Supraspinal baclofen for the treatment of secondary generalized dystonia: A case series

The aim of the study was to evaluate the use of a novel technique for baclofen delivery using an intrathecal catheter inserted through a lumbar laminotomy with the tip placed at the cisterna magna (supraspinal baclofen (SSB)) for the treatment of severe generalized secondary dystonia.A cohort study of six individuals (4M/2F, mean±SD = 15±4.86 years) with generalized dystonia unresponsive to oral medications were treated with SSB and followed clinically for 9 years. Intrathecal catheter tips were positioned under fluoroscopic guidance just above the level of the foramen magnum, at the cisterna magna.Five of the 6 patients experienced sustained benefit with SSB; the group mean modified Fahn-Marsden scale scores decreased from 95 to 55 (t = 3.29, p = 0.02). One patient suffered complex pump pocket infection, and therefore underwent pump explantation.Supraspinal baclofen may be an effective method for infusing baclofen into the ventricular system of the brain for treatment of secondary dystonia which is unresponsive to oral therapy.