ABSTRACT
When we first saw her at age 8 (see video, Case 70 , first segment), there was normal cognitive function for age. She could read well and follow complex commands. Eye movement examination was significant for slow, hypometric saccades. She used head thrusts to permit rapid re-fixation of vision. She had prominent saccadic pursuit. The remainder of the cranial nerve examination was unremarkable. Her speech was normal except for rare breaks associated with generalized chorea, which was of moderate severity and affected all limbs in addition to her trunk. Motor examination was otherwise unremarkable. She had generalized hyporeflexia with flexor plantar responses. There were no significant cerebellar signs. Gait was characterized by excessive arm movements, and tandem gait was mildly impaired apparently because of the chorea.
