Acta neurologica Taiwanica最新文献

{"title":"A Review of Spinocerebellar Ataxias in Taiwan.","authors":"Chia-Ju Lee, Chin-San Liu","doi":"10.4103/ANT.ANT_113_0057","DOIUrl":"https://doi.org/10.4103/ANT.ANT_113_0057","url":null,"abstract":"<p><strong>Abstract: </strong>Spinocerebellar ataxias (SCAs) are a group of neurodegenerative diseases characterized by inherited progressive cerebellar ataxia with or without other associated features. The clinical presentations of these affected SCA patients are very heterogeneous. Genetic testing has been performed in the Taiwanese population to determine the prevalence of SCAs in Taiwan. In general, the diseases can be classified as those caused by tandem repeats and those with conventional mutations. Among the SCAs caused by tandem repeats, polyglutamine SCAs, including SCA1, SCA2, SCA3, SCA6, SCA7, and SCA17, are prevalent in Taiwan and worldwide. Other SCAs caused by non-CAG tandem repeats that have been investigated in Taiwan include SCA8, SCA12, SCA31, and SCA36. However, SCA12 and SCA31 seem either rare or absent in the Taiwanese population. On the other hand, there are SCAs caused by point mutations, duplications, insertions, and deletions. Taiwanese cases with SCAs related to ITPR1 gene mutations, SCA19/22, SCA28, SCA35, SCA47, and SCA48 have been studied and published. To generate a whole picture of SCAs in Taiwan, in this review, we summarized the prevalence of SCAs and described the characteristics of less common types of SCAs in Taiwanese patients.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 2","pages":"55-63"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144236208","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":"Aseptic Meningitis with Cerebellitis Secondary to Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease): A Case Report.","authors":"Tai-Yuan Tseng, Ya-Ju Lin","doi":"10.4103/ANT.ANT_113_0007","DOIUrl":"10.4103/ANT.ANT_113_0007","url":null,"abstract":"<p><strong>Abstract: </strong>Kikuchi-Fujimoto disease (KFD) rarely involves the central or peripheral nervous system, including the cerebellum. Herein, we present a case of KFD, describing its notable clinical findings, diagnostic approach, management, and outcome. A 23-year-old woman received a diagnosis of KFD 5 months before being admitted to our hospital. She presented with fever, headache, and painful cervical lymphadenopathy; low-dose prednisolone (15 mg/day) was administered. She complained of explosive occipital headaches that awakened her in the middle of the night for 2 weeks. Vertigo/dizziness with projectile vomiting was also noted, followed by double vision, unsteady gait, and slower response on the day of admission. Neurological examination revealed drowsy consciousness, bilateral abducens palsy, scanning speech, left limb dysmetria, and truncal ataxia with marked neck stiffness. Brain magnetic resonance imaging (MRI) revealed focal swelling of the left cerebellum and leptomeningeal enhancement. Cerebrospinal fluid measurements revealed extremely high opening pressure (60 cm H 2 O) and lymphocyte-predominant pleocytosis. No other autoimmune or microbiological etiology was identified. We used dexamethasone (20 mg/day) and mannitol for symptomatic treatment, followed by a tapered dose of prednisolone. She recovered rapidly and became nearly symptom-free upon discharge. Follow-up brain MRI at 1.5 and 9 months indicated complete resolution of previous cerebellar swelling and leptomeningitis. The self-limiting nature of the patient's disease course and negative findings for other possible etiologies suggested the diagnosis of aseptic meningitis with cerebellitis secondary to KFD. Other possible diagnoses, including lymphoma, seemed unlikely after serial follow-up imaging.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":" ","pages":"106-108"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144176234","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":"Juvenile Myoclonic Epilepsy Adventure: A Retrospective Study.","authors":"Arzu Eroğlu, Ahmet Sami Güven, Huseyin Caksen","doi":"10.4103/ANT.ANT_113_0026","DOIUrl":"https://doi.org/10.4103/ANT.ANT_113_0026","url":null,"abstract":"<p><strong>Background: </strong>Juvenile Myoclonic Epilepsy (JME) is one of the most common idiopathic generalized epilepsies, typically emerging during adolescence. Despite its characteristic clinical and EEG features, diagnosis may sometimes be delayed.</p><p><strong>Objectives: </strong>The aim of this study is to determine the frequency of juvenile myoclonic epilepsy (JME) among generalized epilepsies in children and the issues that should not be overlooked in its clinical course.</p><p><strong>Materials and methods: </strong>This retrospective cross-sectional study was conducted between 2017 and 2019 at Necmettin Erbakan University Meram Faculty of Medicine, Department of Pediatric Neurology, Electroencephalography (EEG) Unit. A total of 300 patients with generalized epilepsy were studied. A total of 3600 EEG recordings were evaluated in detail by the same pediatric neurologist.</p><p><strong>Results: </strong>JME was detected in 8% of the patients; 80% of the patients were female, 20% were male, median age was 15 years (6-17 years), 44% of the patients had a family history of seizures, and 68% had parental consanguinity. Late diagnosis was observed in 4% of JME patients. The most common myoclonic seizures in both groups consisted of eyelid twitching, continuous hand tremor, and accidental dropping of an object in the hand. The mean time from first seizure to correct diagnosis was 2 years (1-6 years).</p><p><strong>Conclusions: </strong>This study demonstrates that in patients with undiagnosed generalized epilepsy in childhood, seizure characteristics should be assessed through a thorough review of their medical history and careful monitoring during clinical follow-up to accurately determine the true incidence of JME.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 2","pages":"76-80"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144236211","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":"Reply to \"The Diagnosis of Signal Recognition Particle-Associated Immune-Mediated Necrotizing Myopathy Requires the Exclusion of Possible Differential Diagnose\".","authors":"Yueh-Feng Sung","doi":"10.4103/ant.ANT-D-24-00023","DOIUrl":"10.4103/ant.ANT-D-24-00023","url":null,"abstract":"","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 2","pages":"122-123"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144236212","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":"Butane Intoxication: A Case Report with Brain Images Resembling Wernicke Encephalopathy and a Delayed Sequela of Slow Orthostatic Tremor.","authors":"Ting-Yi Tseng, Vai-Hong Fong, Kuei-Hong Kuo, Chien-Tai Hong, Tzu-Hsien Lai","doi":"10.4103/ANT.ANT_113_0027","DOIUrl":"10.4103/ANT.ANT_113_0027","url":null,"abstract":"<p><strong>Abstract: </strong>Butane is a colorless and inflammable gas widely available in daily lives, such as liquefied petroleum gas and fuels for cigarette lighters and camping stoves. It could be misused for recreational purposes, which occasionally leads to mortality and morbidity, including symptoms presumably due to hypoxic encephalopathy. A 34-year-old male with a butane abuse history for 6 years presented with fever, change in consciousness, and seizures. Magnetic resonance imaging of the head showed hyperintensity lesions in the bilateral medial thalamus, hypothalamus, infundibular wall, and periaqueductal gray, typical for Wernicke encephalopathy. After conservative treatment, he was discharged with the sequelae of mental impairment and Parkinsonism. Being abstained from butane for about 1 month, he had a new onset of gait disturbance with slow orthostatic tremor in the lower limbs. Butane encephalopathy may present with brain images typical of Wernicke encephalopathy and a clinical course of delayed deterioration, resembling that of hypoxic encephalopathy.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":" ","pages":"109-112"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144176237","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":"Elevated Homocysteine: A Critical Risk Factor for Stroke Types and Subtypes - A Systematic Review and Meta-Analysis of 96 Studies.","authors":"Manyata Srivastava, Annu Gulia, Pradeep Kumar","doi":"10.4103/ant.ANT-D-24-00004","DOIUrl":"10.4103/ant.ANT-D-24-00004","url":null,"abstract":"<p><strong>Background: </strong>The relationship between homocysteine (Hcy) levels and the risk of stroke has been extensively investigated, yet conclusive evidence remains elusive.</p><p><strong>Objectives: </strong>This study aims to conduct a systematic review and meta-analysis to determine the association between higher Hcy levels and the risk of stroke, including its types and subtypes.</p><p><strong>Materials and methods: </strong>A comprehensive literature search was conducted in PubMed, MEDLINE, Google Scholar, EMBASE, and the Cochrane Library databases to identify relevant studies published up to April 30, 2024. We included case-control studies that examined the association of Hcy levels with risk of stroke and its types. Pooled standardized mean difference (SMD) with 95% confidence intervals (CIs), publication bias, trial sequential analysis (TSA), and quality assessment was conducted.</p><p><strong>Results: </strong>A total of 96 studies were included in the analysis: 83 studies on ischemic stroke (IS), 17 studies on Trial of ORG 10172 in Acute Stroke Treatment (TOAST) subtypes of IS, and 12 studies on hemorrhagic stroke (HS). The meta-analysis revealed a significant association between Hcy levels and risk of IS (SMD = 1.35, 95% CI = 1.14-1.55), TOAST subtypes of IS (SMD = 1.48, 95% CI = 1.03-1.92), and HS (SMD = 1.14, 95% CI = 0.68-1.59). TSA indicated that total sample sizes provided sufficient power (80%) to support findings: IS (n = 33,715), TOAST subtypes of IS (n = 5,655), and HS (n = 4,287).</p><p><strong>Conclusions: </strong>High Hcy levels are significantly associated with an increased risk of stroke, including its types and subtypes. These findings underscore the potential of Hcy as predictive marker for stroke risk. Future research should focus on strategies for detecting hyperhomocysteinemia and lowering elevated Hcy levels in high-risk individuals, which may contribute to stroke prevention.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 2","pages":"81-92"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144236210","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":"Bilateral Anterior Cerebral Artery Stroke.","authors":"Naomi Nazareth Becerra-Aguiar, Amado Jiménez-Ruiz, Daniel Mateo Gutierrez-Baeza, Victor Aguilar-Fuentes, José Luis Ruiz-Sandoval","doi":"10.4103/ANT.ANT_112_0066","DOIUrl":"https://doi.org/10.4103/ANT.ANT_112_0066","url":null,"abstract":"","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 1","pages":"50-51"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144112372","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":"Gut Microbiota and Alzheimer Disease.","authors":"Lester Raymundo Dominguez Huarcaya","doi":"10.4103/ANT.ANT_113_0046","DOIUrl":"10.4103/ANT.ANT_113_0046","url":null,"abstract":"<p><strong>Abstract: </strong>The hallmarks of Alzheimer's disease (AD) include brain dysfunction and the buildup of amyloid and tau proteins. The onset of dementia is one of the latter symptoms. Imaging diagnostics allowed for the detection of amyloid buildup in the brain 10-20 years before the emergence of overt signs of the disease. The application of imaging diagnostic techniques allowed for this identification. Within the next few decades, the incidence and frequency of this disease are expected to reach epidemic proportions unless measures are done to stop or slow its growth. However, unless action is taken to slow or stop the disease's progression, it will continue to threaten the health of the general public. Recently, there has been some speculation that the gut flora might contribute to the development of AD. Not only that, but the rapidly expanding ischemia etiology is another possible contributor to the issue. Rumor has it that there's a network connecting the brain and the stomach called the \"gut-brain-microbiota axis.\" The hypothesis is based on this network. Furthermore, a large amount of evidence implies that the gut microbiota (GMB) could potentially contribute to the onset of AD. It has been suggested that the GMB could play a role in the onset of AD. This notion has been bolstered by new studies. It is quite probable that this review will address the prospect of a link between the microbiome and AD. This concept could be explored as a potential therapy or preventative measure. Some techniques that show promise as new treatments for AD include changes to the GMB, which can be achieved through dietary changes or positive microflora interventions, and changes to microbiological partners and their products, like amyloid protein.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 1","pages":"1-12"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144112704","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":"The Predictive Role of Neutrophil-to-LymPhocyte Ratio in Seizure Recurrence in Patients with Epilepsy.","authors":"Elif Simin Issı, Demet İlhan Algın, Oğuz Osman Erdinç","doi":"10.4103/ANT.ANT_112_0107","DOIUrl":"10.4103/ANT.ANT_112_0107","url":null,"abstract":"<p><strong>Background: </strong>The aim of this retrospective study was to investigate the importance of leukocyte, neutrophil, lymphocyte, and neutrophil/lymphocyte (NLR) ratios in epileptic seizure recurrence and whether there is a relationship between them.</p><p><strong>Objectives: </strong>To evaluate the predictive value of leukocyte, neutrophil, lymphocyte, and monocyte parameters in assessing the likelihood of seizure recurrence in epilepsy patients.</p><p><strong>Materials and methods: </strong>Sixty-five epilepsy patients who were on follow-up for epilepsy were included in the study. The study group consisted of epilepsy patients who had routine complete blood counts taken within the first 24 hours of emergency room visits due to seizures and for control purposes at outpatient clinic visits. The parameters examined were leukocyte, neutrophil, lymphocyte, platelet counts (PLT) (×10³ mm³), mean platelet volume (MPV), and NLR. NLR was calculated by dividing the absolute number of neutrophils by the absolute number of lymphocytes.</p><p><strong>Results: </strong>Leukocyte, neutrophil, NLR, and monocyte values (P < 0.001) were significantly higher in patients with seizure recurrence in the first 30 days compared to those without recurrence. The predictive value of leukocyte, neutrophil, NLR, and monocyte parameters for seizure recurrence was assessed using receiver operating characteristic (ROC) analysis. Since the area under the curve (AUC) value for NLR was larger than that of neutrophil and monocyte values, the diagnostic performance of NLR was found to be superior. For NLR, the AUC value was 0.73 (95% confidence interval: 0.639-0.807, P = 0.0001), with sensitivity and specificity of 52.31% and 94.23%, respectively. NLR had the highest predictive value for seizure recurrence.</p><p><strong>Conclusions: </strong>Our study suggests that neutrophil-mediated inflammation plays a role in epileptic seizures and that monocyte values are also important. NLR and monocyte values may serve as predictive biomarkers for seizure recurrence.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 1","pages":"20-23"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144112955","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":"Focal Chronic Inflammatory Demyelinating Polyneuropathy - A Case Report with Clinical Application and Validation of 2021 European Academy of Neurology/Peripheral Nerve Society Criteria.","authors":"Fu Liong Hiew, Winnie Lee Mei Paan","doi":"10.4103/ANT.ANT_112_0101","DOIUrl":"10.4103/ANT.ANT_112_0101","url":null,"abstract":"<p><strong>Abstract: </strong>The updated 2021 criteria of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) for chronic inflammatory demyelinating polyneuropathy (CIDP) has excellent diagnostic sensitivity and specificity for typical and variants of CIDP. For focal CIDP, applications of these criteria may be different due to limited peripheral nerve involvement. We illustrate using a rare case of focal CIDP on the applicability of 2021 EAN/PNS criteria and its practical limitations. A young woman presented with progressive right foot drop and numbness over 5 years. Clinically, she had areflexic right lower limb monoparesis with a sensory deficit over the dorsum of the right foot. A nerve conduction study showed normal right peroneal distal compound motor action potential but marked temporal dispersion with conduction block and significantly prolonged F-waves latency suggestive of demyelination. Right sural sensory nerve action potentials (SNAPs) were small in amplitude with the absence of right superficial peroneal SNAP. These findings suggested a focal sensorimotor acquired demyelinating neuropathy involving right lower limb nerves and fulfilled possible focal CIDP as per the 2021 EAN/PNS criteria. Cerebrospinal fluid (CSF) analysis revealed albuminocytological dissociation. Magnetic resonance imaging of the lumbosacral plexus was unremarkable. She received intravenous methylprednisolone, followed by oral prednisolone and mycophenolate mofetil. Clinically, right lower limb power is in parallel with functional scores. With an objective positive clinical response to treatment in addition to elevated CSF protein as supportive criteria, the diagnosis of possible focal CIDP was upgraded to focal CIDP. The inclusion of supportive criteria in the updated EAN/PNS diagnostic criteria improves diagnostic certainty in focal CIDP.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 1","pages":"24-27"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144112698","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}