Acta Neurologica Taiwanica

Background Rituximab has been increasingly prescribed in the treatment of multiple sclerosis (MS) over recent years. Tumefactive demyelinating lesions can occur at the onset or over the course of MS. Another major cause of these lesions is the side effects of drugs such as natalizumab or fingolimod. This study is a case report of a young MS patient who suffered from tumefactive lesions following the injection of rituximab. Case presentation The patient was an 18-year-old man with MS who developed double vision, imbalance, and quadriparesis symptoms followed by a decrease in his consciousness two days after administration of rituximab. Tumefactive lesions were observed in the patient's brain magnetic resonance imaging (MRI). Conclusion Rituximab should be considered as a potential cause of tumefactive demyelinating lesions in patients with MS.

Abdorreza Naser Moghadasi

Tumefactive Demyelinating Lesions in a Patient with Multiple Sclerosis Developed two Days after the Injection of Rituximab.

多发性硬化患者在注射利妥昔单抗两天后出现肿块性脱髓鞘病变。

Objective Chronic inflammatory processes involving the vascular wall may induce atherosclerosis. Immune-inflammatory processes proceed throughout all stages of acute stroke. We investigated the association of three immune-inflammatory markers, namely systemic immune-inflammation index (SII), neutrophil-to-lymphocyte ratio (NLR), and neutrophil count (NC), with prehospital delay and clinical features in patients with acute ischemic stroke. Methods We retrospectively enrolled 2543 inpatients admitted within 4 days of symptom onset from May 2010 to February 2020. Patients were stratified into three groups: Group A, comprising 161 patients with tissue plasminogen activator (tPA) treatment; Group B, comprising 415 patients who were eligible for tPA treatment; and Group C, comprising all 2543 patients. Results The levels of all three immune-inflammatory markers had positive linear correlations with onsetto- emergency room time, initial National Institutes of Health Stroke Scale (NIHSS) scores, and discharge modified Rankin Scale scores. In Group B, levels of follow-up, but not initial, immuneinflammatory markers were higher in patients with unfavorable outcomes. Common significant predictors of in-hospital complications and unfavorable outcomes were age > 72 years, female sex, NIHSS > 4, diabetes mellitus, and all three immune-inflammatory markers. When combined with other predictors, NC > 7.2 × 103/mL achieved optimal predictive performance (0.794) for in-hospital complications, and SII > 651, NLR > 2.9, and NC > 7.2 × 103/mL had equal predictive performance up to 0.859 for unfavorable outcomes. Conclusions Immune-inflammatory markers dynamically increased from symptom onset of acute ischemic stroke in patients eligible for thrombolytic therapy. Higher levels of immune-inflammatory markers suggest more in-hospital complications and unfavorable short-term outcomes.

Yu-Wei Chu

Pei-Ya Chen

Shinn-Kuang Lin

Correlation between Immune-Inflammatory Markers and Clinical Features in Patients with Acute Ischemic Stroke

急性缺血性卒中患者免疫炎症标志物与临床特征的相关性

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.

Nai Hsin Huang

Li Ming Lien

Wen Hung Chen

Macrophage Activation Syndrome in a Case of Myasthenia Gravis with Concurrent Cytomegalovirus Infection.

重症肌无力并发巨细胞病毒感染病例中的巨噬细胞活化综合征。

OBJECTIVE Health-related quality of life (HRQoL) has become an important outcome for neurosurgical patients underwent surgery. The authors utilized the European Organization for Research and Treatment of Cancer Quality of Life Core Questionnaire (EORTC QLQ C-30) to assess the quality of life of spheno-orbital meningiomas patients after surgery in one national central referral hospital in Indonesia. METHODS In this cross-sectional study, medical record-based data of 40 spheno-orbital meningioma patients who underwent surgery at Dr. Cipto Mangunkusumo General Hospital, Jakarta, Indonesia from October-December 2016 were analyzed. Differences between pre-and postsurgical EORTC scores was examined using the Student's t test. RESULTS There was a statistically significant increase of EORTC QLQ-C30 scores in global health status, role functioning, emotional functioning, cognitive functioning, and social functioning variables, and statistically significant decrease for clinical symptoms variables. CONCLUSION Evidently, there was improvement in HRQoL in spheno-orbital meningiomas patients after surgery, in whom EORTC QLQ-C30 can be used to assess the HRQoL prior to and after surgical procedures.

Renindra Ananda Aman

Kumara Wisyesa

Aryandhito Widhi Nugroho

Syaiful Ichwan

David Tandian

Samsul Ashari

Setyo Widi Nugroho

Pre-and Post-Surgical Health-Related Quality of Life Evaluation of Spheno-orbital Meningioma Patients Based on EORTC QLQ-C30 Questionnaire at Dr. Cipto Mangunkusumo General Hospital.

基于EORTC QLQ-C30问卷的Cipto Mangunkusumo总医院蝶骨眶脑膜瘤患者术前术后健康相关生活质量评估。

PURPOSE Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism of the pathogenesis has not yet been determined, a standardized therapeutic strategy for SUNCT is unavailable. We present a case of SUNCT syndrome with successful pain relief by intravenous administration of ketamine, an N-methyl-D-aspartate receptor (NMDAR) antagonist. CASE REPORT A 56-year-old male patient reported severe throbbing and shooting pain in forehead, temporal and periorbital region. We confirmed conjunctival injection, lacrimation, blepharoptosis, and miosis as symptoms related to autonomic activity, and made a diagnosis of SUNCT based on ICHD-3 beta. Numerous treatments were attempted, including pregabalin, gabapentine, nonsteroidal antiinflammatory drugs, acetaminophen, steroids, antidepressants, triptans, nerve blocks, and intravenous lidocaine with unsatisfactory results. Intravenous administration of ketamine (0.4 mg/kg) for one hour, was found to relieve the severe pain. CONCLUSION Intravenous ketamine can effectively treat SUNCT syndrome. This case demonstrated that involvement of NMDAR could be one of the mechanisms of SUNCT syndrome pathogenesis and establish a therapeutic strategy for this pain syndrome.

Shunji Shiiba

Teppei Sago

Kazune Kawabata

Pain Relief in Short-Lasting Unilateral Neuralgiform Headache with Conjunctival inJection and Tearing Syndrome with Intravenous Ketamine: A Case Report.

静脉注射氯胺酮治疗短时单侧神经痛样头痛伴结膜充血和流泪综合征的疼痛缓解：一例报告。

Spinal muscular atrophy (SMA) is an autosomal recessive motor neuron disease characterized by progressive weakness and atrophy of skeletal muscles. With homozygous survival motor neuron 1 (SMN1) gene mutation, all SMA patients have at least one copy of the SMN2 gene, which provides an opportunity for drug targeting to enhance SMN expression. Current three disease modifying drugs, including nusinersen, onasemnogene abeparvovec, and risdiplam, have demonstrated impressive effectiveness in SMA treatment. Nusinersen is an antisense oligonucleotide targeting SMN2 pre-messenger RNA (mRNA) to modify alternative splicing and is effective in SMA children and adults, administrating via intermittent intrathecal injection. Onasemnogene abeparvovec is an adeno-associated viral vector carrying human SMN1 gene, featuring intravenous injection once in a lifetime for SMA patients less than 2 years of the age. Risdiplam is a small molecule also targeting SMN2 pre-mRNA and is effective in SMA children and adults with administration via oral intake once per day. Patients with SMA should receive these disease modifying therapies as soon as possible to not only stabilize disease progression, but potentially obtain neurological improvement. The development in these therapies has benefited patients with SMA and will potentially provide insight in future drug discovery for other neurodegenerative diseases. Keywords: Adeno-associated viral vector, antisense oligonucleotide, disease modifying therapy, gene therapy, motor neuron disease, spinal muscular atrophy.

Li-Kai Tsai

Chen-Hung Ting

Yo-Tsen Liu

Cheng-Tsung Hsiao

Wen-Chin Weng

Recent Advance in Disease Modifying Therapies for Spinal Muscular Atrophy

脊髓性肌萎缩症疾病修饰疗法的最新进展

This case report aims to describe a rare manifestation of isolated hypoglossal nerve palsy (HNP) resulting from organophosphate poisoning (OP), specifically organophosphate- induced delayed neuropathy (OPIDN). The primary objective of this case report is to highlight this unusual manifestation and discuss its potential underlying mechanisms, emphasizing the importance of timely diagnosis and appropriate management.A 31-year-old male with a history of consuming a commercial chlorpyrifos formulation presented with acute organophosphate poisoning symptoms that improved with appropriate treatment. However, two weeks later, the patient developed neurological deficits (numbness and weakness spreading from the plantar region to the upper limbs) involving the hypoglossal nerve, manifesting as left-sided tongue deviation, fasciculation, and atrophy, resulting in speech and swallowing difficulties. A comprehensive workup ruled out other potential causes of HNP, supporting the link between OPIDN and isolated HNP. Despite regaining limb strength, the patient's speech and swallowing issues persisted, prompting a follow-up visit to our hospital for further management and rehabilitation.This case report highlights a unique manifestation of OPIDN, resulting in isolated HNP, a rare phenomenon. The correlation between exposure to chlorpyrifos, acute organophosphate poisoning, and delayed onset of HNP suggests a causative relationship. Prompt diagnosis, appropriate treatment, and timely speech and swallowing rehabilitation are vital for optimizing outcomes in such cases. Further research is needed to understand the mechanisms underlying this selective vulnerability of OPIDN and to develop targeted interventions.

Monalisa Jati

Ashmeet Kaur

Isolated Hypoglossal Nerve Palsy: A Rare Complication of Organophosphate Poisoning

孤立性舌下神经麻痹：有机磷中毒的罕见并发症

Self-efficacy is one of the factors affecting on the quality of life and adjustment to the chronic diseases such as multiple sclerosis. Self-efficacy is the individual's belief that they have the ability to overcome challenges presented to them. The current study aimed to develop and validate of selfefficacy questionnaire in Iranian MS patients.This is a methodological study that was performed in three phases. In the first phase, the concept of self-efficacy was defined through qualitative content analysis approach. In the second phase, early items of questionnaire was generated according to findings of the first phase. In the final phase, validation of the developed questionnaire were evaluated using face, content and construct validity as well as reliability.In exploratory factor analysis, four subscales including: adherence to medication regimens (5 items), social performance control (4 items), symptom management (4 items) and maintain independence and activity (5 items) were extracted by Eigen value above one and factor load above 0.3. Internal consistency and stability of the developed questionnaire confirmed with 0.91 and 0.88 respectively that indicated acceptable reliability.The 18-item developed questionnaire is valid and reliable for measurement of self-efficacy of Iranian MS patients.

Ali Dehghani

Development and Validation of the Self-efficacy Questionnaire in multiple sclerosis patients

多发性硬化症患者自我效能量表的编制与验证

Abstract Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a non- atheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months. While most RCVS cases have identifiable triggers, a significant portion occurs without known causes. Recent studies have reported a high prevalence of neurovascular abnormalities in RCVS patients. We present a case of a thirty-year-old woman with a sudden-onset severe headache, diagnosed with RCVS associated with carotid web. The patient had no ischemic involvement and responded well to flunarizine treatment. Follow-up imaging showed no stenosis. This case highlights a potential association between carotid web and RCVS, suggesting that FMD may contribute to vascular hyperreactivity and presents as a risk factor for RCVS. Further investigations are needed to understand the underlying mechanisms connecting these two vascular disorders.

Elizeu Pereira dos Santos Neto

Ícaro Araújo de Sousa

Irapuá Ferreira Ricarte

Octávio Marques Pontes-Neto

Reversible Cerebral Vasoconstriction Syndrome And Fibromuscular Dysplasia: An Epiphenomenon Or A Causal Relationship?

可逆性脑血管收缩综合征与纤维肌发育不良：是偶发现象还是因果关系？

Paraquat is known to cause damage to various organs, including the brain. Although curcumin have anti-inflammatory and anti-oxidant properties, it is not yet clear how they relate to PQ-induced neurotoxicity. This study's objective was to compare the effects of curcumin and nanocurcumin on PQ-induced neurotoxicity in male rats.Treatment with curcumin and nano-curcumin improves brain function in PQ toxicity and nanocurcumin was more advantageous than ordinary curcumin.

Mojdeh Mohammadi

Seyed Sina Tajedini

Zohre Sadeghian

Sara Soleimani Asl

Nejat Kheiripour

Mohammadmahdi Sabahi

Akram Ranjbar

Molecular, Biochemical and Histological Assessment in Paraquat Induced Neurotoxicity: Restoring Role of Nano-curcumin in an Experimental Study

百草枯诱导神经毒性的分子、生化和组织学评估：纳米姜黄素在实验研究中的修复作用

ISSN	1028-768X
h-index	28

Publication Information	Publisher: Neurological Society R.O.C (Taiwan)，Publishing cycle: ，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 1997，Proportion of original research papers: ，Self Citation Rate:， Gold OA Rate:

Acta Neurologica Taiwanica

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