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Variants of windmill nystagmus

Abstract

Windmill nystagmus is characterized by a clock-like rotation of the beating direction of a jerk nystagmus suggesting separate horizontal and vertical oscillators, usually 90° out of phase. We report oculographic characteristics in three patients with variants of windmill nystagmus in whom the common denominator was profound visual loss due to retinal diseases. Two patients showed a clock-like pattern, while in the third, the nystagmus was largely diagonal (in phase or 180° out of phase) but also periodically changed direction by 180°. We hypothesize that windmill nystagmus is a unique manifestation of “eye movements of the blind.” It emerges when the central structures, including the cerebellum, that normally keep eye movements calibrated and gaze steady can no longer perform their task, because they are deprived of the retinal image motion that signals a need for adaptive recalibration.

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Author information

Correspondence to Ji-Soo Kim.

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Video 1. A cycle of windmill nystagmus in patient 1. The nystagmus initially beats downward, and then completes a clockwise cycle (from the patient’s perspective) of beating directions over about 40 s (WMV 6541 kb)

Video 2. A cycle of windmill nystagmus in patient 2. The nystagmus initially beats right downward, and then completes a counterclockwise cycle (from the patient’s perspective) of beating directions over about 35 s. Intermittent small counterclockwise torsional nystagmus (the upper poles of the eyes beating to the left ear) is also observed (WMV 35696 kb)

Video 1. A cycle of windmill nystagmus in patient 1. The nystagmus initially beats downward, and then completes a clockwise cycle (from the patient’s perspective) of beating directions over about 40 s (WMV 6541 kb)

Video 2. A cycle of windmill nystagmus in patient 2. The nystagmus initially beats right downward, and then completes a counterclockwise cycle (from the patient’s perspective) of beating directions over about 35 s. Intermittent small counterclockwise torsional nystagmus (the upper poles of the eyes beating to the left ear) is also observed (WMV 35696 kb)

Video 3. A cycle of periodically reversing windmill nystagmus in patient 3. One half-cycle of the nystagmus consists of reversing diagonal nystagmus beating left upward and then right downward, while the other half-cycle includes reversing diagonal nystagmus beating right upward, and then left downward. The right-beating diagonal nystagmus is associated with small clockwise torsional nystagmus while left-beating diagonal nystagmus is accompanied by counterclockwise torsional nystagmus (WMV 20654 kb)

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Choi, K., Shin, H.K., Kim, J. et al. Variants of windmill nystagmus. J Neurol 263, 1375–1381 (2016) doi:10.1007/s00415-016-8152-x

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Keywords

  • Windmill nystagmus
  • Periodic alternating nystagmus
  • Blindness
  • Velocity-storage mechanism
  • Gaze stabilizing mechanism