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Richard W. Hertle, Dongsheng Yang, Frank Carusone, Stephanie Knox, Nancy Hanna; Head Oscillations And Infantile Nystagmus Syndrome (INS). Invest. Ophthalmol. Vis. Sci. 2012;53(14):517.
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Infantile nystagmus syndrome may be accompanied by head shaking which is more prominent during periods of intense emotion, concentration and visual fixation. This association has been poorly studied but is reported to occur in 10-28% of patients. The purpose of this study was to characterize these head oscillations in a large cohort of patients with INS and study the responsible neck musculature.
From 1999-2010 1,650 patients with INS, who had information collected as part of a prospective database, had statistical analysis of their clinical, electrophysiological and demographic data reviewed for the presence of a head oscillation and its associations. In 20 patients simultaneous head and eye oscillations were electrophysiological recorded and analyzed. In addition, the suboccipital triangle musculature was dissected and studied in cadavers.
29% had an associated head oscillation. There was no association with sex, waveform, eye or systemic conditions. Head recordings averaged, small amplitude (2-7 degrees), multiplanar, 1-4 Hz, variable, but similar, waveforms to the ongoing eye oscillation that were not compensatory to the eye oscillations. Cadaveric analysis showed a pathophysiological role of the subocciptal musculature in the genesis of the oscillation.
Head oscillations promote socially confusing cues in these patients. Almost uniformly, the head oscillations represent an associated involuntary movement of pathological origin and not an adaptive strategy to improve vision. The neck musculature responsible for these small amplitude, multiplanar movements are most likely those of the suboccipital triangle, as a result of this knowledge we are now investigating pharmacological therapy aimed at this musculature.
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