Vertical infantile nystagmus: Explanation of why the direction of nystagmus is vertical

Abstract

This case report presents a rare case of infantile nystagmus syndrome (INS) in which the direction of infantile nystagmus (IN) was vertical. A 66-year-old woman was referred to our department for investigation of abnormal eye movements. She showed a disordered field of view with a homonymous hemianopia in the lower left quadrant and vertical gaze-evoked nystagmus, but there were no other abnormal neurological findings. She did not complain of an oscillopsia. Imaging revealed that the cause of hemianopia was atrophy and low cerebral blood flow in the right occipital lobe. The vertical nystagmus became strong when attempting to fixate to stationary targets. A reversed optokinetic nystagmus response was observed in the vertical optokinetic nystagmus test. From these eye movements, we diagnosed her nystagmus as vertical IN.

Patients with INS see everything by saccades. IN consists of the alternate appearance of saccades and preceding slow eye movements. For these eye movements, a wide visual field is necessary. In this case, vertical IN was caused by the wider vertical than horizontal visual field resulting from homonymous hemianopia. Therefore, the direction of IN is horizontal in most patients with INS because their horizontal visual field is the widest field.