Hou-Chang Chiu, Li-Ming Lien, Wen-Hung Chen","doi":"10.4103/ANT.ANT_112_0081","DOIUrl":"https://doi.org/10.4103/ANT.ANT_112_0081","url":null,"abstract":"<p><strong>Background: </strong>Myasthenia gravis (MG) patients with anti-MuSK (muscle-specific tyrosine kinase) antibody (MuSK-MG) are uncommon. Compare with MG patient with anti-ACh (acetylcholine) receptor antibody (AChR-MG), MuSK-MG exhibit unique features.</p><p><strong>Objectives: </strong>The aim of this study is to analyze the difference of the clinical characteristics and electrophysiological features between MuSK-MG and AChR-MG.</p><p><strong>Materials and methods: </strong>We retrospectively review the medical records of generalized MG patients from 2004 to 2019 at Shin Kong Memorial Wu Ho-Su Hospital. We enrolled 47 MuSK-MG patients (33 females and 14 males), and 48 AChR-MG patient (23 females and 25 males).</p><p><strong>Results: </strong>MuSK-MG patients have a female predominance (70.2% vs. 47.9%, P = 0.027) and no significant difference to AChR-MG in onset age (44.02 ± 15.06 vs. 47.52 ± 15.85, P = 0.273). MuSK-MG are more likely to involve facial (76.6% vs. 16.7%, P < 0.001), bulbar (100% vs. 50%, P < 0.001), neck (55.3% vs. 22.9%, P = 0.001), and respiratory muscles (61.7% vs. 10.4%, P < 0.001). MuSK-MG tent to experience more severe symptoms with MGFA Class III or greater (72.3% vs. 50.0%, P = 0.032). The thymic pathology in MuSK-MG is likely to be normal (55.3% vs. 18.8%) or thymic hyperplasia (44.7% vs. 25%). The overall positive rate for repetitive nerve stimulation (RNS) in MuSK-MG patients is less than AChR-MG patients (66.0% vs. 85.4%, P = 0.027). Further analysis shows the difference is mainly by recording from trapezius muscles (51.1% vs. 79.2%, P = 0.004), whereas there is no difference in nasalis muscles (60% vs. 67.6%, P = 0.544) and abductor digiti minimi muscles (13% vs. 18.4%, P = 0.582). The positive rate for single fiber electromyography (SFEMG) recording from orbicularis oculi is very high in both MuSK-MG and AChR-MG (100% vs. 97.2%, P = 0.289). Both MuSK-MG and AChR-MG patients achieved good outcome after proper treatment (68.1% vs. 75%, P = 0.4455).</p><p><strong>Conclusions: </strong>MuSK-MG patients have a female predominance and more facial, bulbar, neck, and respiratory muscles involvements. The electrodiagnostic features of MuSK-MG are lower positive rate of RNS at trapezius and high positive rate of SFEMG at facial muscles. Although MuSK-MG patients are associated with a more progressive course, proper diagnosis and treatment still can lead to a favorable outcome.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"150-156"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187907","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}

{"title":"Anterior Spinal Cord Artery Dissection.","authors":"Chih-Wei Wu, Chao-Liang Chou","doi":"10.4103/ANT.ANT_113_0006","DOIUrl":"https://doi.org/10.4103/ANT.ANT_113_0006","url":null,"abstract":"","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"184-185"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187832","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}

{"title":"Primary Central Nervous System Melanoma Masquerading as Subarachnoid Hemorrhage: A Case Report.","authors":"Yue-Chin Lin, Chao-Liang Chou","doi":"10.4103/ANT.ANT_112_0104","DOIUrl":"https://doi.org/10.4103/ANT.ANT_112_0104","url":null,"abstract":"<p><p>Primary central nervous system (CNS) malignant melanoma is a rare occurrence that can mimic subarachnoid hemorrhage (SAH) on imaging. We present the case of a patient with primary CNS melanoma presenting as an SAH. A 46-year-old man presented with a 2-week history of subacute thunderclap headaches, blurred vision in the left eye, bilateral hearing impairment, and recent weight loss of 10 kg in a year. Noncontrast head computed tomography scan revealed several spotting hyper-attenuations in the brainstem. Magnetic resonance imaging of the head showed hyper-intensity on the T1 sequence with leptomeningeal enhancement at both cerebral convexities, the surface of the brainstem, the superior cerebellum, both facial/vestibulocochlear nerve complexes, and trigeminal nerves. Cytospin smear of cerebrospinal fluid (CSF) found atypical cells with intracytoplasmic melanin pigment. The patient was operated upon for hydrocephalus, and the pathology result of a surgical piece confirmed the diagnosis of melanoma. Melanoma should be considered in patients with suspected SAH on imaging with nonbloody and nonxantochromatic CSF. A careful cytologic examination of CSF is needed.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"181-183"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145187856","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}