Spasmus nutans-like nystagmus is often associated with underlying ocular, intracranial, or systemic abnormalities

There is uncertainty as to whether spasmus nutans (SN) is an isolated idiopathic entity or whether there are underlying conditions that could cause or be associated with the nystagmus. We undertook this study to determine the frequency of ocular, intracranial, and systemic conditions in patients with nystagmus having characteristics of SN.

We performed a chart review of 22 consecutive patients examined from 2000 through 2005 at the Dean McGee Eye Institute and Children' Hospital of Oklahoma with nystagmus consistent with SN. We collected information related to gender, age at presentation and age at final visit, visual acuity, refractive error, laterality of nystagmus, presence of head nodding and torticollis, pattern of strabismus, neuroimaging and electroretinography results, and other associated clinical findings.

Visual acuity was reduced in 75% of eyes at presentation and 58% of eyes at last visit. Eight patients had significant refractive error. Seven patients had strabismus. Two patients had chiasmal gliomas. Four patients had cone or rod/cone dystrophy. Only three patients had no associated ocular, intracranial, or systemic conditions.

A substantial proportion of patients presenting with SN-like nystagmus have important underlying ocular, intracranial, or systemic abnormalities that may require evaluation and management.