This Video Shows Patient 1 after the Treatment
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The examination was performed at age 25, 4 years after the onset of ataxia. At that time, she was on prednisone 5 mg every other day. She had only very mildly slurred and scanning speech. Eye movement examination revealed no square wave jerks. There was no limitation of eye movements in any direction on both smooth pursuit and saccade examination. There were no apparent saccadic or broken pursuits. There was mild left beating gaze-evoked nystagmus on the extreme left gaze. Saccades were normal. There was neither slowing of saccades nor hypermetric saccade. Mild dysmetria and overshoot dysmetria, left greater than right, were demonstrated on finger-to-nose-to-finger and finger following tests, respectively. There was mild dysdiadochokinesia, which was more prominent on the left. She was able to walk independently, but her gait was wide-based. She had imbalance when standing with both feet together and eyes closed. She required assistance when standing on only one foot, either left or right.
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