Six children who developed paroxysmal tonic upgaze (PTU) at 2.6 to 7.4 years of age were examined at least once per year for a 10 year period of follow-up at the Departments of Paediatrics and Ophthalmology, University of Chieti, Italy. Bouts of sudden sustained upward deviation of the eyes lasted for 10 to 20 seconds each, they appeared in small clusters, and were not associated with loss of consciousness. PTU was typically exacerbated by fatigue and relieved by sleep. Three patients were male and 3 female. Two had a febrile illness preceding the onset, 2 had a personal history of febrile seizures, 1 a father with a history of febrile seizures, and 1 a mother with migraine. One had an epileptiform interictal EEG, with temporo-occipital spikes, mainly in the left hemisphere. MRI, neurologic, and psychological examinations, including WISC-R or Stanford-Binet Intelligence Scales were normal. Episodes of PTU resolved spontaneously after 1 to 4 years, without treatment. 
COMMENT. The first description of the syndrome as “benign paroxysmal tonic upgaze of childhood” was reported in 4 patients by Ouvrier RA and Billson F . Some subsequent reports have included associated neurologic abnormalities, including ataxia (Deonna T et al. 1990), developmental delay, and abnormal brain MRI. Improvement following levodopa therapy in one child suggested a possible dystonia (see Progress in Pediatric Neurology II, PNB Publ, 1994;pl46). Most patients have an early spontaneous resolution of the disorder. The authors suggest that the outcome may be age-dependent, their older patients having a more benign form of the syndrome than that described in younger infants. An immature cortico-mesencephalic control of vertical gaze is postulated as the pathophysiology.