Functional recovery after bilateral pallidotomy for the treatment of early-onset primary generalized dystonia

This report describes the successful treatment of dystonia musculorum defor mans with bilateral stereotactic pallidotomy in a 14-year-old girl in whom the dystonia was diagnosed when she was 7 years old. The patient presented with dystonia of the right upper extremity that progressed to generalized d ystonia. Preoperatively, she required maximal assistance with all activitie s of daily living and transfers. She was not a functional ambulator. Postop eratively, she had remarkable functional recovery. At discharge, she was at modified independence level for all basic activities of daily Living and r equired supervision for household ambulation. No postoperative complication s were noted. We propose that bilateral stereotactic lysis of globus pallid us interna may be an alternative treatment for dystonia musculorum deforman s. The technique of bilateral pallidotomy and theories of its effectiveness are discussed. (C) 1999 by the American Congress of Rehabilitation Medicin e and the American Academy of Physical Medicine and Rehabilitation.