Inflammatory central nervous system (CNS) demyelinating diseases are a group of disorders in which inflammation within the CNS damages the myelin sheath, a protective and conductive layer surrounding nerve fiber in the brain and spinal cord, leading to various neurological symptoms. Common conditions include multiple sclerosis and neuromyelitis optica spectrum disorders, both characterized by relapses and progressive deterioration over time. Given their frequent involvement of the brainstem and cerebellum, these diseases often manifest with oculomotor findings, such as ocular misalignment and instability, providing valuable insights into lesion localization within specific anatomical regions. Recent studies suggest that these findings may also indicate broader pathological burdens, including potential cortical damage, positioning them as potential early biomarkers for disease progression. This review explores oculomotor findings in patients with CNS demyelinating diseases, examining their characteristics, underlying mechanisms, and clinical significance. It also highlights the potential role of these findings as disease biomarkers.
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.