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Diaphragmatic pacing for neurogenic respiratory failure in neuromyelitis optica spectrum disorder: A case series 膈肌起搏治疗视神经脊髓炎谱系障碍的神经源性呼吸衰竭:一系列病例
Neuroimmunology Reports Pub Date : 2023-09-03 DOI: 10.1016/j.nerep.2023.100184
Hannah Kelly , Hesham Abboud , Alessandro Serra , Mary Jo Elmo , Raymond Onders
{"title":"Diaphragmatic pacing for neurogenic respiratory failure in neuromyelitis optica spectrum disorder: A case series","authors":"Hannah Kelly ,&nbsp;Hesham Abboud ,&nbsp;Alessandro Serra ,&nbsp;Mary Jo Elmo ,&nbsp;Raymond Onders","doi":"10.1016/j.nerep.2023.100184","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100184","url":null,"abstract":"<div><p>Diaphragmatic pacing (DP) has not been well-studied in adults with central respiratory failure secondary to inflammatory disorders of the brain and spinal cord. We present two novel cases of DP in the treatment of respiratory failure secondary to brainstem and high cervical spinal cord lesions in neuromyelitis optica spectrum disorder patients. In case 1, DP facilitated tracheostomy reversal and the patient was successfully weaned from DP. In case 2, DP helped improve chronic hypercapnia and the frequency of apneic events. Early initiation of DP should be considered in this patient population to treat central hypoventilation and central sleep apnea.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100184"},"PeriodicalIF":0.0,"publicationDate":"2023-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193932","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}
引用次数: 0
Treatment of recurrent VZV myelitis with intravenous immunoglobulin 静脉注射免疫球蛋白治疗复发性VZV脊髓炎
Neuroimmunology Reports Pub Date : 2023-08-29 DOI: 10.1016/j.nerep.2023.100181
Danelvis Paredes, Elijah Lackey, Christopher Eckstein
{"title":"Treatment of recurrent VZV myelitis with intravenous immunoglobulin","authors":"Danelvis Paredes,&nbsp;Elijah Lackey,&nbsp;Christopher Eckstein","doi":"10.1016/j.nerep.2023.100181","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100181","url":null,"abstract":"<div><h3>Background</h3><p>VZV myelitis is a rare and typically monophasic complication of VZV reactivation. There is no solid evidence for a particular treatment regimen for VZV myelitis, especially in recurrent disease. No prior reports or studies have looked at using intravenous immunoglobulin for this condition, particularly for refractory cases.</p></div><div><h3>Case report</h3><p>A 75-year-old female presented with paresthesia on the right lateral leg, followed by a vesicular rash in a T2 dermatomal distribution. Over a period of 2 weeks, she experienced bilateral lower extremity weakness. MRI revealed a C3-C6 enhancing lesion. VZV positivity was confirmed by skin biopsy. She was treated with 5 days of Solumedrol 1gm IV and valacyclovir 1gm TID with improvement of weakness and rash. However, over the next two years, she continued to get occasional disseminated vesicular rash with each flare of her myelitis despite continued valacyclovir. She was started on monthly IV Ig (1gm/kg IV Ig over 2 days) for her recurrent myelitis. Since starting monthly IV Ig there have been no further zoster outbreaks or episodes of myelitis. Her balance and gait have improved significantly.</p></div><div><h3>Conclusion</h3><p>We present an unusual case of recurrent disseminated zoster with myelitis successfully treated with monthly IV Ig. The successful treatment of this patient should prompt consideration for its use in similar cases of recurrent VZV myelitis and may provide insight for future studies on how to treat VZV-related diseases.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100181"},"PeriodicalIF":0.0,"publicationDate":"2023-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193894","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}
引用次数: 0
Anti-N-methyl-d-aspartate receptor encephalitis in a patient on long-term continuous immunomodulatory therapy 一例长期持续免疫调节治疗的抗N-甲基-d-天冬氨酸受体脑炎患者
Neuroimmunology Reports Pub Date : 2023-08-28 DOI: 10.1016/j.nerep.2023.100182
Lina M. Ariza-Serrano , Nicole Andrea Gómez Perdomo , Antonio Schlesinger , Manuel Tapias , Steven Hurtado
{"title":"Anti-N-methyl-d-aspartate receptor encephalitis in a patient on long-term continuous immunomodulatory therapy","authors":"Lina M. Ariza-Serrano ,&nbsp;Nicole Andrea Gómez Perdomo ,&nbsp;Antonio Schlesinger ,&nbsp;Manuel Tapias ,&nbsp;Steven Hurtado","doi":"10.1016/j.nerep.2023.100182","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100182","url":null,"abstract":"<div><h3>Background</h3><p>Anti-N-methyl-<span>d</span>-aspartate [anti-NMDA] receptor encephalitis is one of the most common types of immune-mediated encephalitis. It is characterized by a neuropsychiatric syndrome associated with immunoglobulin G [IgG]-type antibodies against the NMDA receptor's GluN1 subunit in cerebrospinal fluid [CSF]. To our Knowledge, only six cases of autoimmune encephalitis with positive CSF NMDA receptor IgG autoantibodies have been reported among patients on long-term immunosuppressive therapies. The etiologies of all six cases were related to solid organ transplantation. Therefore, this report is the first to present a case of immunosuppression associated with an etiology other than post-transplant immunosuppression.</p></div><div><h3>Case presentation</h3><p>We present the case of a 59-year-old female patient who was on a long-term continuous immunomodulatory therapy for rheumatoid arthritis, and she consulted the emergency department several times for headache, symptoms of neurologic deficit, and subsequent progression to neuropsychiatric symptoms. CSF examination of the patient confirmed the presence of antibodies against the NMDA receptor; hence, the diagnosis of anti-NMDA receptor encephalitis was made.</p></div><div><h3>Conclusions</h3><p>It is important to recognize the clinical features of autoimmune encephalitis and to consider this condition in the differential diagnosis when neuropsychiatric symptoms are manifested in such patients. Patients on long-term immunomodulatory therapy present with atypical manifestations of anti-NMDA receptor encephalitis. The consideration of these manifestations in the differential diagnosis can ensure efficient diagnosis, management, and reduction in the consequences affecting morbidity and mortality of these patients.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100182"},"PeriodicalIF":0.0,"publicationDate":"2023-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193887","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}
引用次数: 0
Severe motor sensory axonal polyneuropathy in a patient with eosinophilic granulomatosis with polyangiitis post COVID-19 vaccination: Case report 新冠肺炎疫苗接种后一例嗜酸性肉芽肿伴多血管炎患者的严重运动感觉轴突多发性神经病:病例报告
Neuroimmunology Reports Pub Date : 2023-08-19 DOI: 10.1016/j.nerep.2023.100180
Joyce Chen , Cameron T. Landers , Chunyu Cai , Gregory S Carter
{"title":"Severe motor sensory axonal polyneuropathy in a patient with eosinophilic granulomatosis with polyangiitis post COVID-19 vaccination: Case report","authors":"Joyce Chen ,&nbsp;Cameron T. Landers ,&nbsp;Chunyu Cai ,&nbsp;Gregory S Carter","doi":"10.1016/j.nerep.2023.100180","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100180","url":null,"abstract":"","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100180"},"PeriodicalIF":0.0,"publicationDate":"2023-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193929","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}
引用次数: 0
Acute pontine tumefactive multiple sclerosis 急性桥脑肿胀性多发性硬化
Neuroimmunology Reports Pub Date : 2023-08-11 DOI: 10.1016/j.nerep.2023.100179
Yalda Nikanpour , M. Toledano , W. Oliver Tobin
{"title":"Acute pontine tumefactive multiple sclerosis","authors":"Yalda Nikanpour ,&nbsp;M. Toledano ,&nbsp;W. Oliver Tobin","doi":"10.1016/j.nerep.2023.100179","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100179","url":null,"abstract":"<div><p>A 20-year-old woman presented with ataxia, right-sided hemiparesis, headache, and fever. Brain MRI showed a large non enhancing pontine T2 hyperintense lesion with slight diffusion restriction. Her clinical condition progressed to quadriparesis, dysarthria and ophthalmoplegia. The patient was diagnosed with tumefactive multiple sclerosis. She had a positive response to plasma exchange and cyclophosphamide, and ultimately returned to running at 1 year follow up. De novo tumefactive demyelination, diagnosis, and indications for aggressive treatment are discussed.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100179"},"PeriodicalIF":0.0,"publicationDate":"2023-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193888","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}
引用次数: 0
Relapsing Susac syndrome presenting with encephalopathy and refractory hiccups 以脑病和顽固性呃逆为特征的复发性Susac综合征
Neuroimmunology Reports Pub Date : 2023-07-19 DOI: 10.1016/j.nerep.2023.100178
Reza Vosoughi , Nupura Bakshi , Prem A.H Nichani , James J Marriott , Elaheh Shahmiri
{"title":"Relapsing Susac syndrome presenting with encephalopathy and refractory hiccups","authors":"Reza Vosoughi ,&nbsp;Nupura Bakshi ,&nbsp;Prem A.H Nichani ,&nbsp;James J Marriott ,&nbsp;Elaheh Shahmiri","doi":"10.1016/j.nerep.2023.100178","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100178","url":null,"abstract":"<div><p>Susac syndrome is a rare autoimmune endotheliopathy characterized by occlusion of small arteries in brain, retina, and cochlea presenting with triad of encephalopathy, retinal vaso-occlusive disease and hearing loss. The disease can be monophasic or relapsing with variety of symptoms and the majority of the patients do not have the classic triad, especially at the initial clinical presentation. In CNS neuroinflammaory disorders, refractory hiccup is a recognized manifestation of NMOSD area postrema syndrome. To our knowledge, this is the first report of Susac syndrome presenting with encephalopathy and refractory hiccup.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100178"},"PeriodicalIF":0.0,"publicationDate":"2023-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193893","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}
引用次数: 0
Management of Baló Concentric Sclerosis with rituximab: A case study with long-term follow-up 利妥昔单抗治疗Baló同心性硬化症:一项长期随访的病例研究
Neuroimmunology Reports Pub Date : 2023-07-18 DOI: 10.1016/j.nerep.2023.100177
Lina Okar , Beatriz Canibano , Dirk Deleu
{"title":"Management of Baló Concentric Sclerosis with rituximab: A case study with long-term follow-up","authors":"Lina Okar ,&nbsp;Beatriz Canibano ,&nbsp;Dirk Deleu","doi":"10.1016/j.nerep.2023.100177","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100177","url":null,"abstract":"<div><h3>Background</h3><p>: Baló concentric sclerosis (BCS) is a rare demyelinating disorder that overlaps with other demyelinating diseases. BCS usually present in the context of multiple sclerosis (MS) or preceding typical MS. It rarely presents as an isolated lesion, . IV methyl prednisolone (IVMP) is the mainstay in the treatment with various outcomes. Maintenance therapy is still not clearly defined.</p><p>Case presentation/case report: We present a case of isolated BCS that responded clinically and radiologically to long-term rituximab therapy.</p></div><div><h3>Conclusion</h3><p>: A definite guideline for treating patient with BCS either with MS or as isolated entity is still controversial. Our case reflects a remarkable response to rituximab in achieving clinical and radiological long-term remission.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100177"},"PeriodicalIF":0.0,"publicationDate":"2023-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193892","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}
引用次数: 0
Rhombencephalitis in a patient with VEXAS syndrome VEXAS综合征患者的朗伯脑炎
Neuroimmunology Reports Pub Date : 2023-07-07 DOI: 10.1016/j.nerep.2023.100176
Magnus Johnsson
{"title":"Rhombencephalitis in a patient with VEXAS syndrome","authors":"Magnus Johnsson","doi":"10.1016/j.nerep.2023.100176","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100176","url":null,"abstract":"<div><h3>Background</h3><p>VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) is a recently described rare novel autoinflammatory syndrome characterized by systemic inflammatory and hematological symptoms. Based on reported cases thus far, central nervous system involvement is exceptionally infrequent.</p></div><div><h3>Case report</h3><p>A 77-year-old man had a five-year medical history of chronic fluctuating fever, weight loss, skin lesions, and dyshematopoiesis. He presented with acute headache, vomiting, and generalized weakness, and in the following days, he also developed dysarthria, diplopia, and rapidly decreasing consciousness. Investigations showed brainstem encephalitis with no evidence of concurrent infection. Despite treatment with high-dose Solu-Medrol, the patient died 20 days after hospitalization. Genetic screening confirmed the diagnosis of VEXAS syndrome.</p></div><div><h3>Conclusion</h3><p>This case presents a rare case of rhombencephalitis in a patient with VEXAS syndrome. There is a wide array of etiologies in rhombencephalitis, and clinicians may need to consider VEXAS syndrome in the diagnostic work-up if there is a history of systemic inflammatory symptoms.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"4 ","pages":"Article 100176"},"PeriodicalIF":0.0,"publicationDate":"2023-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50193931","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}
引用次数: 0
DPPX antibody-associated encephalitis: A short report on a Chinese patient DPPX抗体相关性脑炎1例报告
Neuroimmunology Reports Pub Date : 2023-01-01 DOI: 10.1016/j.nerep.2023.100171
Yalan Wan , Siming Xian , Xutong Zhao , Xujun Chu , Yun Yuan , Zhaoxia Wang , Hongjun Hao , Feng Gao , Haiqiang Jin
{"title":"DPPX antibody-associated encephalitis: A short report on a Chinese patient","authors":"Yalan Wan ,&nbsp;Siming Xian ,&nbsp;Xutong Zhao ,&nbsp;Xujun Chu ,&nbsp;Yun Yuan ,&nbsp;Zhaoxia Wang ,&nbsp;Hongjun Hao ,&nbsp;Feng Gao ,&nbsp;Haiqiang Jin","doi":"10.1016/j.nerep.2023.100171","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100171","url":null,"abstract":"<div><h3>Background</h3><p>DPPX encephalitis, an ancillary, regulatory protein of the Kv4.2 potassium channel Dipeptidyl-peptidase-like protein-6, is caused by cell surface autoantigens to DPPX, and is considered a rare but treatable autoimmune disease. Patients generally start with gastrointestinal symptoms (diarrhea and abdominal pain) or weight loss, and then precede the central nervous system (CNS) hyperexcitability with or without cognitive dysfunction. Here we reported a DPPX-associated patient, whose titer of DPPX antibody was inconsistent with clinical symptoms.</p></div><div><h3>Case presentation</h3><p>This study presents a case exhibiting mild symptoms of DPPX encephalitis. A 30-year-old male suffered from mild memory loss after a fever, which could be easily misdiagnosed as influenza or other common diseases. Immunological examination based on cell-based assay (CBA) detected DPPX antibody in both cerebrospinal fluid and serum. All the symptoms recovered after the administration of immunotherapy within 3 weeks. The DPPX titer in serum, however, became higher at the same time. Six months later, the re-examination of the cerebrospinal fluid immunological test was negative for the DPPX antibody.</p></div><div><h3>Conclusions</h3><p>In view of the symptoms of DPPX-associated encephalitis and the fact that changes in antibody titers in CSF are more likely to reflect symptomatic changes, we suggest that clinicians actively perform lumbar puncture in suspected patients to avoid misdiagnosis or misjudgment of the disease.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100171"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191177","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}
引用次数: 0
Early bilateral splenium lesions with subsequent progression in Neuromyelitis Optica patients Optica型神经脊髓炎患者早期双侧脾脏病变及随后的进展
Neuroimmunology Reports Pub Date : 2023-01-01 DOI: 10.1016/j.nerep.2023.100169
Majed Mohammedali Alluqmani , Wael Gabr , Rabiaa Douma
{"title":"Early bilateral splenium lesions with subsequent progression in Neuromyelitis Optica patients","authors":"Majed Mohammedali Alluqmani ,&nbsp;Wael Gabr ,&nbsp;Rabiaa Douma","doi":"10.1016/j.nerep.2023.100169","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100169","url":null,"abstract":"<div><h3>Background</h3><p>Neuromyelitis Optica (NMO) is a central nervous system (CNS) demyelinating disease. However, very little is known about callosal lesions in NMO. In addition, extensive splenic involvement as the first radiological finding is unusual and poorly described.</p></div><div><h3>Case Presentation and Report</h3><p>A female in her 30′s and a male in his 50′s were presented with lower limb weakness and blurry vision. MRI of the brain revealed focal lesions in the lateral splenium of the corpus callosum. However, over the next two months, symptoms become worsened. Repeated brain MRI showed a further progression of the splenium lesion. Plasma exchange resulted in clinical improvement and rituximab was used to maintain the clinical stability.</p></div><div><h3>Conclusion</h3><p>Extensive splenium lesion suggests NMO rather than MS, and this pattern can take up to two months to establish. This pattern of splenium involvement can be preceded by bilateral lateral splenium involvement in the early phase. Furthermore, in the acute stage, these patients require aggressive and prompt treatment with plasma exchange rather than steroid to prevent further deterioration.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100169"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191181","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}
引用次数: 0
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