Neurogenic pulmonary edema (NPE) is a well-recognized manifestation of central nervous system insults (Theodore J, 1976). Both NPE and left ventricular failure can be seen in relapses of relapsing remitting multiple sclerosis (RRMS) (Melin J, 1996). This article presents two case reports demonstrating NPE presentation in the absence of left ventricular failure in addition to a review of the existing literature. The presented cases as well as previously published cases suggest NPE to be a rare but potentially fatal complication of MS relapse, especially in patients with medullary demyelination. This article seeks to encourage clinicians to maintain a high index of suspicion of NPE in MS patients presenting with unexplained cardiopulmonary symptoms and emphasizes early detection of brainstem lesions and continuous monitoring of cardiopulmonary function to support improved patient outcomes.
{"title":"Neurogenic pulmonary edema as a manifestation of multiple sclerosis relapses","authors":"Tayyebeh Saberbaghi , Niloofar Mortezaei , Elaheh Shahmiri , Reza Vosoughi","doi":"10.1016/j.nerep.2026.100275","DOIUrl":"10.1016/j.nerep.2026.100275","url":null,"abstract":"<div><div>Neurogenic pulmonary edema (NPE) is a well-recognized manifestation of central nervous system insults (Theodore J, 1976). Both NPE and left ventricular failure can be seen in relapses of relapsing remitting multiple sclerosis (RRMS) (Melin J, 1996). This article presents two case reports demonstrating NPE presentation in the absence of left ventricular failure in addition to a review of the existing literature. The presented cases as well as previously published cases suggest NPE to be a rare but potentially fatal complication of MS relapse, especially in patients with medullary demyelination. This article seeks to encourage clinicians to maintain a high index of suspicion of NPE in MS patients presenting with unexplained cardiopulmonary symptoms and emphasizes early detection of brainstem lesions and continuous monitoring of cardiopulmonary function to support improved patient outcomes.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"9 ","pages":"Article 100275"},"PeriodicalIF":0.0,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146038525","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}
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system. Immunomodulatory therapies are frequently employed in disease management, but they may increase susceptibility to infections.
Case Presentation
A patient with MS on ocrelizumab presented with a rapidly progressing paraclitoral lesion. Serological tests for Treponema pallidum were initially negative, but a wedge excision of the lesion tested positive for syphilis.
Conclusions
Ocrelizumab may interfere with the serological diagnosis of infectious diseases such as syphilis. This case underscores the need for heightened vigilance in diagnosing patients on immunomodulatory therapies, especially when they remain seronegative for syphilis.
{"title":"Rapid progression of a painful vulvar lesion in a multiple sclerosis patient on ocrelizumab: A case report","authors":"Joep Neven , Coen Schreuder , Robbert Bentvelsen , Jean-Luc Murk , Janneke Hoogstad-van Evert , Caspar van Munster","doi":"10.1016/j.nerep.2026.100276","DOIUrl":"10.1016/j.nerep.2026.100276","url":null,"abstract":"<div><h3>Background</h3><div>Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system. Immunomodulatory therapies are frequently employed in disease management, but they may increase susceptibility to infections.</div></div><div><h3>Case Presentation</h3><div>A patient with MS on ocrelizumab presented with a rapidly progressing paraclitoral lesion. Serological tests for <em>Treponema pallidum</em> were initially negative, but a wedge excision of the lesion tested positive for syphilis.</div></div><div><h3>Conclusions</h3><div>Ocrelizumab may interfere with the serological diagnosis of infectious diseases such as syphilis. This case underscores the need for heightened vigilance in diagnosing patients on immunomodulatory therapies, especially when they remain seronegative for syphilis.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"9 ","pages":"Article 100276"},"PeriodicalIF":0.0,"publicationDate":"2026-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146038526","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 : 2026-01-05DOI: 10.1016/j.nerep.2026.100274
Kana Ohnari , Kazumasa Okada , Masayuki Ikegawa , Satoru Ide , Yukio Iwanaka
Background
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune neurological disease mediated by anti-aquaporin-4 (AQP4) antibodies. Current diagnostic criteria prioritize AQP4 antibody positivity, enabling diagnosis even in atypical cases. Here, we report an atypical case of AQP4 antibody-positive NMOSD.
Case Presentation
The patient initially presented with relapsing optic neuritis and, after a prolonged course, developed slowly evolving gait disturbance and cognitive decline with leukodystrophy-like white matter lesions on magnetic resonance imaging under treatment with prednisolone. No other relevant autoantibodies except AQP4 antibody were detected. Oligoclonal IgG bands were persistently detected, and interleukin-6 levels were abnormally elevated in cerebrospinal fluid. Neurological deficits and magnetic resonance imaging abnormalities improved following immunotherapy.
Conclusions
This case highlights that NMOSD can rarely present with atypical features, including slowly evolving neurological symptoms and leukodystrophy-like imaging findings. It underscores the need for long-term monitoring, even in cases that initially appear typical, as well as the importance of appropriate immunotherapy.
{"title":"Atypical neuromyelitis optica spectrum disorder with immunotherapy-responsive neurological symptoms and leukodystrophy-like white matter lesions: A case report","authors":"Kana Ohnari , Kazumasa Okada , Masayuki Ikegawa , Satoru Ide , Yukio Iwanaka","doi":"10.1016/j.nerep.2026.100274","DOIUrl":"10.1016/j.nerep.2026.100274","url":null,"abstract":"<div><h3>Background</h3><div>Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune neurological disease mediated by anti-aquaporin-4 (AQP4) antibodies. Current diagnostic criteria prioritize AQP4 antibody positivity, enabling diagnosis even in atypical cases. Here, we report an atypical case of AQP4 antibody-positive NMOSD.</div></div><div><h3>Case Presentation</h3><div>The patient initially presented with relapsing optic neuritis and, after a prolonged course, developed slowly evolving gait disturbance and cognitive decline with leukodystrophy-like white matter lesions on magnetic resonance imaging under treatment with prednisolone. No other relevant autoantibodies except AQP4 antibody were detected. Oligoclonal IgG bands were persistently detected, and interleukin-6 levels were abnormally elevated in cerebrospinal fluid. Neurological deficits and magnetic resonance imaging abnormalities improved following immunotherapy.</div></div><div><h3>Conclusions</h3><div>This case highlights that NMOSD can rarely present with atypical features, including slowly evolving neurological symptoms and leukodystrophy-like imaging findings. It underscores the need for long-term monitoring, even in cases that initially appear typical, as well as the importance of appropriate immunotherapy.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"9 ","pages":"Article 100274"},"PeriodicalIF":0.0,"publicationDate":"2026-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145979626","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 : 2025-12-08DOI: 10.1016/j.nerep.2025.100273
Marie E Varnet , Cynthia Wang
Interleukin-6 (IL-6) receptor blockers like Tocilizumab (TCZ) have shown safety and efficacy when used in relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), but reported use in the acute phase is scarce. Even less is known about the potential use of TCZ based on clinical suspicion of MOGAD, in conjunction with standard therapies (i.e. high-dose steroids, intravenous immunoglobulins, and plasma exchange); rather than after these have failed to improve symptoms.
We present the case of a 4-year-old boy with acute CNS demyelination and malignant cerebral edema suspected to be secondary to MOGAD. His symptoms were not responsive to high-dose steroids and clinical status was continuing to decline alongside evidence of impending brain herniation.
The patient steadily improved following TCZ administration, with ultimately excellent recovery. He regained baseline cognitive and communication abilities and near baseline motor abilities. Confirmatory MOG-IgG serum titers returned following the first dose of TCZ.
IL-6 receptor blockading agents like TCZ, may be considered acutely in life-threatening CNS demyelinating disease when there is malignant cerebral edema and intracranial hypertension, which can often be associated with MOGAD.
{"title":"Urgent use of tocilizumab in suspected MOGAD with malignant cerebral edema","authors":"Marie E Varnet , Cynthia Wang","doi":"10.1016/j.nerep.2025.100273","DOIUrl":"10.1016/j.nerep.2025.100273","url":null,"abstract":"<div><div>Interleukin-6 (IL-6) receptor blockers like Tocilizumab (TCZ) have shown safety and efficacy when used in relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), but reported use in the acute phase is scarce. Even less is known about the potential use of TCZ based on clinical suspicion of MOGAD, in conjunction with standard therapies (i.e. high-dose steroids, intravenous immunoglobulins, and plasma exchange); rather than after these have failed to improve symptoms.</div><div>We present the case of a 4-year-old boy with acute CNS demyelination and malignant cerebral edema suspected to be secondary to MOGAD. His symptoms were not responsive to high-dose steroids and clinical status was continuing to decline alongside evidence of impending brain herniation.</div><div>The patient steadily improved following TCZ administration, with ultimately excellent recovery. He regained baseline cognitive and communication abilities and near baseline motor abilities. Confirmatory MOG-IgG serum titers returned following the first dose of TCZ.</div><div>IL-6 receptor blockading agents like TCZ, may be considered acutely in life-threatening CNS demyelinating disease when there is malignant cerebral edema and intracranial hypertension, which can often be associated with MOGAD.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"9 ","pages":"Article 100273"},"PeriodicalIF":0.0,"publicationDate":"2025-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145898178","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 : 2025-12-01DOI: 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}
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