Immune-mediated Disorders of the Central Nervous System

2018 ◽  
Author(s):  
Patricia K. Coyle
Keyword(s):  
Disease Activity ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Negative Consequences ◽  
Acute Transverse Myelitis ◽  
Immune Mediated ◽  
Real Risk ◽  
The Central Nervous System

This chapter reviews pregnancy in multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and acute transverse myelitis (ATM) syndrome. MS is a major acquired disease of young adults, with a rising female predominance. MS has no direct negative consequences on fertility or pregnancy. Pregnancy has a profound effect on MS, with decrease in disease activity during the last trimester counteracted by a three-month postpartum increase in disease activity. With the development of disease-modifying therapies, important questions arise about washout periods, the feasibility and risks of treating during pregnancy and breastfeeding, and the potential of treatment-related adverse fetal effects. Fortunately, there is good information to counsel women with MS. Neuromyelitis Optica Spectrum Disorder (NMOSD) is a neuroimmune channelopathy. It is a distinct disorder from MS. NMOSD disease activity is not favorably affected by pregnancy. The postpartum period has real risk for disabling attacks. This influences recommendations about breastfeeding and how quickly to resume therapy postpartum. Acute transverse myelitis (ATM) syndrome can occur in both MS and NMOSD but can also be due to other disorders. Workup and treatment of ATM during pregnancy is reviewed, as well as implications for delivery.

scholarly journals Case Report: Postvaccination Anti–Myelin Oligodendrocyte Glycoprotein Neuromyelitis Optica Spectrum Disorder

2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
First Trimester ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Future Research ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.

Inebilizumab for treatment of neuromyelitis optica spectrum disorder

2021 ◽  
Vol 11 (5) ◽  
pp. 341-352
Author(s):  
Mark J Tullman ◽  
Aram Zabeti ◽  
Scott Vuocolo ◽  
Quinn Dinh
Keyword(s):  
Neuromyelitis Optica ◽  
Plasma Cells ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Neurologic Injury ◽  
Immune Mediated ◽  
The Us ◽  
Us Fda ◽  
Aqp4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease characterized by recurrent optic neuritis and transverse myelitis often resulting in severe disability. Anti-AQP4-immunoglobulin G (IgG) is a pathogenic product of CD19-positive plasma cells found in most, but not all, individuals with NMOSD and is associated with immune-mediated neurologic injury. Inebilizumab, an afucosylated humanized IgG1κ, anti-CD19 monoclonal antibody, may target pathogenic CD19-expressing B cells. In a Phase II/III trial, inebilizumab significantly reduced the proportion of participants experiencing an NMOSD attack and was well tolerated versus placebo. Fewer treated participants had worsening disability than those receiving placebo. Inebilizumab was approved in 2020 by the US FDA for treatment of anti-AQP4 antibody positive NMOSD.

scholarly journals Unrecognized neuromyelitis optica spectrum disorder with pontine and corpus callosum microhemorrhage

2021 ◽  
pp. 86-86
Author(s):  
Igor Nosek ◽  
Jasmina Boban ◽  
Dmitar Vlahovic ◽  
Biljana Radovanovic ◽  
Dejan Kostic ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Cross Sectional ◽  
Immune Mediated ◽  
Demyelinating Lesions ◽  
Improve Patient

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) represents an immune-mediated neuroinflammatory syndrome, classified as separate entity after discovery of aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). The neuroimaging spectrum of NMOSD classically consisted of bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM), recently broadened with lesions in area postrema, diencephalon, brainstem and cerebellum, and extensive cord atrophy. Case report: Here we present a case of an AntiAQP4-positive 65-year old female patient who initially presented with underappreciated LETM and developed multiple cerebral and cerebellar lytic demyelinating lesions associated with acute long segment optic nerve involvement two years later. Two new imaging findings are described in this case: the involvement of complete cross-sectional area of pons and microhemorrhage in the pons and corpus callosum. Conclusion: Raising suspicion of NMOSD is of a crucial importance in cases with isolated LETM in order to prevent relapses in Anti-AQP4 positive cases, improve patient outcome and recovery.

Neuromyelitis optica spectrum disorder as a paraneoplastic syndrome: a rare and challenging diagnosis

2021 ◽  
Vol 14 (5) ◽  
pp. e239389
Author(s):  
Mukhtar Gibril ◽  
Richard Walters
Keyword(s):  
Neuromyelitis Optica ◽  
Cell Lymphoma ◽  
Transverse Myelitis ◽  
Inflammatory Lesion ◽  
B Cell Lymphoma ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Large B Cell Lymphoma ◽  
The Central Nervous System ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system, often associated with aquaporin-4 antibodies (AQP4-Ab). NMOSD may present as a subacute myelopathy, progressing over days with MRI revealing a contiguous inflammatory lesion of the spinal cord, ≥3 vertebral segments, a longitudinally extensive transverse myelitis. We describe an unusual paraneoplastic form of AQP4-Ab NMOSD that developed in a patient with an advanced diffuse large B-cell lymphoma. The patient had an unusual hyperacute onset, reaching a clinical nadir within hours.

Bright spotty lesions as an imaging marker for neuromyelitis optica spectrum disorder

2021 ◽  
pp. 135245852199425
Author(s):  
Sara Salama ◽  
Michael Levy
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Term Treatment ◽  
Spectrum Disorder ◽  
Imaging Features ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Spinal Imaging ◽  
Specificity And Sensitivity ◽  
Long Term Treatment ◽  
The Central Nervous System

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disorder of the central nervous system (CNS). Aquaporin-4 (AQP4) antibodies in the serum are highly specific for the diagnosis of NMOSD, but the sensitivity remains under 90% allowing for diagnosis of AQP4 IgG seronegative disease. It remains of crucial importance to identify seronegative NMOSD myelitis as early as the first attack to initiate long-term treatment that will reduce future relapses and disability and to avoid potentially harmful treatments such as those of multiple sclerosis (MS). Over the years, many spinal imaging features have been reported to favour the diagnosis of NMOSD, but only longitudinally extensive transverse myelitis (LETM) was specific enough to make the diagnostic criteria in the AQP4 IgG seronegative cases. Bright spotty lesions (BSLs), which are defined as hyperintense lesions on axial T2-weighted images and sometimes associated with T1 low signal, are now reported to have a higher specificity and sensitivity compared to LETM in predicting a diagnosis of NMOSD against other causes of myelitis. In the review, we aim to highlight the position of BSLs in diagnosing NMOSD as well as its possible role as a prognostic factor for the clinical outcome.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

scholarly journals Neuromyelitis optica spectrum disorder in pediatrics. Case report

Case reports ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. 11-18
Author(s):  
Jhon Camacho ◽  
Sebastian Zuleta ◽  
Maria Paula Alba ◽  
Andrea Hernandez ◽  
Carlos Navas
Keyword(s):  
Neuromyelitis Optica ◽  
Interesting Case ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Inflammatory Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System ◽  
High Doses ◽  
Aqp4 Antibody ◽  
Timely Treatment

Introduction: Neuromyelitis optica is an inflammatory disorder of the central nervous system that accounts for 5% of demyelinating diseases in pediatrics. Its clinical presentation is variable and associated to the involved area of the central nervous system.Case presentation: This is the case of a 15-year-old patient who consulted several times for nonspecific neurological symptoms. During his last visit to the Clínica Universitaria Colombia in Bogotá, he presented with bilateral optic neuritis, associated with frontal and parietal headache. Immunophenotyping studies were carried out, reporting positive IgG anti-aquaporin 4 antibodies (anti-AQP4 antibody), thus leading to a diagnosis of seropositive neuromyelitis optica spectrum disorder (NMOSD). Management with methylprednisolone pulses was initiated with subsequent outpatient management with rituximab that allowed stabilizing the disease.Discussion: This is an interesting case due to its insidious and uncertain onset in a pediatric patient. It was possible to evaluate clinical and diagnostic differences in relation to its presentation in adults. NMOSD mediated by anti-AQP4 is rare; brain and bone marrow MRI are essential for diagnosis. The treatment of choice for acute conditions consists of high doses of methylprednisolone.Conclusion: This disorder may result in irreversible neurological damage; for this reason, high suspicion is required for early diagnosis and timely treatment.

Neuromyelitis optica spectrum disorder and systemic lupus erythematosus

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1722-1726 ◽  
Author(s):  
M M Thabah ◽  
Sekar D ◽  
R Pranov ◽  
M M V Moulitej ◽  
A Ramesh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Systemic Lupus ◽  
Autoimmune Conditions ◽  
Inflammatory Syndrome ◽  
Extensive Transverse Myelitis

Neuromyelitis optica spectrum disorder is an inflammatory syndrome that is associated with many autoimmune conditions. We present the case of a patient who had longitudinally extensive transverse myelitis and antibodies to aquaporin 4 IgG (AQP4-IgG). Based on presence of lymphopenia, further workup revealed strong ANA positivity, anti-Sm antibodies, and low serum complements suggesting presence of systemic lupus erythematosus. The patient promptly responded to intravenous pulse methylprednisolone and five sessions of plasma exchange. At 1 year, she is on maintenance treatment with low dose prednisolone, azathioprine, and hydroxychloroquine, she has had no relapse and no other clinical features of lupus. This case is an illustration that neuromyelitis optica spectrum disorder can be the first manifestation of systemic lupus erythematosus.

scholarly journals Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Author(s):  
Marco A. Lana-Peixoto ◽  
Natália Talim
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Area Postrema ◽  
Water Channel ◽  
Clinical Manifestations ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Immunosuppressive Drugs ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.

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