Wallerian Degeneration of the Superior Cerebellar Peduncle

mg at days 0 and 14).She continued to experience worsening lower extremity weakness.Eventually, she received 6 plasmapheresis treatments with minimal improvement.During the entire period of follow-up at our center, she required 20 to 30 mg daily of oral prednisone.Neurological examination prior to the onset of HiCy therapy revealed symmetrically reduced arm abduction (4-/5) and hip flexion strength (2/5) and her CK level was 2920 U/L.Given progressive muscle weakness in the absence of a robust response to any immunosuppression, she was treated with HiCy, 50 mg/kg per day, for 4 consecutive days and supportive care, as previously described. 2Although she developed neutropenic fever 9 days later, she recovered successfully.She did not require red blood cell or platelet transfusion, and her neutropenia ultimately resolved 2 weeks after HiCy dosing.Muscle strength gradually improved to normal, and her CK level decreased to 537 U/L within 7 months of treatment.A repeated magnetic resonance image was performed 21 months after treatment (Figure ,B).Her steroids were tapered off within 2 years of HiCy therapy.At her last visit, she had minimal residual weakness (4+/5 deltoids and hip flexors), which did not affect her activities of daily living.She has remained in clinical remission while not taking any immunosuppressive therapies, including glucocorticoids, with her most recent CK level normal at 100 U/L 6 years after HiCy treatment.