Research in Vestibular Science

Case Report

Neuromyelitis Optica Spectrum Disorder Presented with Upbeat Nystagmus and Intractable Vomiting: Hyunsoo Kim, Jae-Myung Kim, Tai-Seung Nam, Seung-Han Lee; Res Vestib Sci. 2019;18(2):50-53. Published online June 15, 2019; DOI: https://doi.org/10.21790/rvs.2019.18.2.50

Abstract PDF: 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.

Neuromyelitis Optica Spectrum Disorder presented with Upbeat Nystagmus and Intractable Vomiting: Hyunsoo Kim, Jae-Myung Kim, Tai-Seung Nam, Seung-Han Lee; Received May 20, 2019 Accepted June 10, 2019 Published online June 10, 2019; [Accepted]

Abstract: Neuromyelitis optica spectrum disorder(NMOSD) is an inflammatory demyelinating autoimmune disease of CNS 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 MRI, and she was diagnosed as the AP syndrome. Ten months later, she experienced a recurrence as a longitudinal 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.

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