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)
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
Clinical Neurology
Shabeer Paul, Gouranga Prasad Mondal, Ramesh Bhattacharyya, Kartik Chandra Ghosh, Imtiyaz Ahmad Bhat
Summary: Over the past two decades, the disease concept of NMOSD has significantly changed with the detection of MOG antibody and the understanding of immune astrocytopathy. The revised diagnostic criteria have widened the clinical spectrum of NMOSD, allowing for earlier diagnosis and prompt initiation of effective immunosuppression for better long-term outcomes. Challenges still remain in treating seronegative NMOSD due to limited treatment options.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(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
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
Michael Levy, Kazuo Fujihara, Jacqueline Palace
Summary: Neuromyelitis optica spectrum disorder is a rare autoimmune disease affecting the CNS, with recent trials showing benefits of new therapies in preventing future attacks. However, differences in efficacy, safety, tolerability, and practical considerations of these therapies may impact their use in real-world populations of patients. Future research should focus on unmet needs, including aquaporin-4 seronegative disease and treatments for acute relapses and recovery from autoimmune attacks in the CNS.
Review
Medicine, General & Internal
Yachinee Naphattalung, Wanicha Leetiratanai Chuenkongkaew, Niphon Chirapapaisan, Poramaet Laowanapiban, Supattra Sawangkul
Summary: The current data from this systematic review are insufficient to conclude definitively whether therapeutic plasma exchange (PLEX) is effective in improving visual acuity in cases of neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD). The objective of this study was to evaluate whether PLEX effectively improves visual function for acute optic neuritis (ON) in NMO or NMOSD. Twelve studies were included in the analysis, but the data were inconclusive in determining the effectiveness of PLEX for treating acute ON in NMO/NMOSD.
ANNALS OF MEDICINE
(2023)
Review
Dermatology
Laurent Misery, Steeve Genestet, Fabien Zagnoli
Summary: Neuromyelitis optica spectrum disorder is a disease characterized by attacks of optic neuritis and/or longitudinally extensive transverse myelitis and the presence of anti-aquaporin-4 autoantibodies. The disease worsens over a few days and slowly improves in the weeks or months after reaching the maximum clinical deficit, but recovery is usually incomplete. Early diagnosis and emergency treatment are crucial for managing the disease. Dermatologists can help in achieving this and neuropathic pruritus can be useful for diagnosing the disease. New skin infections or sexually transmitted diseases can trigger relapses of neuromyelitis optica.
Article
Clinical Neurology
Raffaella Pizzolato Umeton, Michael Waltz, Gregory S. Aaen, Leslie Benson, Mark Gorman, Manu Goyal, Jennifer S. Graves, Yolanda Harris, Lauren Krupp, Timothy E. Lotze, Nikita M. Shukla, Soe Mar, Jayne Ness, Mary Rensel, Teri Schreiner, Jan-Mendelt Tillema, Shelly Roalstad, Moses Rodriguez, John Rose, Emmanuelle Waubant, Bianca Weinstock-Guttman, Charles Casper, Tanuja Chitnis
Summary: This retrospective cohort study evaluated cases of children with NMOSD and found that the use of disease-modifying treatments, particularly rituximab, is associated with a lowered annualized relapse rate in children with NMOSD AQP4+.
Article
Clinical Neurology
Oliver Schmetzer, Elisa Lakin, Ben Roediger, Ankelien Duchow, Susanna Asseyer, Friedemann Paul, Nadja Siebert
Summary: This study examined the role of anti-AQP4-IgG in patients with Neuromyelitis optica spectrum disorder (NMOSD). The results suggest that anti-AQP4-IgG may not play a predominant role in driving NMOSD, but rather may be associated with the disease and could be a relevant factor in only a subgroup of patients.
FRONTIERS IN NEUROLOGY
(2021)
Review
Immunology
Edgar Carnero Contentti, Jorge Correale
Summary: Neuromyelitis optica (NMO) is a chronic inflammatory autoimmune disease characterized by acute optic neuritis and transverse myelitis. It is caused by a pathogenic serum IgG antibody targeting the water channel aquaporin 4 (AQP4). Recent studies have shown significant progress in identifying new treatments for NMOSD.
JOURNAL OF NEUROINFLAMMATION
(2021)
Article
Clinical Neurology
Frederike Cosima Oertel, Svenja Specovius, Hanna G. Zimmermann, Claudia Chien, Seyedamirhosein Motamedi, Charlotte Bereuter, Lawrence Cook, Marco Aurelio Lana Peixoto, Mariana Andrade Fontanelle, Ho Jin Kim, Jae-Won Hyun, Jacqueline Palace, Adriana Roca-Fernandez, Maria Isabel Leite, Srilakshmi Sharma, Fereshteh Ashtari, Rahele Kafieh, Alireza Dehghani, Mohsen Pourazizi, Lekha Pandit, Anitha D'Cunha, Orhan Aktas, Marius Ringelstein, Philipp Albrecht, Eugene May, Caryl Tongco, Letizia Leocani, Marco Pisa, Marta Radaelli, Elena H. Martinez-Lapiscina, Hadas Stiebel-Kalish, Sasitorn Siritho, Jerome de Seze, Thomas Senger, Joachim Havla, Romain Marignier, Alvaro Cobo Calvo, Denis Bichuetti, Ivan Maynart Tavares, Nasrin Asgari, Kerstin Soelberg, Ayse Altintas, Rengin Yildirim, Uygur Tanriverdi, Anu Jacob, Saif Huda, Zoe Rimler, Allyson Reid, Yang Mao-Draayer, Ibis Soto de Castillo, Axel Petzold, Ari J. Green, Michael R. Yeaman, Terry Smith, Alexander U. Brandt, Friedemann Paul
Summary: This study highlights the importance of attack prevention to avoid severe neuroaxonal damage and vision loss caused by ON in NMOSD. Therapies to ameliorate attack-related damage, especially during the first attack, are a clinical need. Mild neuroaxonal changes in ON-unaffected eyes may be solely due to contralateral ON attacks and do not indicate clinically relevant progression, but require further investigation.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Article
Neurosciences
Daissy Liliana Mora Cuervo, Gisele Hansel, Douglas Kazutoshi Sato
Summary: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disease that is characterized by the presence of autoantibodies targeting the water channel aquaporin-4 (AQP4-IgG). The core clinical manifestations of NMOSD include optic neuritis, longitudinally extensive myelitis, and area postrema syndrome. Diagnosis is based on clinical manifestations, magnetic resonance imaging findings, and the presence of AQP4-IgG. Recent advances in the understanding of NMOSD immunobiology have led to approved treatments such as eculizumab, satralizumab, and inebilizumab.
CURRENT OPINION IN NEUROBIOLOGY
(2022)
Article
Clinical Neurology
Sean J. Pittock, Michael Barnett, Jeffrey L. Bennett, Achim Berthele, Jerome de Seze, Michael Levy, Ichiro Nakashima, Celia Oreja-Guevara, Jacqueline Palace, Friedemann Paul, Carlo Pozzilli, Marcus Yountz, Kerstin Allen, Yasmin Mashhoon, Ho Jin Kim
Summary: The CHAMPION-NMOSD study evaluated the efficacy and safety of ravulizumab in adult patients with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder. Ravulizumab, which has a longer half-life compared to eculizumab, significantly reduced relapse risk in these patients.
ANNALS OF NEUROLOGY
(2023)
Editorial Material
Clinical Neurology
Eoin P. Flanagan, Richard W. Marsh, Brian G. Weinshenker
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
Clinical Neurology
Hannah H. Zhao-Fleming, Cristina Valencia Sanchez, Elia Sechi, Jery Inbarasu, Eelco F. Wijdicks, Sean J. Pittock, John J. Chen, Dean M. Wingerchuk, Brian G. Weinshenker, Sebastian Lopez-Chiriboga, Divyanshu Dubey, Jan-Mendelt Tillema, Michel Toledano, Hemang Yadav, Eoin P. Flanagan
Summary: The study compared the frequency, characteristics, and outcomes of MOGAD and AQP4-NMOSD attacks requiring ventilatory support, finding no significant difference in the frequency of attacks needing ventilation between the two, but AQP4-NMOSD attacks may have higher morbidity and mortality. Additionally, there are differences in the indications and duration of ventilatory support between MOGAD and AQP4-NMOSD attacks.
Article
Clinical Neurology
Elia Sechi, Karl N. Krecke, Steven A. Messina, Marina Buciuc, Sean J. Pittock, John J. Chen, Brian G. Weinshenker, A. Sebastian Lopez-Chiriboga, Claudia F. Lucchinetti, Nicholas L. Zalewski, Jan Mendelt Tillema, Amy Kunchok, Salvatore Monaco, Padraig P. Morris, James P. Fryer, Adam Nguyen, Tammy Greenwood, Stephanie B. Syc-Mazurek, B. Mark Keegan, Eoin P. Flanagan
Summary: MOGAD patients more frequently experience complete resolution of MRI T2 lesions compared to AQP4-IgG-NMOSD and MS. This finding has implications for diagnosis, disease activity monitoring, and clinical trial design, while also providing insights into the pathogenesis of CNS demyelinating diseases.
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)
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)