Two Cases of Very-Late-Onset Neuromyelitis Optica Spectrum Disorder (NMOSD) in Patients over the Age of 80

We report two cases of very-late-onset neuromyelitis optica spectrum disorder (NMOSD) in patients over the age of 80 with transverse myelopathy as the initial manifestation. In both cases, the patients presented with paraplegia and sensory, bladder, and rectal disturbances. Thoracic magnetic resonance imaging showed longitudinal high-intensity signals on a T2-weighted image. The patients received high-dose methylprednisolone. Their serum was positive for anti-AQP4 antibody (cell-based assay) during the clinical course. They were diagnosed with NMOSD and treated with immunoadsorption, plasmapheresis, and followed up with daily prednisolone. Very-late-onset NMOSD in patients over the age of 80 has only rarely been reported. The present cases suggest that NMOSD should be considered for elderly patients presenting with transverse myelitis. Early diagnosis and treatment are important.

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Inebilizumab for treatment of neuromyelitis optica spectrum disorder

Neurodegenerative Disease Management ◽

10.2217/nmt-2021-0017 ◽

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.

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Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated With Lung Adenocarcinoma: A Case Report

Frontiers in Medicine ◽

10.3389/fmed.2021.743798 ◽

2022 ◽

Vol 8 ◽

Author(s):

Carlo Maiorca ◽

Federica Moret ◽

Valentina Martines ◽

Daniele Tramontano ◽

Maria Alessia Papassifachis ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Brain Mri ◽

Water Channel ◽

Clinical Signs ◽

Transverse Myelitis ◽

Spectrum Disorder ◽

Whole Body ◽

Parietal Pleura ◽

High Dose ◽

Neuromyelitis Optica Spectrum Disorder

Neuromyelitis Optica spectrum disorder is an inflammatory demyelinating disease affecting the central nervous system (CNS), characterized by triad optic neuritis, transverse myelitis, and area postrema syndrome. Antibodies directed against aquaporin-4 (AQP-4), a water channel expressed on the astrocytic membrane, are supposed to play a pathogenic role and are detected in ~80% of cases. Clinical signs of Neuromyelitis Optica spectrum disorder (NMOSD) in elderly patients should arouse the suspicion of paraneoplastic etiology. In this article, we discussed a case of a 76-year-old woman with a 2-month history of confusion, dysarthria, and progressive bilateral leg weakness. A whole-body CT scan showed a neoformation of 5 cm in diameter in the median lobe infiltrating the mediastinal pleura. The tumor had already spread to both the upper and lower right lobes, parietal pleura, and multiple lymph nodes. Pleural cytology revealed adenocarcinoma cells. The brain MRI documented hyperintense alteration in fluid-attenuated inversion recovery (FLAIR) images, involving the anterior portion of the corpus callosum and the periependymal white matter surrounding the lateral ventricles, with mild contrast enhancement on the same areas and meningeal tissue. T2-weighted spinal cord MRI sequences showed extended signal hyperintensity from bulbo-cervical junction to D7 metamer, mainly interesting the central component and the gray matter. Cerebrospinal fluid analysis revealed no neoplastic cells. Serum AQP-4 immunoglobulin (IgG) antibodies were found. Meanwhile, the patient rapidly developed progressive paraparesis and decreased level of consciousness. High-dose intravenous methylprednisolone therapy was started but her conditions rapidly deteriorated. No other treatment was possible.

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

Multiple Sclerosis Journal ◽

10.1177/1352458517705479 ◽

2017 ◽

Vol 23 (14) ◽

pp. 1950-1954 ◽

Cited By ~ 3

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.

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Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

Multiple Sclerosis Journal ◽

10.1177/1352458511414149 ◽

2011 ◽

Vol 18 (2) ◽

pp. 244-247 ◽

Cited By ~ 8

Author(s):

Sung-Min Kim ◽

Ji-Soo Kim ◽

Young Eun Heo ◽

Hye-Ran Yang ◽

Kyung Seok Park

Keyword(s):

Spinal Cord ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Aquaporin 4 ◽

Neuromyelitis Optica Spectrum Disorder ◽

Ocular Manifestation ◽

Initial Manifestation ◽

Ocular Symptoms ◽

Head Tremor ◽

Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

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Two cases of late-onset neuromyelitis optica spectrum disorder initially presenting with isolated cerebral white matter lesions

eNeurologicalSci ◽

10.1016/j.ensci.2018.11.008 ◽

2018 ◽

Vol 13 ◽

pp. 35-37 ◽

Cited By ~ 1

Author(s):

Tai Otani ◽

Takashi Irioka ◽

Yuko K Takahashi ◽

Kazumasa Soga ◽

Susumu Igarashi ◽

...

Keyword(s):

White Matter ◽

Neuromyelitis Optica ◽

Late Onset ◽

White Matter Lesions ◽

Spectrum Disorder ◽

Cerebral White Matter ◽

Neuromyelitis Optica Spectrum Disorder ◽

Cerebral White Matter Lesions

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Neuromyelitis optica spectrum disorder in pediatrics. Case report

Case reports ◽

10.15446/cr.v5n1.74943 ◽

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.

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AQP4-IgG-positive neuromyelitis optica spectrum disorder with late onset in Mexico.

Multiple Sclerosis and Related Disorders ◽

10.1016/j.msard.2020.102221 ◽

2020 ◽

Vol 43 ◽

pp. 102221 ◽

Cited By ~ 1

Author(s):

Guillermo Delgado-García ◽

Emmanuel Antonio-Luna ◽

Diego López-Mena ◽

Verónica Rivas-Alonso ◽

José Flores-Rivera ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Late Onset ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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Eculizumab in the treatment of neuromyelitis optica spectrum disorder

Immunotherapy ◽

10.2217/imt-2020-0163 ◽

2020 ◽

Vol 12 (14) ◽

pp. 1053-1066

Author(s):

Katrin Giglhuber ◽

Achim Berthele

Keyword(s):

Neuromyelitis Optica ◽

Controlled Trial ◽

Water Channel ◽

Spectrum Disorder ◽

Phase Iii ◽

Neuromyelitis Optica Spectrum Disorder ◽

Humanized Monoclonal Antibody ◽

The Complement System ◽

Terminal Complement ◽

Aqp4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the CNS which is distinct from multiple sclerosis and typically presents with a relapsing course of optic neuritis, myelitis and midline brain inflammatory lesions. In at least two-thirds of cases, antibodies against the water channel AQP4 can be found, which lead to an antibody-mediated activation of the complement system with consecutive damage to neuronal structures. Eculizumab, a humanized monoclonal antibody against the terminal complement component 5, was shown to significantly reduce the risk of NMOSD relapse in a Phase III placebo-controlled trial. Based on this, eculizumab (Soliris®) was the first drug to be formally approved for the treatment of anti-AQP4-antibody positive NMOSD in 2019.

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