Walsh & Hoyt: Other Diseases of Basal Ganglia

A number of conditions other than Parkinsons and Huntingtons that affect the basal ganglia cause abnormal eye movements. Hepatolenticular degeneration (Wilsons disease) and juvenile dystonic lipidosis (Niemann-Pick type 2s) are discussed below. Other conditions include caudate hemorrhage, which has been associated with ipsilateral gaze preference and, rarely, Sydenhams chorea. Vermersch et al. recorded saccadic eye movements in patients with bilateral lesions in the lentiform nucleus and found prominent abnormalities in saccades that required an internal representation of the target (remembered saccades, saccades to sequences, predictive saccades) but normal responses for visually guided saccades, including antisaccades (which are nonetheless triggered by a visual target). Lekwuwa et al. suggested that defects in control of predictive pursuit eye movements are a feature of striatal damage.