Paroxysmal tonic upward gaze constitutes a neuro-ophthalmologic syndrome characterized by episodes of sustained conjugate upward deviation of the eyes, normal horizontal gaze, and ataxia. Its pathogenesis is unknown, and the etiology is heterogeneous. The prognosis is variable. We present a case of "idiopathic paroxysmal tonic upward gaze" with a positive clinical course and spontaneous remission.
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