The aim of this study was to evaluate the use of intraventricular baclofen (IVB) for the treatment of severe generalized secondary and heredodegenerative dystonia.
Nine children and 1 adult with severe dystonia unresponsive to multiple oral medications were treated with IVB. Intraventricular catheters were positioned endoscopically in the third ventricle.
Eight of the 10 patients responded to IVB; their mean dystonia scores on the Barry-Albright dystonia scale decreased from 23 to 8. The 2 patients who did not respond had not responded to previous high doses of intrathecal baclofen. No adverse side effects related to IVB occurred. One child developed a pump infection that required pump removal, 1 developed a CSF infection that cleared after antibiotic administration, and 1 developed ventriculomegaly that required a shunt placement.
Intraventricular baclofen is an effective method of infusing baclofen to treat severe, generalized secondary dystonia, and, at times, heredodegenerative dystonia. The site of baclofen's activity when treating dystonia may be at the cortical level, and intraventricular infusion may result in higher baclofen concentrations over the cortex than intrathecal infusion. Additional studies are necessary to determine whether IVB is effective at lower doses than those used with intrathecal baclofen administration.
Abbreviations used in this paper: BAD = Barry-Albright dystonia; CP = cerebral palsy; DBS = deep brain stimulation; ITB = intrathecal baclofen; IVB = intraventricular baclofen; VP = ventriculoperitoneal.
Address correspondence to: A. Leland Albright, M.D., Department of Neurosurgery, K4-836, University of Wisconsin Health Center, 600 Highland Avenue, Madison, Wisconsin 53792. email:
SiebnerHRDressnandtJAuerCConradB: Continuous intrathecal baclofen infusions induced a marked increase of the transcranially evoked silent period in a patient with generalized dystonia. Muscle Nerve21:1209–12121998
SiebnerHR, DressnandtJ, AuerC, ConradB: Continuous intrathecal baclofen infusions induced a marked increase of the transcranially evoked silent period in a patient with generalized dystonia. 21:1209–1212, 1998)| false