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Pediatric neuromyelitis optica spectrum disorder: Case report of postrema syndrome

Abstract

Link of Video Abstract: https://youtu.be/w-yWNre4RNg

 

Introduction: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an inflammatory disease of the optic nerve and spinal cord characterized by severe immune mediated axonal damage and demyelination. Optic neuritis, transverse myelitis, or combination of both are common first clinical attack symptoms. Area postrema syndrome was infrequently described as an initial presentation of pediatric NMOSD.

Case Presentation: We present the case of 10-year-old male presents with fever, nausea, vomiting, hiccups, and vertigo suggesting an Area Postrema Syndrome. Brain Magnetic Resonance Imaging (MRI) with contrast shows a pathological lesion without contrast enhancement on the periventricle of the 3rd ventricle extends to the bilateral optic tracts. Whole spine MRI showed no abnormalities. Aquaporin-4 was found seropositive on lumbar puncture examination. He was treated with intravenous methylprednisolone for 5 consecutive days followed by IVIG for 5 days and mycophenolate mofetil as maintenance therapy. Patient then showed a significant improvement in symptoms.

Conclusion: Pediatric NMOSD with core criteria of area postrema syndrome as the first clinical attack is rare. Relapse may occur, requiring further follow-up and maintenance therapy. We should raise awareness on the possibility of postrema syndrome in pediatric NMOSD cases that are intractable with medication.

References

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How to Cite

Puspitasari, V., Goenawan, V. F., Solansa, T., & Cenweikiawan, A. (2024). Pediatric neuromyelitis optica spectrum disorder: Case report of postrema syndrome . Bali Medical Journal, 13(1), 722–724. https://doi.org/10.15562/bmj.v13i1.5043

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Vivien Puspitasari
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Vonny Fibrianty Goenawan
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Tracy Solansa
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Anderson Cenweikiawan
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