Paroxysmal tonic upgaze in normal children: A case series and a review of the literature

The purpose of this review was to update the clinical characteristics of paroxysmal tonic upgaze in neurodevelopmentally normal children. We made the diagnosis (between 2008 and 2010) in 8 infants referred to us with suspected epilepsy. We found 38 further cases in the literature.In the 46 children (29 boys and 17 girls) tonic upward ocular deviation was first noticed between the age of 2 weeks and 90 months (median: 9 months). This tendency persisted for between 1 and 48 months (median: 7 months). The duration of paroxysmal events was highly variable: brief events lasted between 3 s and 10 min in 50% of the cases, intermediate events between 5 s and 30 min and long events between 10 s and 2 h. The frequency ranged from one every 3 months to 10 per day. In 15 children the episodes of upward deviation of the eyes were associated with an impaired movement coordination. In 2 further children an impaired movement coordination was noted during febrile illnesses.The results of this review in normal children characterize the entity as follows: onset under 2 years of age, a small predilection for the male gender, eventual improvement and recovery, and impaired movement coordination.