Clinical findings as well as eye and head movement recordings were analyzed from 23 patients with spasmus nutans without central nervous system (CNS) changes, 10 patients with spasmus nutans-like disease (head nodding, intermittent nystagmus associated with intracranial anomalies or visual pathway disorders), and 25 patients with infantile nystagmus. Ten diagnostic signs were established to differentiate between the patient groups. Although they were helpful in separating patients with infantile nystagmus from those with spasmus nutans, no difference was found between the patients with spasmus nutans with and without CNS lesions. This study indicates that eye and head movement recordings do not allow differentiation between benign spasmus nutans and spasmus nutans-like disease. The differentiation must be made on the basis of neuroimaging.