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
Medical Laboratory Technology
Ruba S. Saadeh, Paola A. Ramos, Alicia Algeciras-Schimnich, Eoin P. Flanagan, Sean J. Pittock, Maria Alice Willrich
Summary: Multiple sclerosis (MS) is an immune-mediated central nervous system inflammatory demyelinating disease. Laboratory tests play an important role in the diagnosis of MS. Biomarkers discovered in the past two decades have been incorporated into clinical practice for the diagnosis of MS mimics. This review summarizes the importance of test selection, assay methodology, optimal sample for testing, and diagnostic utility of these biomarkers. Other laboratory testing methods that can aid in the differentiation between MS and biomarker-defined CNS demyelinating diseases are described. The role of biomarkers of disease activity and prognosis is also discussed, along with future perspectives and the potential for discovering additional biomarkers.
CLINICAL CHEMISTRY
(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)
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
Medicine, General & Internal
Evangelos Katsarogiannis, Anne-Marie Landtblom, Anna Kristoffersson, Johan Wikstrom, Robert Semnic, Shala G. Berntsson
Summary: The study aimed to determine the prevalence of OCB-negative MS patients and assess the misdiagnosis rate in this patient group. The results showed that 33% of OCB-negative MS patients were misdiagnosed, indicating that the absence of OCB should raise suspicion of possible misdiagnosis in MS patients and prompt a diagnostic reassessment.
JOURNAL OF CLINICAL MEDICINE
(2023)
Letter
Clinical Neurology
Elia Sechi, Marina Buciuc, Eoin P. Flanagan, Sean J. Pittock, Samantha A. Banks, A. Sebastian Lopez-Chiriboga, M. Tariq Bhatti, John J. Chen
Summary: The variability in cerebrospinal fluid (CSF) findings of myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD) is influenced by attack phenotype and preceding treatment. Patients with clinically isolated optic neuritis have lower white-blood-cell elevation compared to those with myelitis or brain/brainstem attacks. CSF pleocytosis is more common in optic neuritis patients with coexisting asymptomatic brain and/or spine MRI T2-lesions.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2021)
Review
Clinical Neurology
Abdu Kisekka Musubire, Judith Derdelinckx, Tatjana Reynders, David B. Meya, Paul R. Bohjanen, Patrick Cras, Barbara Willekens
Summary: Neuromyelitis optica (NMO) is more frequent in Asian and African populations, with limited data on epidemiology, clinical presentation, and treatment in African continent. This systematic review highlights the challenges in diagnosis and management of NMOSD, as well as the need for systematic data collection and improved access to healthcare services in Africa to adequately monitor and address the burden of the disease.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Review
Clinical Neurology
Peter G. E. Kennedy, Woro George, Xiaoli Yu
Summary: Intrathecal immunoglobulin G (IgG) and oligoclonal bands (OCBs) detected in both the brain and cerebrospinal fluid (CSF) are seminal features of multiple sclerosis (MS). The presence of OCBs correlates with elevated disease burden and severity and supports the diagnosis of MS. Despite numerous investigations, the precise antigenic specificity of OCBs remains elusive.
JOURNAL OF NEUROLOGY
(2023)
Article
Neurosciences
Maria-Eleftheria Evangelopoulos, Georgios Koutsis, Fotini Boufidou, Manolis Markianos
Summary: Cholesterol levels may be associated with disease characteristics in multiple sclerosis, particularly with demyelination and immune response. This study found lower levels of plasma and cerebrospinal fluid cholesterol in patients with multiple sclerosis. Patients with positive oligoclonal bands had even lower cerebrospinal fluid cholesterol levels, which may be related to disease progression and enhanced humoral immune response.
NEUROBIOLOGY OF DISEASE
(2022)
Article
Infectious Diseases
Weny Rinawati, July Kumalawati
Summary: The presence of oligoclonal bands can indicate the presence of disease-specific antibodies, possibly due to the activation of specific clones of B lymphocytes. The detection of oligoclonal bands is useful in the diagnosis of the neurodegenerative disease SSPE.
JOURNAL OF INFECTION IN DEVELOPING COUNTRIES
(2022)
Article
Immunology
Tobias Zrzavy, Romana Hoftberger, Isabella Wimmer, Thomas Berger, Paulus Rommer, Stefan Macher
Summary: Autoimmune encephalitis presents a diagnostic challenge due to its diverse clinical manifestations. A monocentric cohort study showed that some AIE patients had normal CSF results at diagnosis, highlighting the importance of antibody testing in timely diagnosis. OCB status in anti-NMDAR AIE patients may serve as a potential prognostic biomarker, but further studies are needed for validation.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Medicine, General & Internal
Jin Young Kim, Hye Jeong Oh, Yuntae Kim, Jin Myoung Seok
Summary: This report describes a rare case of a patient with ALS who developed NMOSD 4 years after being diagnosed with ALS. The patient was bedridden with a tracheostomy due to ALS at the time of NMOSD onset. Despite receiving high dose methylprednisolone, the patient experienced pain relief but no improvement in leg weakness. Psychological follow-up is recommended for patients with ALS who are at risk of developing other neurological disorders.
Article
Immunology
C. Kyllesbech, N. Trier, R. Slibinskas, E. Ciplys, A. Tsakiri, J. L. Frederiksen, G. Houen
Summary: Intrathecal antibody synthesis to viruses is associated with multiple sclerosis. This study found that IgG levels to certain viruses were elevated in the serum and cerebrospinal fluid of multiple sclerosis patients, while levels of antibodies to other viruses were decreased. Additionally, antigen panels showed high sensitivity and specificity in the diagnosis of multiple sclerosis.
JOURNAL OF NEUROIMMUNOLOGY
(2022)
Article
Clinical Neurology
Yi Xie, Bo Chen, Qiong Wang, Xuejiao Chen, Wenwen Lai, Yaping Xu, Saiyue Deng, Zhiyuan Yu, Minjie Xie, Bitao Bu, Dapeng Mou, Chenju Yi, Fengfei Ding, Wei Wang
Summary: This study provides evidence that astrocytes targeted by NMO-IgG release pathogenic exosomes, which play a crucial role in the pathogenesis of NMOSD. Specifically, miR-129-2-3p is identified as the key miRNA responsible for demyelination. The levels of exosomal miR-129-2-3p are elevated in NMOSD patients and correlated with disease severity.
ANNALS OF NEUROLOGY
(2023)