A case of paroxysmal tonic upward gaze associated with psychomotor retardation

The authors report a female with paroxysmal tonic upward gaze similar to the cases reported by Ouvrier and Billson (1988). She developed abnormal eye-movements at the age of six months. Several anticonvulsants and levodopa were not effective in the control of the episodes; however, her upward eye-deviations spontaneously decreased during the one-year observation. Her clinical features were almost identical to those reported by Ouvrier and Billson, except that she had neurodevelopmental disorder and abnormal brain MRI. More data are required to clarify this unique phenomenon.