Res Vestib Sci.  2019 Jun;18(2):50-53. 10.21790/rvs.2019.18.2.50.

Neuromyelitis Optica Spectrum Disorder Presented with Upbeat Nystagmus and Intractable Vomiting

• Kim H
• Kim JM
• Nam TS
• Lee SH

• KMID: 2450268
• DOI: http://doi.org/10.21790/rvs.2019.18.2.50

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating autoimmune disease of central nervous system characterized by relapsing attacks that target the optic nerves and spinal cord, as well as aquaporin-4 (AQP4) enriched periventricular brain regions. The area postrema (AP), located in the dorsal medulla, is the chemosensitive vomiting center and has high AQP-4 expression. The AP syndrome with unexplained hiccups, nausea, and vomiting is one of the core clinical characteristics in the NMOSD and maybe the first presenting symptom. We experienced a 25-year-old woman presented with intractable vomiting, dizziness and oscillopsia. Upbeat nystagmus detected on the bedside examination led to comprehensive neurological workups including magnetic resonance imaging, and she was diagnosed as the AP syndrome. Ten months later, she experienced a recurrence as a longitudinally extensive transverse myelitis and the diagnosis was finally compatible with NMOSD without AQP4-IgG. NMOSD, especially the AP syndrome, should be considered in any dizzy patient with intractable vomiting, and detailed neuro-otologic and neuro-ophthalmologic examinations are warranted for the correct diagnosis.

Keyword

Area postrema; Neuromyelitis optica spectrum disorder; Vomiting; Vertical nystagmus

MeSH Terms

Adult
Area Postrema
Autoimmune Diseases
Brain
Central Nervous System
Diagnosis
Dizziness
Female
Hiccup
Humans
Magnetic Resonance Imaging
Myelitis, Transverse
Nausea
Neuromyelitis Optica*
Nystagmus, Pathologic
Optic Nerve
Recurrence
Spinal Cord
Vomiting*