Spasmus nutans. A benign clinical entity?

Spasmus nutans occurs in early childhood and consists of a triad of symptoms as follows: small-amplitude, rapid, horizontal nystagmus in one or both eyes asymmetrically; head nodding; and anomalous head position. Once thought to be a benign clinical entity, there are an increasing number of reports linking spasmus nutans to optic nerve and chiasmal gliomas. We describe 14 patients with spasmus nutans, none of whom were found to have a tumor with computed tomographic scanning. One patient, however, had an arachnoid cyst and an empty sella and another patient had a porencephalic cyst. The diagnosis of spasmus nutans, its implications, and its management are discussed.