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Bow-tie nystagmus is a rare phenomenon, which is reportedly associated with cerebellar infarction, brain stem anomalies, and so on. We analyzed herein bow-tie nystagmus in a patient with an Arnold-Chiari malformation using a three-dimensional 240 Hz high speed video oculography (VOG) system. The patient's nystagmus consisted of two aspects slow phases directed upward and quick phases altering the direction obliquely right to left downward in turn (square wave jerks). The dominant frequency of the vertical component was 3.1 Hz, which was exactly twice as fast as that of the horizontal component at 1.55 Hz.

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