This Video Shows Patient 2 after the Treatment

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The examination was performed at age 40, 2 years after the onset of ataxia. She was on azathioprine 150 mg/day and a tapering dose of oral prednisone. The cerebellar features in this patient were overall more prominent than in patient 1. Her speech was moderately slurred and scanning. Eye movement examination demonstrated mild square wave jerks. Pursuits were normal. There were hypermetric saccades, more prominent in horizontal than vertical directions. There was no slowing of saccades or limitation of eye movements in any direction. Mild dystonic posturing of both hands, right slightly greater than left, was demonstrated. Finger-to-nose-to-finger test revealed mild dysmetria, left greater than right. Finger following test demonstrated mild-tomoderate overshoot dysmetria, left greater than right. Mild-to-moderate dysdiadochokinesia, left greater than right, was also demonstrated. She was able to walk independently, but had wide-based gait. She was unable to stand with both feet together without assistance, and had marked difficulty standing on only one foot.

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