scholarly journals Seronegative Neuromyelitis Optica Spectrum - The challenges on disease definition and pathogenesis

2014 ◽  
Vol 72 (6) ◽  
pp. 445-450 ◽  
Author(s):  
Douglas Kazutoshi Sato ◽  
Dagoberto Callegaro ◽  
Marco Aurélio Lana-Peixoto ◽  
Ichiro Nakashima ◽  
Kazuo Fujihara
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Laboratory Findings ◽  
Disease Definition ◽  
Highly Sensitive ◽  
Diagnostic Work ◽  
Work Up ◽  
Extensive Transverse Myelitis ◽  
Aqp4 Antibody

Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.

scholarly journals The expanded spectrum of neuromyelitis optica: evidences for a new definition

2012 ◽  
Vol 70 (10) ◽  
pp. 807-813 ◽  
Author(s):  
Marco A Lana-Peixoto ◽  
Dagoberto Callegaro
Keyword(s):  
Optic Nerve ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Index Event ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

scholarly journals Atypical presentations of neuromyelitis optica

2011 ◽  
Vol 69 (5) ◽  
pp. 824-828 ◽  
Author(s):  
Douglas Sato ◽  
Kazuo Fujihara
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Brain Lesions ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Atypical Presentations ◽  
Different Characteristics ◽  
Extensive Transverse Myelitis ◽  
Intractable Hiccups

Neuromyelitis optica (NMO) is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4) has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.

Contribution of spinal cord biopsy to diagnosis of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder

2013 ◽  
Vol 20 (7) ◽  
pp. 882-888 ◽  
Author(s):  
M Ringelstein ◽  
I Metz ◽  
K Ruprecht ◽  
A Koch ◽  
J Rappold ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Diagnostic Process ◽  
Antibody Testing ◽  
Magnetic Resonance Imaging Mri ◽  
Extensive Transverse Myelitis ◽  
Aqp4 Antibody

Longitudinally extensive transverse myelitis is characteristic but not pathognomonic for neuromyelitis optica spectrum disorders (NMOSDs) and may mimic local tumors. In this retrospective study based on a cohort of 175 NMOSD patients we identified seven patients who initially presented with a longitudinally extensive spinal cord lesion and underwent spinal cord biopsy due to magnetic resonance imaging (MRI)-suspected malignancies. Remarkably, routine neuropathology was inconclusive and did not guide the diagnostic process to anti-aquaporin-4 (AQP4)-seropositive NMOSD. Serious postoperative complications occurred in 5/7 patients and persisted during follow-up in 2/7 patients (29%). Considering these sequelae, AQP4-antibody testing should be mandatory in patients with inconclusive longitudinally extensive spinal cord lesions prior to biopsy.

An early case of neuromyelitis optica: on a forgotten report by Jacob Lockhart Clarke, FRS

2011 ◽  
Vol 17 (11) ◽  
pp. 1384-1386 ◽  
Author(s):  
S Jarius ◽  
B Wildemann
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
English Language ◽  
Medical Literature ◽  
Transverse Myelitis ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Devic’S Syndrome ◽  
Early Case ◽  
Extensive Transverse Myelitis

We discuss a forgotten report by the famous British neuroanatomist, neuropathologist and neurologist Jacob Augustus Lockhart Clarke (1817–1880) about a 17-year-old girl with bilateral optic neuritis and longitudinally extensive transverse myelitis. This report, which appeared in 1865, i.e. 15 years prior to Wilhelm Erb’s much-cited paper on the coincidence of optic neuritis and acute myelitis, represents the first known account of a case of Devic’s syndrome or neuromyelitis optica in the English-language medical literature.

scholarly journals Systemic Lupus Erythematosus (SLE) Complicated by Neuromyelitis Optica (NMO – Devic's Disease): Clinic-Pathological Report and Review of the Literature

2014 ◽  
Vol 7 ◽  
pp. CCRep.S15177 ◽  
Author(s):  
Mohammad Adawi ◽  
Bishara Bisharat ◽  
Abdalla Bowirrat
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Demyelinating Disease ◽  
Clinical Laboratory ◽  
Transverse Myelitis ◽  
Systemic Lupus ◽  
Igg Antibody ◽  
Laboratory Findings

Neuromyelitis optica (NMO) is usually a relapsing demyelinating disease of the central nervous system associated with optic neuritis, transverse myelitis involving three or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody. NMO is often mistaken for multiple sclerosis and there are relatively sporadic publications about NMO and overlapping systemic or organ-specific autoimmune diseases, such as systemic lupus erythematosus (SLE). We described a unique case of a 25-year-old Arab young woman who was diagnosed with SLE, depending on clinical, laboratory investigations and after she had fulfilled the diagnostic criteria for SLE and had presented the following findings: constitutional findings (fatigue, fever, and arthralgia); dermatologic finding (photosensitivity and butterfly rash); chronic renal failure (proteinuria up to 400 mg in 24 hours); hematologic and antinuclear antibodies (positivity for antinuclear factor (ANF), anti-double-stranded DNA antibodies, direct Coombs, ANA and anti-DNA, low C4 and C3, aCL by IgG and IgM). Recently, she presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiological, and laboratory findings especially seropositivity for NMO-IgG were compatible with NMO. Accurate diagnosis is critical to facilitate initiation of immunosuppressive therapy for attack prevention. This case illustrates that NMO may be associated with SLE.

First Presentation of an Acquired Demyelinating Syndrome

2017 ◽  
Vol 16 (03) ◽  
pp. 164-170
Author(s):  
Rachel Gottlieb-Smith ◽  
Amy Waldman
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Optic Neuritis ◽  
Clinical Features ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
The Central Nervous System

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease: practical considerations

2018 ◽  
Vol 19 (3) ◽  
pp. 187-195 ◽  
Author(s):  
Maciej Juryńczyk ◽  
Anu Jacob ◽  
Kazuo Fujihara ◽  
Jacqueline Palace
Keyword(s):  
Neuromyelitis Optica ◽  
Inflammatory Diseases ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Lesion Volume ◽  
Imaging Features ◽  
Visual Disability ◽  
Permanent Disability ◽  
Spectrum Disorders ◽  
Aqp4 Antibody

The field of central nervous system (CNS) inflammatory diseases has recently broadened to include a new condition associated with pathogenic serum antibodies against myelin oligodendrocyte glycoprotein (MOG). This is distinct from multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody neuromyelitis optica spectrum disorders (NMOSD). MOG antibody-associated disease phenotypes are varied and range from classical neuromyelitis optica to acute demyelinating encephalomyelitis and cortical encephalitis. The diagnosis depends on using a reliable, specific and sensitive assay of the antibody. Clinical and imaging features of MOG-associated syndromes overlap with AQP4 antibody NMOSD but can be usually distinguished from MS: in particular, the silent lesions typical of MS that progressively increase lesion volume are rare in MOG antibody disease. The disease can relapse but medium-term immunosuppression appears to be protective. Permanent disability, particularly severe ambulatory and visual disability, is less frequent than in AQP4 antibody NMOSD and usually results from the onset attack. However, sphincter and sexual dysfunction after a transverse myelitis is common. Here we review the practical aspects of diagnosing and managing a patient with MOG antibody-associated disease.

scholarly journals Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sitong Guo ◽  
Hanqiu Jiang ◽  
Libin Jiang ◽  
Jingting Peng ◽  
Hongjuan Liu ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Age At Onset ◽  
Chinese Patients ◽  
Intravenous Methylprednisolone ◽  
Methylprednisolone Pulse ◽  
Factors Influencing ◽  
Intravenous Methylprednisolone Pulse ◽  
Aqp4 Antibody

AbstractThis study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho =  − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

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