Pub Date : 2025-01-01DOI: 10.1016/j.nerep.2025.100256
Ana Rita Castro , Daniela Oliveira , Bárbara Teixeira , João Macedo Cunha , Ivânia Alves , Luis Ruano
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterized by IgG autoantibodies targeting the GluN1 subunit of the NMDAR. We present a case of a 20-year-old male diagnosed with NMDAR encephalitis, who two years later developed symptoms and MRI lesions suggestive of multiple sclerosis (MS).
The patient initially exhibited depressive symptoms progressing to severe neuropsychiatric manifestations including catatonia, hallucinations, and dysphagia. Positive anti-NMDAR antibodies in the CSF and serum were found, and extensive investigations, including imaging and CSF analysis, ruled out alternative diagnoses. After treatment with methylprednisolone and intravenous immunoglobulins the patient recovered completely and remained asymptomatic until presenting two years later with optic neuritis and new demyelinating MRI lesions consistent with MS. The patient had negative tests for aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies, and met 2017 McDonald criteria for MS.
This case adds to the sparse literature on overlap between NMDAR encephalitis and MS, suggesting a potential link between the autoimmune processes in these disorders. Further research is warranted to elucidate the underlying mechanisms and optimize management strategies for such cases.
抗n -甲基- d -天冬氨酸受体（NMDAR）脑炎是一种自身免疫性疾病，其特征是IgG自身抗体靶向NMDAR的GluN1亚基。我们报告一例20岁男性诊断为NMDAR脑炎，两年后出现多发性硬化症（MS）的症状和MRI病变。患者最初表现为抑郁症状，并发展为严重的神经精神症状，包括紧张症、幻觉和吞咽困难。在脑脊液和血清中发现抗nmdar抗体阳性，广泛的调查，包括成像和脑脊液分析，排除了其他诊断。经甲基强的松龙和静脉注射免疫球蛋白治疗后，患者完全恢复，无症状，直到两年后出现视神经炎和新的脱髓鞘MRI病变，与MS一致。患者水通道蛋白-4和髓鞘少突胶质细胞糖蛋白抗体检测阴性，符合2017年MS的麦当劳标准。该病例增加了NMDAR脑炎与MS重叠的稀疏文献。提示这些疾病的自身免疫过程之间存在潜在联系需要进一步的研究来阐明潜在的机制和优化此类病例的管理策略。
{"title":"MS developing after NMDAR encephalitis: A Portuguese case report and literature review","authors":"Ana Rita Castro , Daniela Oliveira , Bárbara Teixeira , João Macedo Cunha , Ivânia Alves , Luis Ruano","doi":"10.1016/j.nerep.2025.100256","DOIUrl":"10.1016/j.nerep.2025.100256","url":null,"abstract":"<div><div>Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterized by IgG autoantibodies targeting the GluN1 subunit of the NMDAR. We present a case of a 20-year-old male diagnosed with NMDAR encephalitis, who two years later developed symptoms and MRI lesions suggestive of multiple sclerosis (MS).</div><div>The patient initially exhibited depressive symptoms progressing to severe neuropsychiatric manifestations including catatonia, hallucinations, and dysphagia. Positive anti-NMDAR antibodies in the CSF and serum were found, and extensive investigations, including imaging and CSF analysis, ruled out alternative diagnoses. After treatment with methylprednisolone and intravenous immunoglobulins the patient recovered completely and remained asymptomatic until presenting two years later with optic neuritis and new demyelinating MRI lesions consistent with MS. The patient had negative tests for aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies, and met 2017 McDonald criteria for MS.</div><div>This case adds to the sparse literature on overlap between NMDAR encephalitis and MS, suggesting a potential link between the autoimmune processes in these disorders. Further research is warranted to elucidate the underlying mechanisms and optimize management strategies for such cases.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100256"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143863354","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}
Pub Date : 2025-01-01DOI: 10.1016/j.nerep.2024.100235
Dr. Jānis Dāvis Osipovs , Dr. Eva Šankova , Prof. Guntis Karelis , Dr. Ziedīte Želve , Dr. Elīna Polunosika , Mg. sc. sal. Līga Savicka
Myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder that can present with various early clinical symptoms and signs. The diagnosis of MG with bulbar symptom onset can be clinically challenging for clinicians with limited experience with neuromuscular disorders.
This case report presents an elderly patient with an abrupt onset of orthostatic dysarthria, in whom a high level of diagnostic suspicion leads to a short period until confirmation of diagnosis. This clinical case shows symptomatic treatment initiation on the 4th day after the onset of symptoms in a patient with bulbar onset MG. Serologic confirmation of MG was received on the 11th hospitalisation day. This case report contains a short video of the evaluation of orthostatic dysarthria.
Orthostatic dysarthria is a rare presentation symptom for bulbar onset MG. Information on the evaluation of orthostatic dysarthria is sparse. Considering that bulbar onset MG can be a life-threatening condition, we would like to share our positive experience of rapid diagnosis and successful treatment.
{"title":"Sudden onset orthostatic dysarthria as a presenting symptom of bulbar onset myasthenia gravis: a video case report","authors":"Dr. Jānis Dāvis Osipovs , Dr. Eva Šankova , Prof. Guntis Karelis , Dr. Ziedīte Želve , Dr. Elīna Polunosika , Mg. sc. sal. Līga Savicka","doi":"10.1016/j.nerep.2024.100235","DOIUrl":"10.1016/j.nerep.2024.100235","url":null,"abstract":"<div><div>Myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder that can present with various early clinical symptoms and signs. The diagnosis of MG with bulbar symptom onset can be clinically challenging for clinicians with limited experience with neuromuscular disorders.</div><div>This case report presents an elderly patient with an abrupt onset of orthostatic dysarthria, in whom a high level of diagnostic suspicion leads to a short period until confirmation of diagnosis. This clinical case shows symptomatic treatment initiation on the 4th day after the onset of symptoms in a patient with bulbar onset MG. Serologic confirmation of MG was received on the 11th hospitalisation day. This case report contains a short video of the evaluation of orthostatic dysarthria.</div><div>Orthostatic dysarthria is a rare presentation symptom for bulbar onset MG. Information on the evaluation of orthostatic dysarthria is sparse. Considering that bulbar onset MG can be a life-threatening condition, we would like to share our positive experience of rapid diagnosis and successful treatment.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100235"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143104688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01DOI: 10.1016/j.nerep.2025.100255
Samar Antoine Abbas , Jad Costa , Joelle Saba , Christian Matta , Halim Abboud
Objectives
report the different etiologies of Longitudinally Extensive Transverse Myelitis (LETM), along with their key characteristics.
Methods
A retrospective study was conducted at Hotel-Dieu de France University Hospital, Beirut, Lebanon. We collected demographic, clinical, radiologic and biochemical data of patients admitted for LETM between January 2021 and September 2024. Patients with compressive myelopathy or missing data were excluded. LETM characteristics were described in the whole sample, as well as in patients with inflammatory myelitis and those with spinal dural arteriovenous fistula (SDAVF).
Results
32 patients were selected, of whom 12 patients were excluded. The most common cause of LETM was SDAVF (25%), followed by MOG antibody-associated disease, multiple sclerosis, idiopathic transverse myelitis, neurosarcoidosis and infectious myelitis (10% each). There was only one case of Neuromyelitis Optica Spectrum Disorder (NMOSD). Other causes included MOG-negative acute disseminated encephalomyelitis, paraneoplastic myelitis, spinal cord neoplasm and subacute combined degeneration of spinal cord (5% each). In total, there were 13 cases of inflammatory myelitis (65%). Patients with SDAVF were predominantly males (80%) and relatively older than those with inflammatory myelitis. Different MRI patterns were described: all inflammatory LETM involved cervical and/or thoracic cord, 69% were partial and enhanced on postcontrast sequences. Vascular myelopathy affected thoracolumbar region in 80% of cases. Perimedullary flow voids were common (80%) but inconsistent.
Conclusions
Neurologist should think beyond NMOSD in case of LETM. The presence of thoracolumbar LETM with or without perimedullary flow voids requires MR angiography, before diagnostic lumbar puncture or empirical steroid therapy.
{"title":"Longitudinally extensive transverse myelitis: Etiologies and common diagnostic pitfalls","authors":"Samar Antoine Abbas , Jad Costa , Joelle Saba , Christian Matta , Halim Abboud","doi":"10.1016/j.nerep.2025.100255","DOIUrl":"10.1016/j.nerep.2025.100255","url":null,"abstract":"<div><h3>Objectives</h3><div>report the different etiologies of Longitudinally Extensive Transverse Myelitis (LETM), along with their key characteristics.</div></div><div><h3>Methods</h3><div>A retrospective study was conducted at Hotel-Dieu de France University Hospital, Beirut, Lebanon. We collected demographic, clinical, radiologic and biochemical data of patients admitted for LETM between January 2021 and September 2024. Patients with compressive myelopathy or missing data were excluded. LETM characteristics were described in the whole sample, as well as in patients with inflammatory myelitis and those with spinal dural arteriovenous fistula (SDAVF).</div></div><div><h3>Results</h3><div>32 patients were selected, of whom 12 patients were excluded. The most common cause of LETM was SDAVF (25%), followed by MOG antibody-associated disease, multiple sclerosis, idiopathic transverse myelitis, neurosarcoidosis and infectious myelitis (10% each). There was only one case of Neuromyelitis Optica Spectrum Disorder (NMOSD). Other causes included MOG-negative acute disseminated encephalomyelitis, paraneoplastic myelitis, spinal cord neoplasm and subacute combined degeneration of spinal cord (5% each). In total, there were 13 cases of inflammatory myelitis (65%). Patients with SDAVF were predominantly males (80%) and relatively older than those with inflammatory myelitis. Different MRI patterns were described: all inflammatory LETM involved cervical and/or thoracic cord, 69% were partial and enhanced on postcontrast sequences. Vascular myelopathy affected thoracolumbar region in 80% of cases. Perimedullary flow voids were common (80%) but inconsistent.</div></div><div><h3>Conclusions</h3><div>Neurologist should think beyond NMOSD in case of LETM. The presence of thoracolumbar LETM with or without perimedullary flow voids requires MR angiography, before diagnostic lumbar puncture or empirical steroid therapy.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100255"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143738719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Data regarding idiopathic autoimmune-mediated optic neuritis is limited to visual acuity at specific time points post-treatment, lacking longitudinal comparisons. This study compared visual outcomes in patients with severe visual impairment due to optic neuritis who were aquaporin-4 (AQP4) immunoglobulin G (IgG)-positive, myelin oligodendrocyte glycoprotein (MOG)-IgG-positive, or double seronegative.
Methods
This retrospective longitudinal study was conducted at the Neurological Institute of Thailand, examining visual outcomes among three patient groups presenting with severe visual impairment—defined as best corrected visual acuity (BCVA) of 20/200 or worse—between June 2020 and May 2023. Visual outcomes were assessed with the “time to good visual recovery”—defined as ≥66.77 % improvement in BCVA from post-attack to baseline—and “complete visual recovery”—defined as BCVA returning to baseline.
Results
This study included 45 affected eyes of 30 patients, grouped as AQP4-IgG-positive (n = 10), MOG-IgG-positive (n = 5), and double seronegative (n = 30). Median BCVA at onset was 1.7 (logMAR). Using MOG-IgG as a comparator, the hazard ratios for complete visual recovery in the AQP4-IgG-positive and double seronegative groups were 0.158 (p = 0.135) and 0.421 (p = 0.288), respectively. For good visual recovery, the AQP4-IgG-positive and double seronegative subtypes had hazard ratios of 0.187 (p = 0.013) and 0.189 (p = 0.005), respectively, compared with the MOG-IgG-positive subtype. Furthermore, all MOG-IgG-positive cases achieved good visual recovery, in contrast to fewer than 50 % of the AQP4-IgG-positive and double seronegative subtypes.
Conclusion
The MOG-IgG-positive subtype exhibited the best visual prognosis and the shortest recovery time compared to the AQP4-IgG-positive and double seronegative subtypes.
{"title":"Comparison of visual outcomes in patients with aquaporin 4 immunoglobulin g-positive, myelin oligodendrocyte glycoprotein immunoglobulin g-positive, and double seronegative optic neuritis following severe optic neuritis","authors":"Rawipreeya Laosirirat, Metha Apiwattanakul, Saharat Aungsumart","doi":"10.1016/j.nerep.2025.100260","DOIUrl":"10.1016/j.nerep.2025.100260","url":null,"abstract":"<div><h3>Background</h3><div>Data regarding idiopathic autoimmune-mediated optic neuritis is limited to visual acuity at specific time points post-treatment, lacking longitudinal comparisons. This study compared visual outcomes in patients with severe visual impairment due to optic neuritis who were aquaporin-4 (AQP4) immunoglobulin G (IgG)-positive, myelin oligodendrocyte glycoprotein (MOG)-IgG-positive, or double seronegative.</div></div><div><h3>Methods</h3><div>This retrospective longitudinal study was conducted at the Neurological Institute of Thailand, examining visual outcomes among three patient groups presenting with severe visual impairment—defined as best corrected visual acuity (BCVA) of 20/200 or worse—between June 2020 and May 2023. Visual outcomes were assessed with the “time to good visual recovery”—defined as ≥66.77 % improvement in BCVA from post-attack to baseline—and “complete visual recovery”—defined as BCVA returning to baseline.</div></div><div><h3>Results</h3><div>This study included 45 affected eyes of 30 patients, grouped as AQP4-IgG-positive (<em>n</em> = 10), MOG-IgG-positive (<em>n</em> = 5), and double seronegative (<em>n</em> = 30). Median BCVA at onset was 1.7 (logMAR). Using MOG-IgG as a comparator, the hazard ratios for complete visual recovery in the AQP4-IgG-positive and double seronegative groups were 0.158 (<em>p</em> = 0.135) and 0.421 (<em>p</em> = 0.288), respectively. For good visual recovery, the AQP4-IgG-positive and double seronegative subtypes had hazard ratios of 0.187 (<em>p</em> = 0.013) and 0.189 (<em>p</em> = 0.005), respectively, compared with the MOG-IgG-positive subtype. Furthermore, all MOG-IgG-positive cases achieved good visual recovery, in contrast to fewer than 50 % of the AQP4-IgG-positive and double seronegative subtypes.</div></div><div><h3>Conclusion</h3><div>The MOG-IgG-positive subtype exhibited the best visual prognosis and the shortest recovery time compared to the AQP4-IgG-positive and double seronegative subtypes.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100260"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144239683","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}
扫码关注我们
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号