Article
Immunology
Yun-Fan You, Man Chen, Yue Tang, Wen-Xiang Yu, Xiao-Wei Pang, Yun-Hui Chu, Hang Zhang, Ke Shang, Gang Deng, Luo-Qi Zhou, Sheng Yang, Wei Wang, Jun Xiao, Dai-Shi Tian, Chuan Qin
Summary: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory demyelinating disease of the central nervous system (CNS). TREM2, expressed by microglia, plays a crucial role in microglial activation and function during NMOSD demyelination.
JOURNAL OF NEUROINFLAMMATION
(2023)
Review
Medicine, General & Internal
Wenqun Li, Jiaqin Liu, Wei Tan, Yedi Zhou
Summary: NMOSD is an autoimmune neurological disease where microglia play key roles in immune regulation and pathological progression. Activation of microglia by IL-6 and IFN-Is in NMOSD leads to inflammation and disease development.
INTERNATIONAL JOURNAL OF MEDICAL SCIENCES
(2021)
Article
Medicine, General & Internal
Carlo Maiorca, Federica Moret, Valentina Martines, Daniele Tramontano, Maria Alessia Papassifachis, Simone Bini, Claudia Caramazza, Mario Fontana, Piernatale Lucia, Maurizio Inghilleri
Summary: This article discusses a case of a 76-year-old female patient with Neuromyelitis Optica spectrum disorder (NMOSD), suspected to be associated with a paraneoplastic etiology. Through various imaging studies and laboratory tests, the patient's condition was confirmed, and the treatment outcomes were poor.
FRONTIERS IN MEDICINE
(2022)
Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica and its spectrum is a relapsing demyelinating disorder of the central nervous system, encompassing six syndromes and associated with aquaporin-4 autoantibodies. Effective treatment is able to prevent relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Medicine, General & Internal
Dean M. Wingerchuk, Claudia F. Lucchinetti
Summary: Neuromyelitis Optica, previously known as Devic's disease, is a relapsing demyelinating disorder of the central nervous system that encompasses six syndromes and is associated with aquaporin-4 autoantibodies. Effective treatment is crucial in preventing relapses.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Article
Clinical Neurology
Sophie Chatterton, John Douglas Edward Parratt, Karl Ng
Summary: Eculizumab has been shown to be effective in maintaining disease remission in NMOSD patients who are AQP4-IgG seropositive. The utility of eculizumab in treating acute episodes of NMOSD is under review, but it may potentially aid recovery from severe attacks and minimize disability accrual.
FRONTIERS IN NEUROLOGY
(2022)
Article
Ophthalmology
Andrew R. Carey, J. Fernando Arevalo
Summary: A retrospective chart review of 572 NMOSD patients revealed that 1% of them had uveitis, with bilateral anterior uveitis being the most common subtype. The majority of patients were treated with rituximab for their NMOSD, and in many cases, uveitis attacks preceded demyelination attacks.
OCULAR IMMUNOLOGY AND INFLAMMATION
(2022)
Article
Immunology
Ke Shang, Chang Cheng, Chuan Qin, Jun Xiao, Gang Deng, Bi-Tao Bu, Sha-Bei Xu, Dai-Shi Tian
Summary: This case report is the first to describe a demyelination illness associated with anti-flotillin autoantibodies, showing characteristics of both neuromyelitis optica spectrum disease and multiple sclerosis. Although it currently seems to be a subtype of MS, there is still a possibility that it may be distinct from both MS and NMOSD.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Clinical Neurology
Yong Guo, Vanda A. Lennon, Joseph E. Parisi, Bogdan Popescu, Christina Vasquez, Sean J. Pittock, Charles L. Howe, Claudia F. Lucchinetti
Summary: Neuromyelitis optica is an autoimmune inflammatory disorder that affects CNS astrocytes. This study reveals the complex astrogliotic reactions and their role in the evolution and potential for repair of lesions, independent of aquaporin-4 loss or lysis.
Review
Allergy
Nadim Taheri, Julie Sarrand, Muhammad S. Soyfoo
Summary: This article reviews the recent pathogenic findings in NMO, particularly the newly discovered role of anti-aquaporin-4 antibodies in triggering cerebral lesions. The concept of the spectrum of diseases associated with NMO is also discussed.
CURRENT ALLERGY AND ASTHMA REPORTS
(2023)
Article
Immunology
Sheng-Hui Chang, Jing Wang, Xu Zhang, Ning Zhao, Kun Jia, Ming Yi, Qiu-Xia Zhang, Hui Zhai, Xiao-Wen Li, Chun-Sheng Yang, Li Yang, Lin-Jie Zhang
Summary: A small proportion of NMOSD patients have anti-NF155 antibodies, which are more likely to co-exist with autoimmune diseases. These patients with anti-NF155 antibodies tended to show mildly abnormal results in nerve conduction studies.
CLINICAL AND EXPERIMENTAL IMMUNOLOGY
(2021)
Article
Immunology
Zhuhe Liu, Yuanyuan Wang, Yuewen Ding, Haitao Wang, Jun Zhang, Honghao Wang
Summary: The level of CXCL7 is increased in NMOSD patients and mouse models, and is associated with astrocyte injury, inflammatory infiltration, and demyelination. Inhibiting CXCL7 can reverse AQP4 loss and attenuate inflammation.
CLINICAL IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Koon-Ho Chan, Chi-Yan Lee
Summary: NMOSD is an autoimmune disorder that predominantly affects females, leading to serious CNS inflammatory disorders. The pathogenesis involves AQP4-IgG autoantibodies targeting aquaporin-4, triggering astrocytopathy and neuroinflammation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Immunology
Tingjun Chen, Dale B. Bosco, Yanlu Ying, Dai-Shi Tian, Long-Jun Wu
Summary: NMO is an autoantibody-triggered neuro-inflammatory disease that mainly affects the spinal cord and optic nerve. Studies have shown significant microglial activation in NMO lesions, indicating a potential role for microglia in NMO pathology.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Clinical Neurology
Yoshiki Takai, Tatsuro Misu, Kazuo Fujihara, Masashi Aoki
Summary: This review summarizes the pathological findings of MOGAD and discusses unresolved issues. The pathological features of MOGAD are clearly different from those of multiple sclerosis (MS) and AQP4 antibody-positive neuromyelitis optica spectrum disorders (NMOSD), suggesting that MOGAD is an independent autoimmune demyelinating disease entity. Further research is needed to clarify the exact pathomechanisms of demyelination and its relation to the clinical phenotype and symptoms leading to disability in MOGAD patients.
FRONTIERS IN NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
Eoin P. Flanagan, Richard W. Marsh, Brian G. Weinshenker
Article
Clinical Neurology
Elia Sechi, Karl N. Krecke, Sean J. Pittock, Divyanshu Dubey, A. Sebastian Lopez-Chiriboga, Amy Kunchok, Brian G. Weinshenker, Nicholas L. Zalewski, Eoin P. Flanagan
Summary: A negative spinal cord MRI should not discourage MOG-IgG testing in patients with acute/subacute myelitis. It is important to recognize that myelitis symptoms can still indicate MOG-IgG-associated myelitis even when initial MRI results are negative.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Andrew J. Solomon, Roman Pettigrew, Robert T. Naismith, Salim Chahin, Stephen Krieger, Brian Weinshenker
Summary: A survey involving both NR and MSS revealed knowledge gaps in specific core elements of the McDonald criteria, leading to potential misdiagnosis of multiple sclerosis. Errors were particularly noted in identifying typical MS syndromes and correctly identifying MRI lesions, suggesting a need for concerted educational efforts to prevent misdiagnosis.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Roman M. Kassa, Elia Sechi, Eoin P. Flanagan, Timothy J. Kaufmann, Orhun H. Kantarci, Brian G. Weinshenker, Jay Mandrekar, William F. Schmalstieg, M. Mateo Paz Soldan, B. Mark Keegan
Summary: The study compared the onset of progressive motor impairment in patients with highly restricted versus unlimited CNS lesion burden. It found that the age of motor-progression onset is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden. The critical demyelinating lesion is a major contributor to motor-progression onset.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Elia Sechi, Steven Messina, B. Mark Keegan, Marina Bitciuc, Sean J. Pittock, Orhun H. Kantarci, Brian G. Weinshenker, Eoin P. Flanagan
Summary: In long-standing MS patients, the presence of potential critical spinal cord lesions is more common in those with secondary progressive MS compared to those with relapsing-remitting MS. These critical lesions may play an important role in motor progression in MS.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Amy Kunchok, Eoin P. Flanagan, Melissa Snyder, Ruba Saadeh, John J. Chen, Brian G. Weinshenker, Andrew McKeon, Sean J. Pittock
Summary: MOG-IgG1-associated disorders are not strongly associated with non-organ and organ-specific autoantibodies, while AQP4-IgG+ NMOSD is significantly associated with systemic lupus erythematous (SLE), indicating differences in co-existing systemic and organ-specific autoimmunity between the two conditions.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Shreya Nayak, Elia Sechi, Eoin P. Flanagan, Steven Messina, Roman Kassa, Orhun Kantarci, Brian G. Weinshenker, B. Mark Keegan
Summary: In patients with motor progression due to critical demyelinating lesions, the likelihood of new inflammatory activity, defined by active lesions and clinical relapses, is low. Disease-modifying therapies that reduce demyelinating relapses and active MRI lesions may have uncertain benefit in these cohorts.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Amy Kunchok, Eoin P. Flanagan, Karl N. Krecke, John J. Chen, J. Alfredo Caceres, Justin Dominick, Ian Ferguson, Revere Kinkel, John C. Probasco, Miguel Ruvalcaba, Jonathan D. Santoro, Kurt Sieloff, Jeremy Timothy, Brian G. Weinshenker, Andrew McKeon, Sean J. Pittock
Summary: This study investigated the co-existence of neuronal antibodies in patients with MOG-IgG1, finding that NMDA-R-IgG was the most frequently detected neuronal antibody. Patients with both MOG-IgG1 and NMDA-R-IgG were more likely to present with encephalopathy and seizures. Testing for both MOG-IgG1 and NMDA-R-IgG may be important in patients with these clinical features.
MULTIPLE SCLEROSIS JOURNAL
(2021)
Article
Clinical Neurology
Romain Marignier, Jeffrey L. Bennett, Ho Jin Kim, Brian G. Weinshenker, Sean J. Pittock, Dean Wingerchuk, Kazuko Fujihara, Friedemann Paul, Gary R. Cutter, Ari J. Green, Orhan Aktas, Hans-Peter Hartung, Fred D. Lublin, Ian M. Williams, Jorn Drappa, Dewei She, Daniel Cimbora, William Rees, Michael Smith, John N. Ratchford, Eliezer Katz, Bruce A. C. Cree
Summary: In the N-MOmentum trial, inebilizumab reduced the risk of 3-month disability progression compared to placebo in participants with NMOSD. Baseline subgroups did not affect the treatment effect observed with inebilizumab, and participants treated with inebilizumab were more likely to have a favorable outcome on the mRS.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Editorial Material
Clinical Neurology
Adrian Budhram, Shailee S. Shah, Christopher P. Wood, Michael A. Lane, Kyle Smoot, Brian G. Weinshenker
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2022)
Article
Medicine, General & Internal
Ruba S. Saadeh, Sandra C. Bryant, Andrew McKeon, Brian Weinshenker, David L. Murray, Sean J. Pittock, Maria Alice Willrich
Summary: The study aims to determine and validate a cerebrospinal fluid (CSF) k (KCSF) value comparable to the detection of cerebrospinal fluid-specific oligoclonal bands (OCB) for diagnosing multiple sclerosis (MS). The results show that a KCSF value of 0.1 mg/dL can be used as a valid alternative to OCB testing, providing a standardized quantitative measure with similar sensitivity and specificity, and reducing human error and cost.
MAYO CLINIC PROCEEDINGS
(2022)
Review
Clinical Neurology
Sean J. Pittock, Anastasia Zekeridou, Brian G. Weinshenker
Summary: NMOSD is a rare inflammatory CNS disease primarily manifesting as severe optic neuritis and myelitis. Recent studies have shown that targeted therapies effectively prolong the time to first relapse in NMOSD.
NATURE REVIEWS NEUROLOGY
(2021)
Article
Clinical Neurology
Nathan D. Schilaty, Filippo Savoldi, Zahra Nasr, Brian G. Weinshenker
Summary: The study identified neuromotor control factors associated with the McArdle sign that can differentiate multiple sclerosis patients from healthy controls and other myelopathy conditions. These factors largely determine muscle force production changes observed in the McArdle sign.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2022)
Article
Clinical Neurology
Shailee S. Shah, Pearse Morris, Marina Buciuc, Deena Tajfirouz, Dean M. Wingerchuk, Brian G. Weinshenker, Eric R. Eggenberger, Marie Di Nome, Sean J. Pittock, Eoin P. Flanagan, M. Tariq Bhatti, John J. Chen
Summary: This study aimed to characterize interattack optic nerve enhancement in neuromyelitis optica spectrum disorder (NMOSD) patients. The results showed that approximately 16.8% of patients with preceding optic neuritis (ON) had optic nerve enhancement during the interattack period. These enhancements typically occurred at the site of prior ON and may represent intermittent breakdown of the blood-brain barrier or subclinical ON. Occasionally, new asymptomatic lesions were observed. However, these findings were not associated with visual recovery.
Article
Clinical Neurology
Rafid Mustafa, Theodore J. Passe, Alfonso S. Lopez-Chiriboga, Brian G. Weinshenker, Karl N. Krecke, Nicholas L. Zalewski, Felix E. Diehn, Elia Sechi, Jay Mandrekar, Timothy J. Kaufmann, Padraig P. Morris, Sean J. Pittock, Michel Toledano, Giuseppe Lanzino, Allen J. Aksamit, Neeraj Kumar, Claudia F. Lucchinetti, Eoin P. Flanagan
Summary: The study shows that MRI gadolinium enhancement patterns can assist in the diagnosis of myelopathies with longitudinally extensive T2 lesions, with excellent agreement between raters educated on this topic.
NEUROLOGY-CLINICAL PRACTICE
(2021)
