Paroxysmal tonic upgaze of childhood: effect of age-of-onset on prognosis

Paroxysmal tonic upgaze (PTU) is a syndrome of childhood manifesting as sudden ocular movements with sustained upward deviation of the eyes. We describe the outcome of 6 patients, after a follow-up of 10 years, with onset of the disease in childhood. The aims of this study were to clarify some clinical features of this syndrome and to evaluate the long-term prognosis of these children. In all the patients, tonic upgaze episodes disappeared with time to remission, varying from 1 to 4 y, without any therapy and without any change in psychomotor development, EEG and neuroimaging. Only one child had pathologic interictal EEG with temporo-occipital spikes, which persisted after the offset of the disease. CONCLUSION: From a long-term follow-up, we can confirm the good prognosis of PTU and suggest it is possible to define a distinct syndrome of childhood without any neurological abnormalities and with spontaneous resolution.