The effect of cervical and vestibular reflexes on eye movements in Huntington's chorea

In 8 patients with manifest Huntington's Chorea vestibulo-ocular (VOR) and cervico-ocular (COR) reflexes were compared with eye movements during active head turnings. Seated patients were stimulated with their eyes closed by sinusoidal swings around the vertical axis at frequencies of 0.05, 0.1 and 0.2s-1 with amplitudes of 20, 40 and 60 degrees. 1) With all stimuli and in all patients a weak nystagmus was elicited in the direction of head movements, superimposed on larger slow eye deviations. 2) The averaged total saccadic amplitudes were smaller than in normals, increased with stimulus amplitudes and were smallest for COR, followed by VOR and active head movements. 3) The gain (peak velocity of slow phase of nystagmus to peak stimulus velocity) was only slightly below norm values and decreased with increasing stimulus frequency and amplitude. 4) The peak amplitudes of average slow eye deviations increased with stimulus amplitudes. In VOR they were comparable to norm values but were below them during COR and active head movements. 5) In normal subjects these slow eye deviations were compensatory to head movements in VOR but anticompensatory in COR and during active head movements. In choreic patients during COR and more often during active head movements these slow eye movements were compensatory for the head turning.