Article
Clinical Neurology
Paula Barreras, Eleni S. Vasileiou, Angeliki G. Filippatou, Kathryn C. Fitzgerald, Michael Levy, Carlos A. Pardo, Scott D. Newsome, Ellen M. Mowry, Peter A. Calabresi, Elias S. Sotirchos
Summary: Rituximab treatment reduces the annualized relapse rate in AQP4-IgG-seropositive NMOSD, especially when administered consistently without gaps in treatment or B-cell reconstitution. In MOGAD patients, although a decrease in relapses was observed after rituximab initiation, this effect appeared to be less pronounced compared to AQP4-IgG-seropositive NMOSD. Severe infections and hypogammaglobulinemia were seen in a significant number of patients, underscoring the importance of close monitoring for infectious complications.
Review
Clinical Neurology
Tania Kuempfel, Katrin Giglhuber, Orhan Aktas, Ilya Ayzenberg, Judith Bellmann-Strobl, Vivien Haeussler, Joachim Havla, Kerstin Hellwig, Martin W. Huemmert, Sven Jarius, Ingo Kleiter, Luisa Klotz, Markus Krumbholz, Friedemann Paul, Marius Ringelstein, Klemens Ruprecht, Makbule Senel, Jan-Patrick Stellmann, Florian Then Bergh, Corinna Trebst, Hayrettin Tumani, Clemens Warnke, Brigitte Wildemann, Achim Berthele
Summary: This manuscript provides practical recommendations for managing acute attacks and implementing preventive immunotherapies for neuromyelitis optica spectrum disorders (NMOSD). The authors discuss the pillars of NMOSD therapy, diagnostic markers, recent advances in treatment, and the development of new therapies. The manuscript also offers an overview of the current state of knowledge on NMOSD treatments and provides recommendations for therapy management and the use of immunotherapies.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Su Yeon Park, Young Nam Kwon, Sunyoung Kim, Seung-Hyun Kim, Jong Kuk Kim, Jun-Soon Kim, Tai-Seung Nam, Young Gi Min, Kyung Seok Park, Jin-Sung Park, Jin Myoung Seok, Jung-Joon Sung, Eunhee Sohn, Kyong Jin Shin, Jin-Hong Shin, Ha Young Shin, Seong-il Oh, Jeeyoung Oh, Byeol-A Yoon, Sanggon Lee, Jong-Mok Lee, Hye Lim Lee, Kyomin Choi, So-Young Huh, Myoung-jin Jang, Ju-Hong Min, Byoung Joon Kim, Sung-Min Kim
Summary: This multicentre retrospective study found that early initiation of rituximab treatment can reduce long-term disability in patients with NMOSD, especially among those with early to middle-age onset, female sex, and severe attacks.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
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
Biochemistry & Molecular Biology
Tzu-Lun Huang, Jia-Kang Wang, Pei-Yao Chang, Yung-Ray Hsu, Cheng-Hung Lin, Kung-Hung Lin, Rong-Kung Tsai
Summary: This review provides an update on recent studies related to neuromyelitis optica spectrum disorder (NMOSD), including the pathophysiology of the disease, the potential use of serum and cerebrospinal fluid cytokines as biomarkers, the clinical utilization of ocular coherence tomography, and the comparison of different animal models of NMOSD.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
Qin Du, Ziyan Shi, Hongxi Chen, Ying Zhang, Jiancheng Wang, Yuhan Qiu, Zhengyang Zhao, Qin Zhang, Hongyu Zhou
Summary: This study investigated mortality and causes of death among Chinese NMOSD patients, finding that lack of regular immunotherapy, short first interattack interval, and older age were independent predictors of death. Infections, particularly respiratory infections, were the most common cause of death, highlighting the importance of early and consistent immunotherapy in preventing deaths from infections in NMOSD patients.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2021)
Review
Chemistry, Multidisciplinary
Subramanian Thangaleela, Bhagavathi Sundaram Sivamaruthi, Arumugam Radha, Periyanaina Kesika, Chaiyavat Chaiyasut
Summary: Neuromyelitis optica (NMO) is a rare autoimmune disorder that affects the optic nerve and spinal cord, causing severe symptoms such as optic neuritis and transverse myelitis. It was initially misdiagnosed as multiple sclerosis until the discovery of the AQP4 antibody. Various factors, including genetics, environment, medication, and vitamin deficiencies, contribute to the development of NMO. The binding of AQP4 antibodies leads to damage in astrocytes and neuroinflammation. Treatment options for NMO include corticosteroids, apheresis therapies, immunosuppressive drugs, and B cell inactivating and complement cascade blocking agents.
APPLIED SCIENCES-BASEL
(2023)
Article
Clinical Neurology
Ingo Kleiter, Anthony Traboulsee, Jacqueline Palace, Takashi Yamamura, Kazuo Fujihara, Albert Saiz, Adil Javed, David Mayes, H-Christian von Buedingen, Gaelle Klingelschmitt, Daniela Stokmaier, Jeffrey L. Bennett
Summary: Satralizumab demonstrates long-term efficacy in reducing relapse risk in AQP4-IgG+ NMOSD patients, with a sustained effect over 3.5 years of treatment.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2023)
Article
Cardiac & Cardiovascular Systems
Kei Inai, Ryo Inuzuka, Hiroshi Ono, Masaki Nii, Shinichi Ohtsuki, Yoshihiko Kurita, Atsuhito Takeda, Keiichi Hirono, Kohta Takei, Satoshi Yasukouchi, Tadahiro Yoshikawa, Yoshiyuki Furutani, Eriko Shimada, Tokuko Shinohara, Tomohiro Shinozaki, Yutaka Matsuyama, Hideaki Senzaki, Toshio Nakanishi
Summary: This study aimed to identify the risk factors for mortality among haemodynamic parameters obtained early after the Fontan operation. The study found that older age, low ejection fraction, low oxygen saturation, high central venous pressure, and low mean systemic arterial pressure were independent predictors of mortality.
EUROPEAN HEART JOURNAL
(2022)
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
Immunology
Jinhua Tang, Xiaopeng Zeng, Jun Yang, Lei Zhang, Hang Li, Rui Chen, Shi Tang, Yetao Luo, Xinyue Qin, Jinzhou Feng
Summary: This study explores the expression patterns of RGMa in NMOSD and its correlation with the clinical features of the disease. The results suggest that RGMa may serve as a potential biomarker for predicting the severity, disability, and clinical features of NMOSD.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Valentina Camera, Silvia Messina, Kariem Tarek Elhadd, Julia Sanpera-Iglesias, Romina Mariano, Yael Hacohen, Ruth Dobson, Stefano Meletti, Evangeline Wassmer, Ming J. Lim, Saif Huda, Cheryl Hemingway, Maria Isabel Leite, Sithara Ramdas, Jacqueline Palace
Summary: The study identified predictors of first relapse and long-term disabilities in pediatric-onset AQP4-IgG NMOSD, including age at onset, race, onset symptoms, and response to acute therapy. These predictors can help guide future pediatric clinical trials and early therapeutic decisions in AQP4-IgG NMOSD.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Medicine, General & Internal
Vinita Gupta, Saurabh Luthra, Shrey Maheshwari, Shrutanjoy M. Das
Summary: Early differentiation between NMOSD and MS is crucial, as NMOSD (especially the relapsing variant) has more severe morbidity. Clinically, changes in symptoms can guide treatment plans; for patients with recurrent optic neuritis, timely spinal MRI and aquaporin-4 antibody testing are crucial steps in diagnosis.
Article
Immunology
Yacen Hu, Qiying Sun, Fang Yi, Lingyan Yao, Yun Tian, Haiyun Tang, Mengchuan Luo, Nina Xie, Zhiqin Wang, Xinxin Liao, Lin Zhou, Hongwei Xu, Yafang Zhou
Summary: This study investigated the clinical characteristics and prognosis outcomes in NMOSD patients with different age of onset, finding that late-onset NMOSD patients have more frequent transverse myelitis but less optic neuritis and brainstem/cerebral attacks as the first attack compared to early-onset NMOSD patients. Older age at disease onset is associated with increased protein levels in cerebrospinal fluid, longer spinal cord lesions, and higher EDSS score at last follow-up.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Multidisciplinary Sciences
Eun Bin Cho, Daegyeom Kim, ByeongChang Jeong, Jong Hwa Shin, Yeon Hak Chung, Sung Tae Kim, Byoung Joon Kim, Cheol E. Han, Ju-Hong Min
Summary: This study compared the alterations of white matter connectivity between multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and healthy controls (HC). The results showed that both MS and NMOSD groups had disrupted white matter networks compared to HC, and these alterations were associated with clinical parameters.
SCIENTIFIC REPORTS
(2022)
Correction
Oncology
N. Cayuela, E. Jaramillo-Jimenez, C. Majos, R. Velasco, T. Boget, F. Graus, J. Bruna, M. Simo
Article
Clinical Neurology
Mickael Bonnan, Paul Money, Pauline Desblache, Raluca Marasescu, Lise-Marlene Puvilland, Stephanie Demasles, Camille Dahan, Elsa Krim, Alan Tucholka, Senan Doyle, Bruno Barroso
Summary: Recent studies have found chronic leptomeningeal enhancement in late-acquired FLAIR sequences of secondary progressive multiple sclerosis patients, which may lead to long-term cortical atrophy. A case report of spontaneously remitting meningeal uptake suggests that MRI may overlook the potential burden of previous regressive meningeal lesions.
NEUROLOGICAL SCIENCES
(2021)
Article
Clinical Neurology
Mar Petit-Pedrol, Mar Guasp, Thais Armangue, Cinzia Lavarino, Andres Morales La Madrid, Albert Saiz, Francesc Graus, Josep Dalmau
Summary: This study did not find GluD2 antibodies in a large cohort of patients with Opsoclonus-Myoclonus Syndrome (OMS), suggesting that these antibodies may not be biomarkers of OMS.
Editorial Material
Clinical Neurology
Francesc Graus
Summary: This study explores the clinical and immunological features of paraneoplastic cochleovestibulopathy which is associated with cancer.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2021)
Article
Public, Environmental & Occupational Health
Calixto Aguero-Martinez, Alina Gonzalez-Quevedo
Summary: Cytokine storm is a severe complication that can occur during the course of COVID-19, leading to excessive inflammatory response and ultimately resulting in tissue damage, organ failure, and death.
Article
Clinical Neurology
Mickael Bonnan, Emeline Berthelot, Philippe Cabre
Summary: Initial clinical manifestations of NMOSD may rarely overlap with MS. Fingolimod may trigger severe attacks in patients with NMOSD previously misdiagnosed as MS. Among high-risk Afro-Caribbean patients, the risk of misdiagnosed NMOSD in MS was estimated to be 1.9%. After initiation of fingolimod, relapses occurred within a month in five patients, with a shift to more severe disorder and large brain lesions noted.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2021)
Article
Clinical Neurology
Zurina Lestayo O'Farrill, Alina Gonzalez-Quevedo, Joel Gutierrez-Gil, Jose Luis Hernandez-Caceres, Vivian Sistach-Vega
Summary: A functional assessment tool for acute inflammatory neuropathies (FAAIN-GBS) was constructed and validated in this study. The tool consists of two subscales (extension and intensity) and the final score is obtained by averaging both dimensions. The validation results showed good internal consistency and validity, indicating its potential usefulness in neurological practice. However, further studies with larger populations are needed to improve this instrument.
NEUROLOGICAL RESEARCH
(2022)
Article
Public, Environmental & Occupational Health
Calixto Machado-Curbelo, Joel Gutierrez-Gil, Alina Gonzalez-Quevedo
Summary: Globally, the omicron variant of SARS CoV-2 has caused a significant increase in COVID-19 cases with milder clinical symptoms and reduced hospitalizations. The omicron wave has spread faster than previous waves, completely replacing the delta variant within weeks, raising global concerns about pandemic control. The symptoms associated with omicron resemble those of the common cold and rarely include loss of taste and smell. Research suggests that direct invasion of the brainstem by SARS-CoV-2 through the olfactory nerves may explain the refractory respiratory distress syndrome seen in severe cases.
Article
Medicine, General & Internal
Sara Beca, Claudia Elera-Fitzcarrald, Albert Saiz, Sara Llufriu, Maria C. Cid, Bernardo Sanchez-Dalmau, Alfredo Adan, Gerard Espinosa
Summary: This study retrospectively observed a cohort of patients with Susac syndrome (SS), describing their clinical characteristics, diagnostic results, treatment regimens, and clinical course. The study found that most patients eventually developed the classical clinical triad during the disease course, and majority of the cases went into remission within two years. A detailed diagnostic workup and follow-up should be conducted for suspected SS patients to achieve a definite diagnosis.
JOURNAL OF CLINICAL MEDICINE
(2022)
Letter
Otorhinolaryngology
Calixto Machado, Joel Gutierrez-Gil, Alina Gonzalez-Quevedo
INTERNATIONAL FORUM OF ALLERGY & RHINOLOGY
(2023)
Article
Clinical Neurology
Zenaida Milagros Hernandez-Diaz, Marisol Pena-Sanchez, Mayelin Pimienta-Medina, Sergio Gonzalez-Garcia, Marta Brown Martinez, Marianela Arteche Prior, Yasmany Martin Arias, Edwin Humberto Hodelin-Maynard, Armando Abreu Duque, Alina Gonzalez-Quevedo
Summary: This study aimed to evaluate the relationship between cardiovascular risk calculated with the HEARTS app and cerebral small vessel disease (CSVD) burden in a population without cardio-cerebrovascular diseases, and estimate the prevalence of CSVD in low risk individuals. The results showed that CSVD was present even in individuals with low cardiovascular risk. Older age and arterial hypertension were independent predictors of the severity of CSVD.
BMJ NEUROLOGY OPEN
(2023)
Article
Clinical Neurology
Mickael Bonnan, Henri Courtade, Stephane Debeugny
Summary: This study investigates the influence of disease-modifying drugs (DMD) and high-dose corticosteroids (CS) on immunoglobulin (Ig) levels in patients with multiple sclerosis (MS). The results demonstrate that CS infusion is strongly associated with a decrease in Ig levels during DMD treatment, suggesting the need for further studies considering the effect of CS on Ig levels.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Clinical Neurology
Mickael Bonnan, Henri Courtade, Stephane Debeugny
Summary: This study describes the natural history of Ig levels in multiple sclerosis (MS) patients. The results show that IgG levels were higher in samples taken before the onset of MS symptoms. Additionally, overall Ig levels remained stable during the first 1 to 20 years after onset.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Article
Clinical Neurology
Mickael Bonnan, Stephane Olindo, Aissatou Signate, Quentin Lobjois, Maeva Stephant, Dalia Dimitri Boulos, Philippe Cabre
Summary: The study investigated the overlap between NMOSD features and HTLV1 infection, finding that acute onset of NMOSD-like HAM may be more common than expected and should be considered in high-risk patients. HTLV1 infection may lead to excessive inflammation of the targeted funiculi, resulting in extensive but often transient cord lesions. Some HAM cases demonstrate specific MRI lesions, and the most severe cases may mimic NMOSD attacks.
MULTIPLE SCLEROSIS JOURNAL-EXPERIMENTAL TRANSLATIONAL AND CLINICAL
(2021)
Article
Clinical Neurology
Mickael Bonnan, Sylvie Ferrari, Henri Courtade, Paul Money, Pauline Desblache, Bruno Barroso, Stephane Debeugny
Summary: The study aimed to evaluate the safety and efficacy of intrathecal rituximab in progressive MS, with results showing no significant changes in clinical outcomes and biomarkers of inflammation, possibly due to its limited efficiency in the cerebrospinal fluid.
MULTIPLE SCLEROSIS INTERNATIONAL
(2021)