{"title":"Very late-onset neuromyelitis optica presenting with simultaneous bilateral optic neuritis and myelitis: A case report","authors":"S Gandelman , S Parauda , C Dohle","doi":"10.1016/j.nerep.2024.100227","DOIUrl":"10.1016/j.nerep.2024.100227","url":null,"abstract":"<div><h3>Background</h3><p>New-onset Neuromyelitis Optica Spectrum Disorder (NMOSD) has rarely been reported in adults over age 70. We describe a severe presentation in a 77-year-old female.</p></div><div><h3>Case</h3><p>The patient presented with left arm numbness, followed by left leg numbness and bilateral optic neuritis. Neuroimaging showed a C2-T1 lesion with C4-C7 holocord enhancement, and bilateral optic nerve enhancement extending to the optic chiasm. Serum testing detected anti-aquaporin-4 antibodies. She was left with dyschromatopsia, myelopathy, and ambulatory dysfunction. She had a history notable for rheumatoid arthritis, treated with rituximab, which had been discontinued six years prior to presentation.</p></div><div><h3>Discussion</h3><p>Very-late-onset NMOSD encompasses a unique cohort of patients with significant immunogenicity despite immunosenescence. Limited data suggest diagnostic delays, severe morbidity, and decreased utilization of disease-modifying therapies.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100227"},"PeriodicalIF":0.0,"publicationDate":"2024-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000287/pdfft?md5=911d5b73fdd8fbd05a4f3ee1c3b475a6&pid=1-s2.0-S2667257X24000287-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142241537","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}
Krithika Arrabothu , Srinivas Govindan , Wilson Rodriguez , Christopher Tapia , Robert L. White III
{"title":"A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma","authors":"Krithika Arrabothu , Srinivas Govindan , Wilson Rodriguez , Christopher Tapia , Robert L. White III","doi":"10.1016/j.nerep.2024.100226","DOIUrl":"10.1016/j.nerep.2024.100226","url":null,"abstract":"<div><p>Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder characterized by subacute neuropsychiatric symptom development and rapid remission with corticosteroid therapy. In this paper, we describe a case of GFAP encephalitis found to have renal cell carcinoma. The patient was a 71-year-old man with a history of transient ischemic attacks, severe diabetic retinopathy and monoclonal gammopathy of undetermined significance, who presented with subacute cognitive decline. Anti-GFAP antibodies were detected in the cerebrospinal fluid. Computer Tomography (CT) of chest, abdomen and pelvis identified a renal mass that was resected. Subsequent pathology confirmed clear cell renal cell carcinoma. The patient was treated with corticosteroids with cognitive recovery near to baseline. In conclusion, we encourage the early consideration of autoimmune GFAP astrocytopathy for patients with clinical manifestation of subacute neuropsychiatric symptoms after other common conditions have been ruled out, as it usually has a great response to immunosuppressive therapy. In addition, this case highlights the importance of full body scan to identify possible underlying malignancy as a possible precipitating factor.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100226"},"PeriodicalIF":0.0,"publicationDate":"2024-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000275/pdfft?md5=2065be24e880ef5864893cd0798d0453&pid=1-s2.0-S2667257X24000275-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142241536","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}
Bhanu B Gowda , Snehal Shah , Rahul Lakshmanan , Jonathan Silberstein
{"title":"Hypertrophic polyneuropathy in CMV related GBS in a paediatric patient","authors":"Bhanu B Gowda , Snehal Shah , Rahul Lakshmanan , Jonathan Silberstein","doi":"10.1016/j.nerep.2024.100225","DOIUrl":"10.1016/j.nerep.2024.100225","url":null,"abstract":"","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100225"},"PeriodicalIF":0.0,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000263/pdfft?md5=40986e4a76b366f1461794031233cb24&pid=1-s2.0-S2667257X24000263-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142097485","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":"Resolution of optic neuritis and probable multiple sclerosis after long-term ingestion of very high doses of vitamin D3: A case report","authors":"Nipith Charoenngam , Michael F. Holick","doi":"10.1016/j.nerep.2024.100224","DOIUrl":"10.1016/j.nerep.2024.100224","url":null,"abstract":"<div><p>A 36-year-old male presented with 2 months of left-eye visual disturbance and was diagnosed with optic neuritis due to probable multiple sclerosis (MS). He was advised to undergo periodic ophthalmology follow-up without immunosuppressive treatment. Due to persistent symptoms, he expressed interest in very high-dose vitamin D<sub>3</sub> therapy of 54,000 IUs/day (1,000 IUs/kg/day) along with a zero-calcium diet. After starting the therapy, he experienced sustained symptomatic improvement of visual symptoms over 4 years, along with radiological stability of the optic neuritis lesion without developing hypercalcemia. This case supports the potential therapeutic efficacy of very high-dose vitamin D for MS.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100224"},"PeriodicalIF":0.0,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000251/pdfft?md5=7b1df6ff327984b259e7f1d7ec65313f&pid=1-s2.0-S2667257X24000251-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141952470","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}
Antonella Frattari , Maria Vittoria De Angelis , Mariangela Battilana , Ennio Polilli , Alessandro Ferrieri , Daniela Onofrillo , Nicole Santoro , Antonella Sau , Anna D'Andreagiovanni , Pierluigi Tocco , Donatella Bosco , Giustino Parruti
{"title":"High viral load VZV encephalitis complicated by super refractory status epilepticus in a vaccinated child with in Acute Lymphocytic Leukemia: Case report and review of the literature","authors":"Antonella Frattari , Maria Vittoria De Angelis , Mariangela Battilana , Ennio Polilli , Alessandro Ferrieri , Daniela Onofrillo , Nicole Santoro , Antonella Sau , Anna D'Andreagiovanni , Pierluigi Tocco , Donatella Bosco , Giustino Parruti","doi":"10.1016/j.nerep.2024.100223","DOIUrl":"10.1016/j.nerep.2024.100223","url":null,"abstract":"<div><p>Varicella is a benign, self-limiting disease, but both primary VZV infection and VZV reactivation can be life-threatening in immunocompromised children, due to CNS and systemic dissemination of the virus. Reactivation of the vaccine-type VZV (vOka) has been reported sporadically, although SARS COV2 infection may have recently played a role in facilitating VZV reactivation. Here we report on the case of a young boy with Acute Lymphocytic Leukemia, in remission after induction chemotherapy, and recent SARS COV 2 infection. He developed VZV encephalitis due to massive reactivation of VZV virus, complicated by PRES, diagnosed with brain MRI, and Super Refractory Status Epilepticus, lasting until substantial suppression of VZV replication in the CNS. We also report and discuss the role of the remarkably augmented renal clearance persistently observed in our patient, complicating CNS involvement and making both antiviral and antiepileptic treatments more difficult to manage. Effective neuroprotection was completed by physical hypothermia and infusion of IVIG and steroids. The patient obtained complete functional recovery, with reversion of MRI signs of occipital involvement at presentation, at 6-month follow-up. Intense and daily interplay of intensivists, neurologists, hematologists and infectious disease experts likely made his uneventful recovery possible, combining all skills necessary to manage his unusual and complex clinical picture.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100223"},"PeriodicalIF":0.0,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X2400024X/pdfft?md5=78f3514e7bb97f38fd7091c4f3ca5aaf&pid=1-s2.0-S2667257X2400024X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141952469","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":"Pediatric Multiple Sclerosis: Unveiling the Trajectory of a Toddler-Onset Case","authors":"Rufaidah Rushdi , Nevin Shalaby","doi":"10.1016/j.nerep.2024.100222","DOIUrl":"10.1016/j.nerep.2024.100222","url":null,"abstract":"<div><p>Pediatric multiple sclerosis (MS) is predominantly a relapsing-remitting demyelinating disorder. Toddler-onset MS (12–36 months) is rarely studied despite the likelihood of early-life adversity influencing its incidence. In this case, we present a 9-year-6-month-old girl who first exhibited symptoms at 30 months. Her disease course reflects the challenging mission of reaching a definitive MS diagnosis. Noteworthy observations include a complex perinatal history, initially normal diagnostic workup, and complete recovery after each relapse. It is crucial to recognize the hidden dynamics of MS, namely smoldering MS and the related progression independent of relapse activity, to commence a high-efficacy early treatment modality and establish a comprehensive multidisciplinary management strategy.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100222"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000238/pdfft?md5=c53ddea9b608eb80571a67a5888cbe36&pid=1-s2.0-S2667257X24000238-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141710339","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}
Samantha Anne Gutierrez , Alex Vu , Napo Kasirye-Mbugua , Jodie Burton
{"title":"FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) presenting with psychosis and refractory seizures","authors":"Samantha Anne Gutierrez , Alex Vu , Napo Kasirye-Mbugua , Jodie Burton","doi":"10.1016/j.nerep.2024.100221","DOIUrl":"10.1016/j.nerep.2024.100221","url":null,"abstract":"<div><p>Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is an idiopathic inflammatory demyelinating disease of the central nervous system commonly associated with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis and cortical encephalitis. A somewhat rare and less recognized syndrome in MOGAD has been identified, and is characterized by cortical encephalitis and seizures with cortical FLAIR-hyperintense lesions on the MRI aptly termed FLAMES (<strong>F</strong>LAIR-hyperintense <strong>L</strong>esions in <strong>A</strong>nti-<strong>M</strong>OG-associated <strong>E</strong>ncephalitis with <strong>S</strong>eizures). We report a previously healthy middle-aged man who presented with focal seizures and status epilepticus refractory to initial interventions. He later developed headache, mood and behavioral changes and ultimately tested positive for anti-MOG antibody in the serum using a fixed cell-based assay. When his MRI was re-assessed in retrospect, it was determined to be in keeping with FLAMES.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100221"},"PeriodicalIF":0.0,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000226/pdfft?md5=6860536818566cd5bfe9f7aa2d79514c&pid=1-s2.0-S2667257X24000226-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629999","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}
Zohreh Abna , Seyed Amirhossein Fazeli , Ziba Khanmoradi , Mohammad Ali Sahraian
{"title":"Neuromyelitis Optica Spectrum Disorders (NMOSD) and structural renal diseases: A literature review","authors":"Zohreh Abna , Seyed Amirhossein Fazeli , Ziba Khanmoradi , Mohammad Ali Sahraian","doi":"10.1016/j.nerep.2024.100220","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100220","url":null,"abstract":"<div><p>Neuromyelitis Optica Spectrum Disorders (NMOSD) is an immune-mediated disease of the central nervous system that often leads to severe attacks of optic nerves and spinal cord. The discovery of Aquaporine 4 (AQP4) antibody as a potential biomarker of NMOSD has changed the global approach to this disease. There have been associations between NMOSD and systemic autoimmune diseases, both organ specific and non-organ specific. Here, we aimed to review the literature on co-incidence or possible association of kidney diseases with NMOSD.</p></div><div><h3>Methods</h3><p>we included All English relevant publications (reviews, original articles, case reports, case series and abstracts) from 1998 to 2023 using PubMed and google scholar database for this review.</p></div><div><h3>Results</h3><p>Nine case reports of co-existing NMOSD with renal disorders were evaluated. The patients were involved from 10–90 years old. The most common association was with nephrotic syndrome and in the context of systemic autoimmune disorders. However a case of NMOSD coincident with renal cell carcinoma without paraneoplastic etiology was reported.</p></div><div><h3>Conclusion</h3><p>Despite the expression of AQP4 in the collecting duct cells of kidney, whether AQP4-IgG, the pathogenic antibody in NMOSD, can damage to the kidney is still unknown.</p><p>Can structural kidney damages lead to AQP4 antigen being exposed to immune system and triggers the cascade of autoimmunity is a question that should be investigated in future studies.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100220"},"PeriodicalIF":0.0,"publicationDate":"2024-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000214/pdfft?md5=e281dd2eb65a975e216c4e913f51525a&pid=1-s2.0-S2667257X24000214-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141595000","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}
Cesar D. Bautista-Sanchez , Luis E. Martínez-Bravo , Diego A. Hidalgo-Díaz
{"title":"Pancytopenia in neuromyelitis optica spectrum disorder induced by azathioprine-allopurinol interaction","authors":"Cesar D. Bautista-Sanchez , Luis E. Martínez-Bravo , Diego A. Hidalgo-Díaz","doi":"10.1016/j.nerep.2024.100219","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100219","url":null,"abstract":"<div><h3>Background</h3><p>Azathioprine (AZA) remains a widely used treatment for neuromyelitis optica spectrum disorder (NMOSD). It heightens the risk of myelosuppression, a risk further intensified with the addition of allopurinol.</p></div><div><h3>Case presentation</h3><p>To the best of our knowledge, there have been no reported cases of this complication in NMOSD.</p></div><div><h3>Case report</h3><p>We present a 63-year-old female with NMOSD on AZA, who experienced severe myelosuppression upon initiating allopurinol. Following the discontinuation of these drugs, we prescribed granulocyte stimulants, recombinant erythropoietin, and supportive care, which resulted in hematologic improvement.</p></div><div><h3>Conclusions</h3><p>This case highlights a potential myelosuppression risk when combining AZA and allopurinol in NMOSD patients.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100219"},"PeriodicalIF":0.0,"publicationDate":"2024-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000202/pdfft?md5=61c35b54f2390ba44144e23ca8c6129b&pid=1-s2.0-S2667257X24000202-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141481654","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}
Feng Xiang, Xu Zhang, Rongfei Wang, Xiangqing Wang
{"title":"Case report: Neuromyelitis optica spectrum disorder in a patient presented with orbital apex syndrome","authors":"Feng Xiang, Xu Zhang, Rongfei Wang, Xiangqing Wang","doi":"10.1016/j.nerep.2024.100216","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100216","url":null,"abstract":"<div><h3>Background</h3><p>Orbital apex syndrome as the presentation of neuromyelitis optica spectrum disorder has not been reported in literature.</p></div><div><h3>Case presentation</h3><p>We reported a case in which the patient initially presented with ptosis, diplopia, ophthalmoplegia and visual impairment of the right eye. Clinical examination revealed no light perception in the right eye with the presence of relative afferent pupillary defect and involvement of cranial nerves II, III, V, and VI. Magnetic resonance imaging of the cranial and orbital regions revealed thickening in the intraorbital segment of the right optic nerve with T2-weighted hyperintensity, and Gadolinium-enhancement of the right optic nerve and its myelin sheath. Longitudinally extensive transverse myelitis subsequently developed, with detection of serum anti-aquaporin-4 antibody. Intravenous methylprednisolone was initiated, leading to significant clinical improvement.</p></div><div><h3>Conclusion</h3><p>This case report highlights the diverse manifestations of neuromyelitis optica spectrum disorder, including the orbital apex syndrome.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100216"},"PeriodicalIF":0.0,"publicationDate":"2024-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000172/pdfft?md5=7d2932e7bbd9739ff7dbc452293d28ce&pid=1-s2.0-S2667257X24000172-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141481655","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}