Neuroimmunology Reports最新文献

{"title":"Total right visual loss due to Temporal Arteritis recognised only after left visual deterioration, necessitating maximal steroid therapy and tocilizumab","authors":"Danny Lam ,&nbsp;Log Tung Lai ,&nbsp;Ashish Agar ,&nbsp;Tim Y-T. Lu ,&nbsp;Minas T. Coroneo ,&nbsp;Ian C. Francis","doi":"10.1016/j.nerep.2022.100159","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100159","url":null,"abstract":"<div><h3>Background</h3><p>Temporal Arteritis is the most common panarteritis in adults and typically affects elderly people, particularly women. This occlusive vasculitis is associated with at least 51 symptoms and signs and can result in bilateral blindness and potentially death from aortic root dilatation and rupture.</p></div><div><h3>Case Report</h3><p>This report demonstrates a case of Temporal Arteritis with bilateral, rapidly progressive arteritic anterior ischaemic optic neuropathy despite recommended high dose oral steroid therapy, with right eye blindness only recognised after left visual loss developed. The addition of tocilizumab was required for resolution of the symptoms.</p></div><div><h3>Conclusions</h3><p>It is essential to examine thoroughly all the parameters of visual function in a patient who has lost vision. Such an examination, especially in patients with TA, consists as a minimum of visual acuity, pupil reactions, confrontation fields and fundi in both eyes in every patient.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100159"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191182","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":"Multiple sclerosis presenting with progressive visual impairment independent of relapse activity: A case report","authors":"Shun Akaike ,&nbsp;Tomoko Okamoto ,&nbsp;Ariko Miyazawa ,&nbsp;Yuji Takahashi","doi":"10.1016/j.nerep.2023.100172","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100172","url":null,"abstract":"<div><h3>Introduction</h3><p>Visual impairment due to optic neuritis is one of the most common manifestations of multiple sclerosis (MS); however progressive visual loss without relapse is rare. Furthermore, the association of progression independent of relapse activity (PIRA) with visual impairment has not been largely explored. We report a rare case of MS associated with progressive visual loss, independent of relapse, with detailed follow-up data of visual acuity, critical flicker fusion frequency (CFF), and visual-evoked potential (VEP), which provided supporting evidence for monitoring visual function.</p></div><div><h3>Case report</h3><p>A 44-year-old Japanese woman with MS presented with decreased visual acuity in both eyes. Her symptoms progressed slowly without relapse and did not respond to intravenous methylprednisolone or plasma exchange. Her CFF and VEP also showed gradual exacerbation. Magnetic resonance imaging revealed no new lesions of MS. Visual impairment was correlated with physical worsening, suggesting progression independent of relapse. After ofatumumab was initiated, her visual acuity mildly improved.</p></div><div><h3>Conclusion</h3><p>We describe a patient with visual impairment that was considered the PIRA. Impairment of visual function, in addition to impaired walking ability and cognitive function, should be considered when assessing disability progression in MS. CFF and P100 latency of VEP may aid in the early diagnosis of PIRA, potentially leading to timely treatment.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100172"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191183","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":"Extensive rebound MS activity following dimethyl fumarate discontinuation in a 63 year old – A case report","authors":"Laura Ghezzi ,&nbsp;Victoria A. Levasseur ,&nbsp;Dana C. Perantie ,&nbsp;Gregory F. Wu ,&nbsp;Anne H. Cross","doi":"10.1016/j.nerep.2023.100175","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100175","url":null,"abstract":"<div><h3>Background</h3><p>Dimethyl fumarate (DMF) is an oral disease modifying therapy (DMT) approved for the treatment of relapsing-remitting multiple sclerosis (RRMS). DMF is effective at reducing relapses, and decreasing disease activity. Reports of rebound activity following DMF discontinuation are rare.</p></div><div><h3>Case report</h3><p>We report the case of a 63-year old woman with RRMS, showing an extensive radiological and clinical rebound after DMF discontinuation. She started DMF in 2014 at which time her EDSS was 1.5. She was clinically stable until 2019, when she noted the onset of progressive left leg weakness but without signs of radiological activity on brain MRI. In 2020, she experienced further worsening and MRI showed one new brain lesion. She was treated with oral steroids with partial recovery. She self-discontinued DMF in February 2021. Approximately 5 months after discontinuation she developed severe left side weakness and her brain MRI showed 34 enhancing lesions. She was treated with another course of oral steroids with clinical benefit and her DMT was switched to oral cladibrine.</p></div><div><h3>Conclusions</h3><p>This case is unusual because MS disease rebound is uncommon after DMF discontinuation and because disease activity of this magnitude with over 30 gadolinium enhancing lesions is rare in people with MS over the age of 60. Our case stresses that a reactivation of MS activity including a large number of enhancing lesions can occur in older individuals, despite a presumably senescent immune system. Monitoring for disease activity before and after discontinuing a DMT is important, regardless of the patient's age.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100175"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191185","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":"A case of MOGAD optic neuritis initially mis-classified as CLIPPERS","authors":"Ethan Zerpa ,&nbsp;Stan C Kunigelis ,&nbsp;Stacy V Smith","doi":"10.1016/j.nerep.2022.100160","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100160","url":null,"abstract":"<div><h3>Background</h3><p>MOG antibody disease presents along a spectrum that includes acute disseminated encephalomyelitis, transverse myelitis, and optic neuritis. CLIPPERS is a rare condition that may complicate an accurate MOGAD diagnosis. This is in part due to overlapping clinical and imaging features with MOGAD.</p></div><div><h3>Case report</h3><p>Here we report a case of a 63-year-old woman with relapsing optic neuritis due to MOGAD that was initially concerning for CLIPPERS. The patient was seropositive for MOG-ab and responded well to high dose corticosteroid therapy which was tapered over 9-months.</p></div><div><h3>Conclusion</h3><p>This case underscores the importance of recognizing the overlap in clinical presentation that may occur between MOGAD and CLIPPERS despite both conditions having distinct biological origins. CLIPPERS criteria and the exclusion of alternative causes can help distinguish between the two. A MOG-ab titer should be used to screen MOGAD as a CLIPPERS mimicker. Antibody testing, clinical imaging, steroid responsiveness, history of present illness, and the extent of existing disability may provide a complete diagnostic picture.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100160"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191175","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":"Autoantibody-negative but probable pediatric autoimmune encephalitis following COVID-19 vaccination: A case report","authors":"Masato Ando,&nbsp;Kunihiro Matsunami,&nbsp;Sotaro Yuzawa,&nbsp;Tatsuya Sakashita,&nbsp;Hiroaki Murakami,&nbsp;Shuji Kuwabara,&nbsp;Eiji Matsukuma,&nbsp;Atsushi Imamura","doi":"10.1016/j.nerep.2023.100173","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100173","url":null,"abstract":"<div><h3>Background</h3><p>Large-scale vaccination against the novel coronavirus (COVID-19) occurred globally at an unprecedented pace. Sporadic cases of autoimmune encephalitis (AE) have been reported following COVID-19 vaccination, mainly in adults.</p></div><div><h3>Case report</h3><p>A 14-year-old girl developed altered mental status and was brought to our emergency department because of a seizure 19 days after receiving the third dose of COVID-19 vaccination. She was treated with steroid pulse therapy and fully recovered. The diagnosis of probable autoantibody-negative AE was finally made.</p></div><div><h3>Conclusion</h3><p>This case met the criteria for probable autoantibody-negative AE in children, as well as adults. Because of the temporal association and absence of another identifiable cause, her conditions may have been triggered by the COVID-19 vaccination. To our knowledge, this is the first published pediatric case of autoantibody-negative but probable AE following COVID-19 vaccination.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100173"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191178","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":"Neuromyelitis optica spectrum disorder after BIBP COVID-19 vaccine: A case report","authors":"Cristiam Gutierrez ,&nbsp;Sandro Rodríguez ,&nbsp;Miguel Trillo ,&nbsp;Alfredo Vásquez ,&nbsp;Wilfor Aguirre-Quispe","doi":"10.1016/j.nerep.2023.100174","DOIUrl":"https://doi.org/10.1016/j.nerep.2023.100174","url":null,"abstract":"<div><h3>Background</h3><p>With the COVID-19 vaccine now available, there have been occasional reports of post-vaccination neurological complications.</p></div><div><h3>Case presentation</h3><p>In this report, we present a case of neuromyelitis optica spectrum disorder (NMOSD) that developed one month after the patient received the second dose of BIBP COVID-19 vaccine (SARS-CoV-2-Vaccine [Vero Cell] Inactivated). The patient presented with itching, numbness in the hand and right side of the face, as well as nausea, vomiting, and hiccups. Brain MRI revelead lesions in the area postrema, medulla, and bilateral hypothalamus, which are typical of NMOSD. Serum antibodies to anti-AQP4 and anti-MOG were negative.</p></div><div><h3>Conclusions</h3><p>The pathogenesis of NMOSD development after vaccination is still unknown. NMOSD is generally aggressive and disabling, it is important for the neurologist to be attentive to the highly variable clinical presentation after COVID-19 vaccination for early diagnosis and effective treatment.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100174"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191180","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":"Clinical experience with the α2A-adrenoceptor agonist, guanfacine, and N-acetylcysteine for the treatment of cognitive deficits in “Long-COVID19”","authors":"Arman Fesharaki-Zadeh ,&nbsp;Naomi Lowe ,&nbsp;Amy F.T. Arnsten","doi":"10.1016/j.nerep.2022.100154","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100154","url":null,"abstract":"<div><h3>Background</h3><p>Prolonged cognitive deficits (“brain fog”) following COVID19 infection (long-COVID) are common and debilitating, yet there are currently no approved treatments. Cognitive impairment particularly targets the working memory and executive functions of the prefrontal cortex (PFC). The PFC has unusual neurotransmission and neuromodulation that render it vulnerable to stressors, and basic research has identified mechanisms that protect PFC connections. Based on the basic neuroscience data, we tried a combined open label treatment to bolster prefrontal function: the α2A-adrenoceptor agonist, guanfacine, which strengthens prefrontal connectivity, and the anti-oxidant, N- acetylcysteine (NAC), which protects mitochondria and reduces kynurenic acid blockade of NMDA receptors.</p></div><div><h3>Case report</h3><p>Twelve patients with “brain fog” including difficulties in executive functions were treated with guanfacine (1mg, PO bedtime for the first month, increased to 2mg after 1 month, if well-tolerated) and 600 mg NAC daily. Guanfacine+NAC improved cognitive abilities in eight of the twelve patients; four patients discontinued therapy, two for unspecified reasons and two due to hypotension and/or dizziness, common side effects of guanfacine. Those who stayed on guanfacine+NAC reported improved working memory, concentration, and executive functions, including a resumption of normal workloads. One patient briefly stopped taking guanfacine due to a hypotensive episode and reported a return of cognitive deficits that abated with resumed guanfacine treatment.</p></div><div><h3>Conclusion</h3><p>Although placebo-controlled trials will be needed to more rigorously demonstrate efficacy, as these agents have established safety, they may be immediately helpful in treating the large number of patients suffering from prolonged cognitive deficits following COVID19 infection.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100154"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191504","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":"Encephalopathy in 2 paediatric patients with SARS-CoV-2 infection benefited from early immunosuppressive treatment: A case report","authors":"Ka Fai Chan,&nbsp;Chi Lap Yuen","doi":"10.1016/j.nerep.2022.100162","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100162","url":null,"abstract":"<div><h3>Background</h3><p>Reports of neurological involvement are building up ever since COVID-19 has become a pandemic. Although rare, SARS-CoV-2 encephalopathy is a devastating complication associated with poor prognosis. Cytokine storm has been implicated in its pathophysiology.</p></div><div><h3>Case presentation</h3><p>We are reporting two paediatric patients who have contracted with COVID-19 and developed encephalopathy, who presented with status epilepticus and altered consciousness. Early aggressive immunosuppressive treatment was initiated promptly and both of them were able to recover without neurological deficit.</p></div><div><h3>Conclusion</h3><p>Patients with SARS-CoV-2 encephalopathy should be put on immunosuppressive therapy early for better neurological outcome. This improvement after the use of immunosuppressants further strengthened the cytokine storm theory in SARS-CoV-2 encephalopathy.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100162"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191505","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":"Amyloid Beta-Related Angiitis Presenting as Multiple Cerebral Infarcts","authors":"Amit Mehta ,&nbsp;Jonathan Isaacson ,&nbsp;Samuel Calabria ,&nbsp;Shannon P. Burton ,&nbsp;Veronica A. Craft ,&nbsp;Sana Somani ,&nbsp;Brent T. Harris ,&nbsp;Yongwoo Kim ,&nbsp;Jeffrey C. Mai ,&nbsp;Amie W. Hsia","doi":"10.1016/j.nerep.2022.100158","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100158","url":null,"abstract":"<div><h3>Background</h3><p>Amyloid beta-related angiitis (ABRA) is a rare sporadic granulomatous angiitis that includes features from both primary angiitis of the central nervous system angiitis (PACNS) and cerebral amyloid angiopathy (CAA). (<span>Rigby et al., 2011</span>) PACNS is clinically defined as a history of unexplained neurological symptoms in the setting of central nervous system vasculitis. (<span>Rigby et al., 2011</span>) CAA is pathologically defined by amyloid-beta (Aβ) deposition in the walls of arteries and arterioles. (<span>Rigby et al., 2011</span>) ABRA incorporates the diagnostic criteria of PACNS and distinct pathological features of CAA with the addition of intramural multinucleated giant cell infiltration and vessel wall destruction. (<span>Rigby et al., 2011</span>)</p></div><div><h3>Case Presentation</h3><p>This 67-year-old right-handed African American female with a past medical history of hypertension, diabetes, and alpha thalassemia trait presented to an outside hospital in an acute confusional state.</p></div><div><h3>Case Report</h3><p>The patient was transferred to our tertiary medical center after initial head CT was concerning for intraparenchymal and subarachnoid hemorrhages. Subsequent MRI brain confirmed a right thalamic hemorrhage and right cavernous hemangioma in addition to a right frontal subcortical ischemic infarct. A four-vessel diagnostic cerebral angiogram revealed severe distal stenoses in noncontiguous segments of the anterior and posterior intracranial arteries. MRI brain was repeated after the patient experienced new right sided weakness, which showed new scattered multi-territory ischemic infarcts. Laboratory studies were significant for and consistent with non-specific inflammation, and a right frontal brain biopsy was consistent with cerebral amyloid angiopathy. The patient was suspected to have ABRA, and her clinical condition stabilized yet remained disabled with corticosteroids and cyclophosphamide.</p></div><div><h3>Conclusion</h3><p>ABRA, an inflammatory disease related to PACNS, is a sequela of Aβ deposition in the cerebral vasculature. In this patient, we suspect that Aβ deposits in the cerebral vasculature triggered an intramural inflammatory cascade resulting in scattered ischemic infarcts. This case study highlights the unique clinical presentation, required diagnostic evaluation, and treatment options for this rare form of cerebral angiitis.</p></div>","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"3 ","pages":"Article 100158"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50191184","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":"COMPLEX HYPERKINETIC MOVEMENT DISORDER IN NEUROMYELITIS OPTICA SPECTRUM DISORDER EXACERBATION: A CASE REPORT","authors":"Ross Elizabeth Cortes, Jarunchai Anton Vatanagul, G. Saranza","doi":"10.1016/j.nerep.2022.100152","DOIUrl":"https://doi.org/10.1016/j.nerep.2022.100152","url":null,"abstract":"","PeriodicalId":100950,"journal":{"name":"Neuroimmunology Reports","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80657961","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}