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August 2022; 12 (4) Clinical/Scientific Note

Area Postrema Syndrome

An Initial Presentation of Double-Seropositive AQP4 and MOG Antibodies

Abhay Ranjan, Sanaullah Mudassir, Neetu Sinha, Ankit Panjwani, Ashok Kumar
First published July 11, 2022, DOI: https://doi.org/10.1212/CPJ.0000000000200065
Abhay Ranjan
Department of Neurology (AR, SM, AP, AK) and Department of Radiology (NS), IGIMS, Patna, India.
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Sanaullah Mudassir
Department of Neurology (AR, SM, AP, AK) and Department of Radiology (NS), IGIMS, Patna, India.
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Neetu Sinha
Department of Neurology (AR, SM, AP, AK) and Department of Radiology (NS), IGIMS, Patna, India.
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Ankit Panjwani
Department of Neurology (AR, SM, AP, AK) and Department of Radiology (NS), IGIMS, Patna, India.
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Ashok Kumar
Department of Neurology (AR, SM, AP, AK) and Department of Radiology (NS), IGIMS, Patna, India.
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Citation
Area Postrema Syndrome
An Initial Presentation of Double-Seropositive AQP4 and MOG Antibodies
Abhay Ranjan, Sanaullah Mudassir, Neetu Sinha, Ankit Panjwani, Ashok Kumar
Neurol Clin Pract Aug 2022, 12 (4) e82-e84; DOI: 10.1212/CPJ.0000000000200065

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Abstract

Objectives Area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD). APS is mostly associated with neuromyelitis optica (NMO) and rarely reported in myelin oligodendrocyte glycoprotein antibody disease. We herein report a case of APS as an initial presentation of double-seropositive aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibodies.

Methods The patient fulfilled the NMOSD diagnostic criteria. Brain MRI, CSF studies, electrophysiologic test, and serum NMO and MOG antibody testing were performed.

Results An elderly woman initially presented to a gastroenterology outpatient department with a history of nausea, vomiting, and hiccups for 3 weeks. A detailed medical evaluation, including upper gastrointestinal endoscopy, was performed, which showed normal findings with no improvement with symptomatic therapy. A neurologic examination showed bilateral nystagmus, postural imbalance, and gait ataxia. An MRI examination of the brain showed T2/fluid attenuated inversion recovery hyperintensity in the dorsal medulla involving area postrema. Both anti-NMO and anti-MOG antibodies were found to be positive in serum. She was treated with intravenous methyl prednisolone with complete symptomatic resolution.

Discussion Double-seropositive APS-onset NMOSD has not been previously reported in literature. An early diagnosis and treatment result in the resolution of APS-related symptoms and prevent further progression of the disease.

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.

  • Submitted and externally peer reviewed. The handling editor was Deputy Editor Kathryn Kvam, MD.

  • Glossary

    AP
    area postrema
    APS
    area postrema syndrome
    AQP4
    aquaporin-4
    MOG
    myelin oligodendrocyte glycoprotein
    MOGAD
    myelin oligodendrocyte glycoprotein antibody disease
    NMO
    neuromyelitis optica
    NMOSD
    neuromyelitis optic spectrum disorder
    • Received December 24, 2021.
    • Accepted June 13, 2022.
    • © 2022 American Academy of Neurology
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    The Nerve!: Rapid online correspondence

    • Reader Response: Area Postrema Syndrome: An Initial Presentation of Double-Seropositive AQP4 and MOG Antibodies
      • Jodie Burton, Associate Professor, University of Calgary
      Submitted August 09, 2022
    • RE: Area Postrema Syndrome: An Initial Presentation of Double-Seropositive AQP4 and MOG Antibodies
      • Khichar Shubhakaran, Senior Professor Neurology, M D M Hospital, Dr S N Medical College, Jodhpur (Rajasthan) India
      Submitted July 25, 2022
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