Neuroimmunology Reports最新文献

{"title":"Concurrent neuromyelitis optica spectrum disorder and neurofascin-155 antibody associated peripheral demyelination in a child","authors":"Brianna N Brun ,&nbsp;Monica E Kim ,&nbsp;Rae Leonor F Gumayan ,&nbsp;Anne M Connolly ,&nbsp;Sean C Rose ,&nbsp;Richard Miller ,&nbsp;Jennifer L McKinney ,&nbsp;Hersh Varma ,&nbsp;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":"2025-12-01","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}

{"title":"Risk mitigation of autoimmune encephalitis recurrence in the setting of immune-checkpoint inhibitor therapy: a case report","authors":"João Vitor Mahler ,&nbsp;Arvind Ravi ,&nbsp;Kristin Galetta ,&nbsp;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":"2025-12-01","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}

{"title":"Decoding a cryptic brain","authors":"Sreelakshmi Narayanan,&nbsp;Mahima Sriram,&nbsp;Tejas Shivarthi,&nbsp;Sudheeran Kannoth,&nbsp;Siby Gopinath,&nbsp;Vivek Nambiar,&nbsp;Udit Saraf,&nbsp;Gopikrishnan Unnikrishnan,&nbsp;Anandkumar Anandakuttan","doi":"10.1016/j.nerep.2025.100261","DOIUrl":"10.1016/j.nerep.2025.100261","url":null,"abstract":"<div><h3>Background</h3><div>Cryptococcosis is a deadly infection caused by Cryptococcus which majorly affects immunosuppressed individuals but may also be seen in immunocompetent hosts. It may present as meningitis, meningoencephalitis and CNS cryptococcomas. We report a unique presentation of CNS cryptococcosis.</div></div><div><h3>Results</h3><div>We report a case of a 48 year old lady with a background of diabetes mellitus and hypothyroidism, who presented with apathy and aphasia. Her routine blood investigations were normal and her MRI Brain revealed T2 fluid attenuated inversion recovery (FLAIR) hyperintensities with post contrast enhancement and diffusion restriction seen in cortical and deep white matter.</div><div>CSF showed mild pleocytosis and all infective panels and routine auto-antibody panels were negative. Considering a possible etiology of demyelination, she was pulsed with steroids during the course of disease yet worsened clinico-radiologically. Her brain biopsy was indicative of demyelination, and Cryptococcus PCR in CSF and biopsy specimen were positive. Repeat India ink and fungal culture persisted to be negative. The patient was treated with amphotericin and fluconazole.</div></div><div><h3>Conclusions</h3><div>The immunopathogenesis in cryptococcal infection is complex and the fungus-host interaction determines the outcome. Our case showed extensive demyelination due to the excess inflammation mounted by the host, but the process was ineffective in clearing the fungus as evidenced by PCR positivity on CSF and biopsy specimens. Worsening on steroids and positive response to anticryptococcal regime gives the evidence of a fungus mediated disease. This case shows that any demyelination should be extensively investigated for secondary causes before labelling it resistant.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"8 ","pages":"Article 100261"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144665893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum and cerebrospinal fluid AQP4-MOG double-positive longitudinally extensive transverse myelitis: a rare case report and literature review","authors":"Lulu Chu ,&nbsp;Wenbo Yang ,&nbsp;Hai Yu ,&nbsp;Xiang Zhang ,&nbsp;Xiaoni Liu ,&nbsp;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":"2025-12-01","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}

{"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 ,&nbsp;Lowe Mallory ,&nbsp;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}

{"title":"Myasthenic crisis following multiple bee stings: A rare case of neuromuscular dysfunction triggered by hymenoptera envenomation","authors":"Vivek Hari ,&nbsp;Basavaprabhu Achappa ,&nbsp;Ayushi Gupta ,&nbsp;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":"2025-12-01","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}

{"title":"Atypical initial presentation of MOGAD in a patient with probable tuberculous meningoencephalitis: Case report","authors":"Diego Canales,&nbsp;Stefany Espinoza,&nbsp;Maria Burgos,&nbsp;Pablo Orosco","doi":"10.1016/j.nerep.2024.100237","DOIUrl":"10.1016/j.nerep.2024.100237","url":null,"abstract":"<div><div>Tuberculosis (TB) is an endemic disease in our country that effects on various organs and at the nervous system. The diagnosis requires confirmatory tests, however if the suspicion is high, empirical treatment can be initiated with subsequent follow-up of the patient.</div><div>TB has been associated with neuromyelitis optica, however it didn't associate with MOG antibody disease (MOGAD). MOGAD is a rare entity in the adult population; its main manifestation is bilateral optic neuritis or acute disseminated encephalitis. Which also requires confirmatory tests and immunosuppressive treatment.</div><div>We report the case of a patient diagnosed with TB meningoencephalitis, in the second month of empirical treatment with anti-TB drugs, who was admitted to our service due to persistence of epileptic seizures and greater deterioration in the level of alertness. She was tested for infectious, autoimmune, tumor and metabolic diseases, which came out within the normal range. Magnetic resonance imaging showed apparently sequelae diffuse bilateral leukodystrophy without contrast enhancement. The possibility of autoimmune encephalitis was evaluated. Therefore, it decided to start treatment with immunoglobulin for 6 cycles. During her evolution, clinical improvement was evident, without epileptic seizures, with better interaction with his environment and she began to walk with a walker. At the end of the anti-TB and immunoglobulin treatment, she presented an episode of dysarthria and facial asymmetry. The new image showed nodular lesions in the midbrain and cerebellum, some of which captured contrast. Therefore, the studies were expanded and an anti-MOG test was performed, which was positive. It was decided to start immunoglobulin cycles. With apparently good clinical evolution.</div></div><div><h3>Conclusion</h3><div>We present the case of an atypical initial presentation of MOGAD. This should be taken into account in cases where there is no adequate clinical improvement, since treatment with corticosteroids or immunoglobulins may be effective in these patients and prevent serious sequelae.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100237"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171843","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}

{"title":"Chronic levamisole-induced leukoencephalopathy: Uncommon presentation of two cases with adult-onset progressive symptoms","authors":"João Victor Cabral Correia Férrer,&nbsp;Lara Santos Machado,&nbsp;Luiz José Malta Gaia Ferreira Júnior,&nbsp;Luccas Almeida Andrade,&nbsp;Marianna Pinheiro Moraes de Moraes,&nbsp;José Luiz Pedroso,&nbsp;Orlando Graziani Povoas Barsottini","doi":"10.1016/j.nerep.2025.100247","DOIUrl":"10.1016/j.nerep.2025.100247","url":null,"abstract":"<div><div>Toxic leukoencephalopathies are abnormalities in the cerebral white matter associated with the use of legal or illegal substances. Levamisole-induced leukoencephalopathy (LILE) is a rare condition that typically presents with a subacute progression and has a clear temporal relationship to compound exposure. However, we describe two cases with a chronic progression of adult-onset progressive spastic-ataxia syndrome, likely due to recurrent exposure to levamisole. Both cases exhibited typical abnormalities on brain magnetic resonance imaging (MRI) and had normal cerebrospinal fluid (CSF) analysis. In both instances, clinical stability was closely associated with drug abstinence. These cases are distinct from the literature due to their chronic presentation, and LILE should always be considered as a differential diagnosis when toxic exposure is suspected.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100247"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171842","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}

{"title":"Erratum to “Atypical presentation of rare entity autoimmune autonomic ganglionopathy: Another potential cause of hypertensive crisis” [Neuroimmunology Reports Volume 2, 2022, 100062]","authors":"Baljinder Singh,&nbsp;Janani Ravichandran,&nbsp;Mohammad Rauf Chaudry,&nbsp;Simrandeep Kaur Brar,&nbsp;Isabel Narvaez,&nbsp;Salvador Cruz-Flores","doi":"10.1016/j.nerep.2024.100244","DOIUrl":"10.1016/j.nerep.2024.100244","url":null,"abstract":"","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100244"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171972","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}

{"title":"Exposure to satralizumab in AQP4-positive NMOSD during pregnancy: A case report and review of the literature","authors":"Ricardo Alonso","doi":"10.1016/j.nerep.2025.100248","DOIUrl":"10.1016/j.nerep.2025.100248","url":null,"abstract":"<div><div>Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disease of the central nervous system. It mainly affects young women and can lead to serious episodes involving the spinal cord and optic nerves. Treatments, may pose risks to the fetus, with limited safety data available. Satralizumab, is effective in reducing relapse rates but lacks sufficient safety data for use during pregnancy. The case of a 32-year-old woman with NMOSD who was treated with satralizumab during her first trimester is presented. This case provides preliminary evidence on satralizumab use in pregnancy, suggesting it might be a viable option with careful management.</div></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"7 ","pages":"Article 100248"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171844","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}