Purchase this article with an account.
Nechama Shoshani, Sylvia Kodsi, Steven E Rubin; Spasmus nutans: an analysis of systemic and ocular comorbidities.. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):5215.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
Spasmus nutans has often been described as a benign, self-limited disorder of childhood, yet case studies have reported systemic comorbidities in children presenting with this disorder. Previous studies have not evaluated the systemic comorbidities associated with the classic triad of nystagmus, head nodding and torticollis. We performed a retrospective chart review to evaluate whether this disorder is truly benign and self-limited.
We reviewed the charts of all pediatric patients who were diagnosed with nystagmus at the pediatric ophthalmology center of a tertiary care center during the years of 2004-2014. Inclusion criteria required that the patient present with nystagmus and head nodding and/or torticollis. 20 patients were identified. Patient characteristics, and information related to age, gender, vision, refraction, strabismus, amblyopia, neuroimaging, and ocular and systemic comorbidities were recorded.
Initial vision was found to be reduced in one or both eyes in 30% of patients, and final vision in 47%. 55% of patients had refractive errors requiring glasses wear. 50% had amblyopia, and 50% had strabismus. There were findings on brain MRI in 5 of 15 patients for whom MRI was available; these included one each of CNS tumor, poorly formed corpus callosum, and benign external hydrocephalus, and two others with benign external hydrocephalus vs. volume loss. Systemic disease was present in 50%; these children all had a systemic disorder that could account for their nystagmus. Developmental delay was present in 40%. Overall, 14 of the 20 children (70%) had ocular, neurologic, or systemic comorbidities aside from amblyopia and strabismus. We also found there to be no resolution of nystagmus in 8 of 14 patients (57%) with last visit at age 45 months or greater (mean age 85 months); however 63% of those patients with unresolved nystagmus did have resolution of their head nodding and torticollis.
In the majority of children presenting with the triad of spasmus nutans, the disorder does not appear to be an isolated entity, but carries serious visual and systemic implications. Given the number of patients with continuing nystagmus and underlying diagnoses, the description of spasmus nutans as a benign, self-limited entity may be inaccurate. We recommend that children presenting with features of spasmus nutans be evaluated carefully for underlying ocular, intracranial, and systemic abnormalities.
This PDF is available to Subscribers Only