Pub Date : 2025-12-01DOI: 10.1016/j.nerep.2025.100271
Amanda Piquet , Lowe Mallory , Jeffrey Bennett
Introduction
Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system associated with pathogenic aquaporin-4 (AQP4) immunoglobulin G (IgG) antibodies. Without treatment, patients experience recurrent attacks, which can lead to significant disability from accumulated neurological damage. Agents targeting CD19, interleukin-6 receptor, and complement C5 have been approved for treatment of patients with AQP4-IgG+ NMOSD. Real-world data on patients transitioning from an anti-CD19 therapy to a C5 inhibitor are limited, and there are no published data on transitioning between C5 inhibitors, eculizumab and ravulizumab.
Methods
We describe a patient with AQP4-IgG+ NMOSD who transitioned from inebilizumab to eculizumab after developing hypogammaglobulinemia and infections; the patient later transitioned to ravulizumab for less frequent dosing.
Results
Throughout treatment with inebilizumab, eculizumab, and ravulizumab, the patient remained clinically and radiographically stable.
Conclusion
This complex case highlights the importance of monitoring and managing treatment-related complications associated with anti-CD19 therapy and key factors to consider when transitioning from B-cell–depleting therapy to complement inhibition therapy.
{"title":"Transitioning to a C5 inhibitor for aquaporin-4 antibody–positive neuromyelitis optica spectrum disorder intolerant of anti-CD19 therapy: a case report","authors":"Amanda Piquet , Lowe Mallory , Jeffrey Bennett","doi":"10.1016/j.nerep.2025.100271","DOIUrl":"10.1016/j.nerep.2025.100271","url":null,"abstract":"<div><h3>Introduction</h3><div>Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease of the central nervous system associated with pathogenic aquaporin-4 (AQP4) immunoglobulin G (IgG) antibodies. Without treatment, patients experience recurrent attacks, which can lead to significant disability from accumulated neurological damage. Agents targeting CD19, interleukin-6 receptor, and complement C5 have been approved for treatment of patients with AQP4-IgG+ NMOSD. Real-world data on patients transitioning from an anti-CD19 therapy to a C5 inhibitor are limited, and there are no published data on transitioning between C5 inhibitors, eculizumab and ravulizumab.</div></div><div><h3>Methods</h3><div>We describe a patient with AQP4-IgG+ NMOSD who transitioned from inebilizumab to eculizumab after developing hypogammaglobulinemia and infections; the patient later transitioned to ravulizumab for less frequent dosing.</div></div><div><h3>Results</h3><div>Throughout treatment with inebilizumab, eculizumab, and ravulizumab, the patient remained clinically and radiographically stable.</div></div><div><h3>Conclusion</h3><div>This complex case highlights the importance of monitoring and managing treatment-related complications associated with anti-CD19 therapy and key factors to consider when transitioning from B-cell–depleting therapy to complement inhibition therapy.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100271"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145623217","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 : 2025-11-15DOI: 10.1016/j.nerep.2025.100270
Brianna N Brun , Monica E Kim , Rae Leonor F Gumayan , Anne M Connolly , Sean C Rose , Richard Miller , Jennifer L McKinney , Hersh Varma , Kelsey E Poisson
We present a case of pediatric aquaporin-4 positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD) with delayed diagnosis due to co-occurrence of neurofascin-155 (NF155) nodopathy. A 7-year-old African American girl presented with vomiting, weight loss, dysarthria, and dysphagia. Examination showed multiple cranial nerve abnormalities, reduced pinprick sensation distal to mid-shin, and ascending areflexia. A non-enhancing lesion centered in the dorsal left medulla was detected on MRI. Nerve conduction studies revealed a length-dependent, demyelinating, sensorimotor polyneuropathy. IgM and IgG NF155 antibodies were present in serum. Treatment included intravenous immunoglobulin (IVIG) and high dose methylprednisolone followed by a gradual corticosteroid taper over 11 months with good recovery and normalization of imaging findings. One year later, the child again presented with brainstem syndrome, but without peripheral nerve involvement, and was found to have enhancing dorsal pontine and medullary lesions with additional involvement in the septum pellucidum and fornices. She responded well to methylprednisolone, IVIG, and plasmapheresis and later tested positive for AQP4-IgG. AQP4+ NMOSD frequently co-occurs with other autoantibody-mediated conditions which may confound the diagnostic approach. Children presenting with any core clinical characteristic of NMOSD should be tested for AQP4-IgG, even in the setting of additional symptoms localizing to the peripheral nervous system or to other systemic autoimmune disease.
{"title":"Concurrent neuromyelitis optica spectrum disorder and neurofascin-155 antibody associated peripheral demyelination in a child","authors":"Brianna N Brun , Monica E Kim , Rae Leonor F Gumayan , Anne M Connolly , Sean C Rose , Richard Miller , Jennifer L McKinney , Hersh Varma , Kelsey E Poisson","doi":"10.1016/j.nerep.2025.100270","DOIUrl":"10.1016/j.nerep.2025.100270","url":null,"abstract":"<div><div>We present a case of pediatric aquaporin-4 positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD) with delayed diagnosis due to co-occurrence of neurofascin-155 (NF155) nodopathy. A 7-year-old African American girl presented with vomiting, weight loss, dysarthria, and dysphagia. Examination showed multiple cranial nerve abnormalities, reduced pinprick sensation distal to mid-shin, and ascending areflexia. A non-enhancing lesion centered in the dorsal left medulla was detected on MRI. Nerve conduction studies revealed a length-dependent, demyelinating, sensorimotor polyneuropathy. IgM and IgG NF155 antibodies were present in serum. Treatment included intravenous immunoglobulin (IVIG) and high dose methylprednisolone followed by a gradual corticosteroid taper over 11 months with good recovery and normalization of imaging findings. One year later, the child again presented with brainstem syndrome, but without peripheral nerve involvement, and was found to have enhancing dorsal pontine and medullary lesions with additional involvement in the septum pellucidum and fornices. She responded well to methylprednisolone, IVIG, and plasmapheresis and later tested positive for AQP4-IgG. AQP4+ NMOSD frequently co-occurs with other autoantibody-mediated conditions which may confound the diagnostic approach. Children presenting with any core clinical characteristic of NMOSD should be tested for AQP4-IgG, even in the setting of additional symptoms localizing to the peripheral nervous system or to other systemic autoimmune disease.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100270"},"PeriodicalIF":0.0,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145579037","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 : 2025-10-14DOI: 10.1016/j.nerep.2025.100269
Rong Fu , Ling Gong , Zhongyong Peng , Ying Zhang , Zhun Ding , Ying Bai
Background
Antibody against glycine receptor 1 (GlyR1) is frequently detected in autoimmune encephalitis, where they primarily manifest as stiff- person syndrome or progressive encephalomyelitis with rigidity and myoclonus and epilepsy. While GlyR1 antibody positivity is often associated with thymomas, B-cell lymphoma, Hodgkin lymphoma, breast cancer, and small cell lung cancer, its association with glioma has not been reported to date.
Case presentation
We report a 47-year-old male patient who presented with acute symptomatic seizures and lesions in the left temporal and hippocampus are typically detected by magnetic resonance imaging. Antibodies related to autoimmune encephalitis was tested positive for GlyR1 receptor antibodies in serum (titer is 1:30) but not in cerebrospinal fluid. The patient underwent resection of a left cerebral hemisphere lesion, which was pathologically diagnosed as astrocytoma, and carried out radiotherapy and chemotherapy. Without postoperative immunotherapy, the patient remained free of epileptic symptoms following oral sodium valproate antiepileptic treatment. We thus attributed the epilepsy to astrocytoma rather than GlyR antibody-associated autoimmune encephalitis. Due to poor immunity after radiotherapy and chemotherapy for the tumor, the patient suffered from status epilepticus and multiple organ failure, and eventually passed away.
Conclusions
This case is the first to report the coexistence of GlyR antibodies and glioma, revealing the association between autoimmune antibodies and tumors. In the future, more research needs to focus on the mechanism underlying their coexistence.
{"title":"Case report: Astrocytoma coexisting with serum glycine receptor 1 antibody","authors":"Rong Fu , Ling Gong , Zhongyong Peng , Ying Zhang , Zhun Ding , Ying Bai","doi":"10.1016/j.nerep.2025.100269","DOIUrl":"10.1016/j.nerep.2025.100269","url":null,"abstract":"<div><h3>Background</h3><div>Antibody against glycine receptor 1 (GlyR1) is frequently detected in autoimmune encephalitis, where they primarily manifest as stiff- person syndrome or progressive encephalomyelitis with rigidity and myoclonus and epilepsy. While GlyR1 antibody positivity is often associated with thymomas, B-cell lymphoma, Hodgkin lymphoma, breast cancer, and small cell lung cancer, its association with glioma has not been reported to date.</div></div><div><h3>Case presentation</h3><div>We report a 47-year-old male patient who presented with acute symptomatic seizures and lesions in the left temporal and hippocampus are typically detected by magnetic resonance imaging. Antibodies related to autoimmune encephalitis was tested positive for GlyR1 receptor antibodies in serum (titer is 1:30) but not in cerebrospinal fluid. The patient underwent resection of a left cerebral hemisphere lesion, which was pathologically diagnosed as astrocytoma, and carried out radiotherapy and chemotherapy. Without postoperative immunotherapy, the patient remained free of epileptic symptoms following oral sodium valproate antiepileptic treatment. We thus attributed the epilepsy to astrocytoma rather than GlyR antibody-associated autoimmune encephalitis. Due to poor immunity after radiotherapy and chemotherapy for the tumor, the patient suffered from status epilepticus and multiple organ failure, and eventually passed away.</div></div><div><h3>Conclusions</h3><div>This case is the first to report the coexistence of GlyR antibodies and glioma, revealing the association between autoimmune antibodies and tumors. In the future, more research needs to focus on the mechanism underlying their coexistence.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100269"},"PeriodicalIF":0.0,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145320247","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 : 2025-10-02DOI: 10.1016/j.nerep.2025.100268
Y. Mishan , D. Schwartz , D. Elefant , S. Gandelman
{"title":"Late-onset MOGAD: A case series and literature review","authors":"Y. Mishan , D. Schwartz , D. Elefant , S. Gandelman","doi":"10.1016/j.nerep.2025.100268","DOIUrl":"10.1016/j.nerep.2025.100268","url":null,"abstract":"","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100268"},"PeriodicalIF":0.0,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145265962","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 : 2025-09-26DOI: 10.1016/j.nerep.2025.100267
Monique Anderson, Michael Levy
There are currently four FDA approved medications for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in the United States. Each of these medications is formulated as a monoclonal antibody. While these novel treatments have greatly advanced the prevention of relapses in NMOSD, they can unfortunately be cleared by immunoglobulin or plasma exchange (PLEX). Given the high prevalence of hypogammaglobulinemia with extended use of B-cell depletion therapites, and rituximab in particular, this can present a treatment dilemma when attempting to correct and treat this immunodeficiency in NMOSD patients requiring continued maintenance therapy for relapse prevention. Here we detail a case of an NMOSD patient with severe hypogammaglobulinemia resulting from previous long-term rituximab use and how this was treated with supplemental immunoglobulin in conjunction with zilucoplan for relapse prevention.
{"title":"A case of a patient with neuromyelitis optica spectrum disorder with hypogammaglobulinemia managed with immunoglobulin and zilucoplan","authors":"Monique Anderson, Michael Levy","doi":"10.1016/j.nerep.2025.100267","DOIUrl":"10.1016/j.nerep.2025.100267","url":null,"abstract":"<div><div>There are currently four FDA approved medications for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in the United States. Each of these medications is formulated as a monoclonal antibody. While these novel treatments have greatly advanced the prevention of relapses in NMOSD, they can unfortunately be cleared by immunoglobulin or plasma exchange (PLEX). Given the high prevalence of hypogammaglobulinemia with extended use of B-cell depletion therapites, and rituximab in particular, this can present a treatment dilemma when attempting to correct and treat this immunodeficiency in NMOSD patients requiring continued maintenance therapy for relapse prevention. Here we detail a case of an NMOSD patient with severe hypogammaglobulinemia resulting from previous long-term rituximab use and how this was treated with supplemental immunoglobulin in conjunction with zilucoplan for relapse prevention.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100267"},"PeriodicalIF":0.0,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145219188","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 : 2025-09-15DOI: 10.1016/j.nerep.2025.100266
João Vitor Mahler , Arvind Ravi , Kristin Galetta , Giovanna Manzano
Background
Immune checkpoint inhibitors (ICIs) have transformed cancer therapy but can lead to serious immune‐related adverse events (irAEs), including provocation of autoimmune encephalitis.
Case Report
A middle-aged woman presented with new-onset confusion, memory impairment, epileptic seizures leading to a diagnosis of limbic encephalitis, as supported by objective findings of inflammation on neuroimaging and CSF analysis. Metastatic urothelial carcinoma was discovered during her diagnostic evaluation for limbic encephalitis. Administration of high-dose corticosteroids and plasma exchange achieved neurologic stabilization acutely; however, her malignancy proved challenging to treat.
Case Presentation
Tumor progression prompted a multi-disciplinary plan to trial ICIs despite concern of resultant neurotoxicity. To offset potential iatrogenic neuroinflammation, the patient received maintenance intravenous immunoglobulin (IVIg) 0.4 g/kg ideal body weight biweekly in conjunction with oncologic-directed immunotherapy. Over a six-month follow-up period, the patient maintained neurologic stability without worsening encephalitis. She tolerated the combined IVIg–ICI regimen without infusion reactions or exacerbation of neurologic toxicity.
Conclusion
This case suggests that prophylactic maintenance IVIg may allow for safe administration of ICIs in patients with pre-morbid limbic encephalitis. Prospective studies with a larger sample size are needed to validate IVIg as a strategy to prevent provocation of pre-morbid limbic encephalitis in those requiring oncologic-directed immunotherapy.
{"title":"Risk mitigation of autoimmune encephalitis recurrence in the setting of immune-checkpoint inhibitor therapy: a case report","authors":"João Vitor Mahler , Arvind Ravi , Kristin Galetta , Giovanna Manzano","doi":"10.1016/j.nerep.2025.100266","DOIUrl":"10.1016/j.nerep.2025.100266","url":null,"abstract":"<div><h3>Background</h3><div>Immune checkpoint inhibitors (ICIs) have transformed cancer therapy but can lead to serious immune‐related adverse events (irAEs), including provocation of autoimmune encephalitis.</div></div><div><h3>Case Report</h3><div>A middle-aged woman presented with new-onset confusion, memory impairment, epileptic seizures leading to a diagnosis of limbic encephalitis, as supported by objective findings of inflammation on neuroimaging and CSF analysis. Metastatic urothelial carcinoma was discovered during her diagnostic evaluation for limbic encephalitis. Administration of high-dose corticosteroids and plasma exchange achieved neurologic stabilization acutely; however, her malignancy proved challenging to treat.</div></div><div><h3>Case Presentation</h3><div>Tumor progression prompted a multi-disciplinary plan to trial ICIs despite concern of resultant neurotoxicity. To offset potential iatrogenic neuroinflammation, the patient received maintenance intravenous immunoglobulin (IVIg) 0.4 <em>g</em>/kg ideal body weight biweekly in conjunction with oncologic-directed immunotherapy. Over a six-month follow-up period, the patient maintained neurologic stability without worsening encephalitis. She tolerated the combined IVIg–ICI regimen without infusion reactions or exacerbation of neurologic toxicity.</div></div><div><h3>Conclusion</h3><div>This case suggests that prophylactic maintenance IVIg may allow for safe administration of ICIs in patients with pre-morbid limbic encephalitis. Prospective studies with a larger sample size are needed to validate IVIg as a strategy to prevent provocation of pre-morbid limbic encephalitis in those requiring oncologic-directed immunotherapy.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100266"},"PeriodicalIF":0.0,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145104481","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 : 2025-08-30DOI: 10.1016/j.nerep.2025.100265
Vivek Hari , Basavaprabhu Achappa , Ayushi Gupta , Shivananda Pai D
Introduction
Hymenoptera stings which include stings from bees, wasps, hornets, and fire ants, typically cause minor local reactions. However, systemic responses, including anaphylaxis and rare delayed complications, can occur. One such rare complication is Myasthenia Gravis (MG), a neuromuscular junction disorder characterized by skeletal muscle weakness due to antibodies against acetylcholine receptors (AChR). We report a case of acute neuromuscular weakness resembling a myasthenic crisis shortly after multiple bee stings.
Case Presentation
A 64-year-old male with type 2 diabetes and a history of Lumbar IVDP, presented after being stung by approximately 200 bees. He initially complained of facial puffiness, limb swelling, and generalized itching. He was febrile, bradycardic, and had diffuse erythema and edema. Initial management included antihistamines, systemic steroids, and stinger removal. Twelve hours later, he developed nausea, vomiting, and sudden onset dysphagia, ptosis, and proximal muscle weakness. Shortly after, he became dyspneic and required ICU admission with ventilatory support. Laboratory investigations revealed elevated CPK (2468 mcg/L) and negative Anti-AChR antibodies. On clinical grounds, he was treated with pyridostigmine and emergency plasmapheresis, resulting in gradual neuromuscular improvement. Supportive care was continued, and he was subsequently weaned off ventilator and was tracheostomized.
Conclusion
This case illustrates a rare myasthenia-like crisis triggered by multiple bee stings. Although confirmatory electrophysiological and serological evidence was lacking, the clinical presentation and therapeutic response strongly suggested neuromuscular junction dysfunction. The sequential association of bee sting and the onset of myasthenia gravis is too close to suppose chance association. Lack of immunologic abnormality may suggest the same. Though few cases of wasp sting causing Myasthenia have been reported, bee sting leading to neurological symptoms are unheard of. Our case highlights the importance of anticipating as well as early recognition of such complications.
{"title":"Myasthenic crisis following multiple bee stings: A rare case of neuromuscular dysfunction triggered by hymenoptera envenomation","authors":"Vivek Hari , Basavaprabhu Achappa , Ayushi Gupta , Shivananda Pai D","doi":"10.1016/j.nerep.2025.100265","DOIUrl":"10.1016/j.nerep.2025.100265","url":null,"abstract":"<div><h3>Introduction</h3><div>Hymenoptera stings which include stings from bees, wasps, hornets, and fire ants, typically cause minor local reactions. However, systemic responses, including anaphylaxis and rare delayed complications, can occur. One such rare complication is Myasthenia Gravis (MG), a neuromuscular junction disorder characterized by skeletal muscle weakness due to antibodies against acetylcholine receptors (AChR). We report a case of acute neuromuscular weakness resembling a myasthenic crisis shortly after multiple bee stings.</div></div><div><h3>Case Presentation</h3><div>A 64-year-old male with type 2 diabetes and a history of Lumbar IVDP, presented after being stung by approximately 200 bees. He initially complained of facial puffiness, limb swelling, and generalized itching. He was febrile, bradycardic, and had diffuse erythema and edema. Initial management included antihistamines, systemic steroids, and stinger removal. Twelve hours later, he developed nausea, vomiting, and sudden onset dysphagia, ptosis, and proximal muscle weakness. Shortly after, he became dyspneic and required ICU admission with ventilatory support. Laboratory investigations revealed elevated CPK (2468 mcg/L) and negative Anti-AChR antibodies. On clinical grounds, he was treated with pyridostigmine and emergency plasmapheresis, resulting in gradual neuromuscular improvement. Supportive care was continued, and he was subsequently weaned off ventilator and was tracheostomized.</div></div><div><h3>Conclusion</h3><div>This case illustrates a rare myasthenia-like crisis triggered by multiple bee stings. Although confirmatory electrophysiological and serological evidence was lacking, the clinical presentation and therapeutic response strongly suggested neuromuscular junction dysfunction. The sequential association of bee sting and the onset of myasthenia gravis is too close to suppose chance association. Lack of immunologic abnormality may suggest the same. Though few cases of wasp sting causing Myasthenia have been reported, bee sting leading to neurological symptoms are unheard of. Our case highlights the importance of anticipating as well as early recognition of such complications.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100265"},"PeriodicalIF":0.0,"publicationDate":"2025-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145018606","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}
Autoimmune disorders and human immunodeficiency virus (HIV) positivity rarely coexist. We report a case of anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MuSK+ MG) in an HIV positive patient. Antiretroviral therapy resulted in an immune reconstitution syndrome, resulting in worsening of MG symptoms. However, the role of additional immunomodulation in this setting has yet to be clarified.
Case Presentation
A 44-year-old woman with virologically suppressed HIV infection presented with fluctuating oculobulbar symptoms, limb weakness, and respiratory failure. These symptoms were noticed around 6 months following antiretroviral therapy (ART) modification. Electrophysiological studies and elevated anti-MuSK antibody levels confirmed MuSK + MG. An increase in the CD4 count from 115 to 681 cells/µL following the ART modification was consistent with immune reconstitution. Treatment with corticosteroids, Rituximab, and opportunistic infection prophylaxis resulted in a reduction in her MG symptoms with no adverse side effects.
Conclusions
This case underscores the complexities of managing autoimmune diseases in HIV, where ART-induced immune reconstitution may unmask or trigger autoimmunity. Early diagnosis and targeted therapies can improve outcomes. In this case, Rituximab initiation facilitated symptom control and steroid tapering. The need for a multidisciplinary approach to balance immunosuppression and infection risks has been emphasized.
{"title":"“When HIV meets MG: Is immune reconstitution in the mix?”","authors":"Aditya Vijayakrishnan Nair, Lesley Ponraj, Ajith Sivadasan, Sanjit Aaron","doi":"10.1016/j.nerep.2025.100263","DOIUrl":"10.1016/j.nerep.2025.100263","url":null,"abstract":"<div><h3>Background</h3><div>Autoimmune disorders and human immunodeficiency virus (HIV) positivity rarely coexist. We report a case of anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MuSK+ MG) in an HIV positive patient. Antiretroviral therapy resulted in an immune reconstitution syndrome, resulting in worsening of MG symptoms. However, the role of additional immunomodulation in this setting has yet to be clarified.</div></div><div><h3>Case Presentation</h3><div>A 44-year-old woman with virologically suppressed HIV infection presented with fluctuating oculobulbar symptoms, limb weakness, and respiratory failure. These symptoms were noticed around 6 months following antiretroviral therapy (ART) modification. Electrophysiological studies and elevated anti-MuSK antibody levels confirmed MuSK + MG. An increase in the CD4 count from 115 to 681 cells/µL following the ART modification was consistent with immune reconstitution. Treatment with corticosteroids, Rituximab, and opportunistic infection prophylaxis resulted in a reduction in her MG symptoms with no adverse side effects.</div></div><div><h3>Conclusions</h3><div>This case underscores the complexities of managing autoimmune diseases in HIV, where ART-induced immune reconstitution may unmask or trigger autoimmunity. Early diagnosis and targeted therapies can improve outcomes. In this case, Rituximab initiation facilitated symptom control and steroid tapering. The need for a multidisciplinary approach to balance immunosuppression and infection risks has been emphasized.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100263"},"PeriodicalIF":0.0,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144886906","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 : 2025-08-18DOI: 10.1016/j.nerep.2025.100264
Lulu Chu , Wenbo Yang , Hai Yu , Xiang Zhang , Xiaoni Liu , Xiangjun Chen
Objective
The following case report details a patient with serum and cerebrospinal fluid AQP4-MOG double-positive longitudinally extensive transverse myelitis, whose condition was found to be progressively exacerbated and which resulted in severe disability.
Case presentation
A 51-year-old female patient initially presented with pain and discomfort in the left upper limb and posterior occipital region for a period of 10 days. Unfortunately, she then developed hypoesthesia and flaccid paralysis, in addition to urinary retention within a few days. Concurrently, she exhibited symptoms consistent with area postrema syndrome (nausea, vomiting, and hiccups) and brain-stem syndrome (hoarseness and dysphagia). Concomitantly, AQP4 and MOG antibodies were identified as positive in both the serum and cerebrospinal fluid (CSF). MRI findings were consistent with longitudinally extensive transverse myelitis (LETM), spanning cervical through thoracic spinal cord levels. Despite presenting with meningismus, the patient's gadolinium-enhanced brain MRI revealed no abnormal leptomeningeal enhancement. Following treatment with pulse intravenous methylprednisolone and plasmapheresis, a stabilisation of symptoms was observed.
Conclusion
Patients presenting with longitudinally extensive transverse myelitis may exhibit a positive double-immunofluorescence test result for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) in both serum and CSF. The administration of early, more potent immunotherapy has the potential to enhance outcomes.
{"title":"Serum and cerebrospinal fluid AQP4-MOG double-positive longitudinally extensive transverse myelitis: a rare case report and literature review","authors":"Lulu Chu , Wenbo Yang , Hai Yu , Xiang Zhang , Xiaoni Liu , Xiangjun Chen","doi":"10.1016/j.nerep.2025.100264","DOIUrl":"10.1016/j.nerep.2025.100264","url":null,"abstract":"<div><h3>Objective</h3><div>The following case report details a patient with serum and cerebrospinal fluid AQP4-MOG double-positive longitudinally extensive transverse myelitis, whose condition was found to be progressively exacerbated and which resulted in severe disability.</div></div><div><h3>Case presentation</h3><div>A 51-year-old female patient initially presented with pain and discomfort in the left upper limb and posterior occipital region for a period of 10 days. Unfortunately, she then developed hypoesthesia and flaccid paralysis, in addition to urinary retention within a few days. Concurrently, she exhibited symptoms consistent with area postrema syndrome (nausea, vomiting, and hiccups) and brain-stem syndrome (hoarseness and dysphagia). Concomitantly, AQP4 and MOG antibodies were identified as positive in both the serum and cerebrospinal fluid (CSF). MRI findings were consistent with longitudinally extensive transverse myelitis (LETM), spanning cervical through thoracic spinal cord levels. Despite presenting with meningismus, the patient's gadolinium-enhanced brain MRI revealed no abnormal leptomeningeal enhancement. Following treatment with pulse intravenous methylprednisolone and plasmapheresis, a stabilisation of symptoms was observed.</div></div><div><h3>Conclusion</h3><div>Patients presenting with longitudinally extensive transverse myelitis may exhibit a positive double-immunofluorescence test result for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) in both serum and CSF. The administration of early, more potent immunotherapy has the potential to enhance outcomes.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100264"},"PeriodicalIF":0.0,"publicationDate":"2025-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145157552","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}
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