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}
{"title":"Severe presentation of myelin oligodendrocyte glycoprotein antibody-associated disease: A case report","authors":"Dominique Comeau , Olivia Cull , Yanis Saheb , Remi Leblanc , Ludivine Chamard-Witkowski","doi":"10.1016/j.nerep.2024.100217","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100217","url":null,"abstract":"<div><h3>Background</h3><p>Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system. Relapse may be moderate to severe with an Expanded Disability Status Scale (EDSS) above 4.0 in half of patients, albeit most experience good to excellent motor recovery.</p></div><div><h3>Results</h3><p>Herein, we present an atypically severe case of MOGAD with an unusual clinical course. Patient initially presented with diplopia, lower limb motor deficit and hypoesthesia which rapidly deteriorated into quadriplegia. Corticosteroid regimen did not initially translate to significant clinical improvement (EDSS=9.0). At the 1 year follow up, patient had regained some mobility although with marked sequela (EDSS=6.5).</p></div><div><h3>Conclusion</h3><p>Although MOGAD is generally thought to be benign, it could also present with severe episodes.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"6 ","pages":"Article 100217"},"PeriodicalIF":0.0,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000184/pdfft?md5=06ef7b6b09aee0cdcbb316f6cf60bb56&pid=1-s2.0-S2667257X24000184-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141332976","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}
Mohammad Amin Sadri , Sarina Loghmani , Zahra Nikoo , Farinaz Tabibian , Vahid Shaygannejad
{"title":"Susac syndrome with complete triad and concurrent kidney failure treated with plasmapheresis and rituximab: A case report","authors":"Mohammad Amin Sadri , Sarina Loghmani , Zahra Nikoo , Farinaz Tabibian , Vahid Shaygannejad","doi":"10.1016/j.nerep.2024.100210","DOIUrl":"10.1016/j.nerep.2024.100210","url":null,"abstract":"<div><h3>Introduction</h3><p>Susac syndrome (SuS) is a rare autoimmune disease characterized by the clinical triad of brain dysfunction, branch retinal artery occlusion, and hearing loss. Many cases have been reported with irreversible sequelae due to misdiagnosis and late treatment of the disease.</p></div><div><h3>Case</h3><p>In this article, we describe the clinical course and diagnosis of SuS in a young woman with a history of type 1 diabetes mellitus and kidney transplantation who presented with the complete triad. The complex medical history required a tailored treatment approach, including plasmapheresis and rituximab, which significantly improved her condition.</p></div><div><h3>Conclusion</h3><p>This report highlights the diagnostic complexity of SuS and the value of personalized treatment strategies, contributing to the understanding of this rare disorder, and briefly reviews the current knowledge of the disease and discusses the probable pathophysiological relationship between SuS and kidney failure.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100210"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000111/pdfft?md5=13d1bb55b1b04759af609450efe4a57e&pid=1-s2.0-S2667257X24000111-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140757340","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":"Progressive multifocal leukoencephalopathy successfully treated with intravenous immunoglobulin","authors":"Swansu Batra, Ruchika Tandon, Saurabh Nigam","doi":"10.1016/j.nerep.2024.100214","DOIUrl":"https://doi.org/10.1016/j.nerep.2024.100214","url":null,"abstract":"<div><p>A 52-year-old lady developed apathy, reduced verbal output, decreased visuospatial orientation, cognitive decline, visual impairment and left hemiparesis for 4 months. On MRI head, she displayed T2/FLAIR asymmetric white matter hyperintensities in parietal and occipital lobes and cerebellar peduncle and her CSF PCR was positive for John Cunningham (JC) virus and HIV ELISA and other bacterial, mycobacterial and viral markers were negative, confirming Progressive multifocal leukoencephalopathy (PML). Thereafter, we administered monthly intravenous immunoglobulin (IVIg) and patient's condition and neuroimaging has been improving for past 8 months. Hence, IVIg improving the condition of a potentially fatal disease patient, offers some hope.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100214"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X24000159/pdfft?md5=bf17e38c5cb88fa4347a2b32e7aff696&pid=1-s2.0-S2667257X24000159-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140948779","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":"Two cases of nasopharyngeal negative COVID-19 infections in patients with demyelinating diseases on anti-CD20 treatments","authors":"Alexandra Balshi, Jacob A Sloane","doi":"10.1016/j.nerep.2023.100197","DOIUrl":"10.1016/j.nerep.2023.100197","url":null,"abstract":"<div><p>We report two cases of nasopharyngeal negative COVID-19 infections in patients with demyelinating diseases on anti-CD20 treatments. Despite negative nasopharyngeal polymerase chain reaction (PCR) tests, both patients exhibited radiological features consistent with SARS-CoV-2 infection, with one confirmed in bronchoalveolar lavage fluid. Immunocompromised patients may have limited immune responses, possibly contributing to persistent COVID-19 infections despite vaccination history. These findings underscore the need for increased vigilance and consideration of lower respiratory tract infections in immunocompromised patients on anti-CD20 therapies.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"5 ","pages":"Article 100197"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2667257X23000359/pdfft?md5=fcae974d72a548531ac7e57592fa75a4&pid=1-s2.0-S2667257X23000359-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138988574","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}