Predictive Factors of Resistance to High-Dose Steroids Therapy in Acute Attacks of Neuromyelitis Optica Spectrum Disorder

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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EBI2-expressing B cells in neuromyelitis optica spectrum disorder with AQP4-IgG: Association with acute attacks and serum cytokines

Journal of Neuroimmunology ◽

10.1016/j.jneuroim.2021.577637 ◽

2021 ◽

pp. 577637

Author(s):

Seol-Hee Baek ◽

Boram Kim ◽

Je-Young Shin ◽

Seok-Jin Choi ◽

So Hyun Ahn ◽

...

Keyword(s):

B Cells ◽

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Serum Cytokines ◽

Acute Attacks

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Outcomes from acute attacks of neuromyelitis optica spectrum disorder correlate with severity of attack, age and delay to treatment

Multiple Sclerosis and Related Disorders ◽

10.1016/j.msard.2018.12.010 ◽

2019 ◽

Vol 28 ◽

pp. 60-63 ◽

Cited By ~ 2

Author(s):

Aditya Banerjee ◽

Jennifer Ng ◽

Jessica Coleman ◽

Juan Pablo Ospina ◽

Maureen Mealy ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Acute Attacks

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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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First Manifestation of AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder Associated with the COVID-19 mRNA Vaccine BNT162b2

10.21203/rs.3.rs-1152345/v1 ◽

2022 ◽

Author(s):

Shu Umezawa ◽

Katsura Ioka ◽

Satoshi Aizawa ◽

Yuichi Tashiro ◽

Kazuo Yoshizawa

Keyword(s):

Neuromyelitis Optica ◽

Plasma Cells ◽

Area Postrema ◽

Cervical Spinal Cord ◽

Large Population ◽

Spectrum Disorder ◽

Cervical Cord ◽

High Dose ◽

Neuromyelitis Optica Spectrum Disorder ◽

Lateral Column

Abstract BNT162b2 is one of the effective COVID-19 vaccines. However, some researchers have also reported that the vaccines caused some neurological complications. Here, we present a case of a 52-year-old female who developed aquaporin (AQP) 4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) fourteen days after the first dose of BNT162b2. She experienced pain of the neck, weakness of the left arm and leg, numbness of the left hand, and impaired temperature sensation of the right leg. MRI showed T2WI hyperintense lesions in the area postrema and cervical spinal cord ranging from C1 to C6 level, and Gd-enhanced lesions from C3 to C5 level; especially left lateral column was predominantly enhanced. Cell-based assays showed anti-AQP4 antibody (AQP4Ab) was positive. We diagnosed AQP4-IgG-positive NMOSD. After high-dose glucocorticoid therapy, she is showing improved symptoms. The present case was characterized by the findings that a Gd-enhanced lesion in the cervical cord localized dominantly at the left lateral column, consistent with the side of the shoulder where the vaccine was injected. Many studies suggested that AQP4-IgG-positive NMOSD development has multistep mechanisms following the blood-brain barrier (BBB) breakdown. We suspected that BNT162b2-associated immune responses lead to BBB disruptions. Through the limitedly damaged BBB, the plasma cells producing AQP4Abs might be recruited to CNS, and AQP4Abs might bind to the cervical cord and the area postrema. A large population-based study revealed that BNT162b2-associated complications were less likely to be observed than COVID-19 infectious symptoms. However, considering the present case, neurologists need to observe the conditions following vaccination.

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