Citation: wu j, ceverha b, yeh by, bedell kk.Supraspinal baclofen for the treatment of secondary generalized dystonia: a case series.J pediatr rehabil med.2022;15(1):145-150.10.3233/prm-210044 summary/abstract: purpose: 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.Methods: 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.Results: five of the 6 patients experienced sustained benefit withssb; 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.Conclusion: 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 reported events: the patient in case 1 underwent ssb pump placement in the last 3 weeks of a 4-month hospitalization for multiple concurrent medical complications related to their dystonia but unrelated to the ssb pump and was discharged to home on post-operative day 19 following completion of medical therapies.The post-operative course of the patient in case 6 was notable for initial tachycardia and hypertension which had self-resolved by post-operative day 5; they were discharged to home on post-operative day 8.The patient in case 1 died of an unrelated cause.At time of death in case 1, they continued to demonstrate benefit from ssb with clinically significant improvement in dystonia score compared to pre-operative score.The patient in case 6 previously had suboptimal response to high-dose intrathecal baclofen infusion with the catheter tip at t5 in addition to high-dose oral anti-spasticity medications.At the time of end-of-battery-life, the decision was made for intrathecal catheter tip reimplantation to a supraspinal location concurrent with pump replacement, and the patient did well.Interestingly, when the catheter tip was at t5, the patient was noted to have improvement in lower extremity spasticity symptoms and concurrent worsening of dystonia symptoms.Following reimplantation of the catheter tip for ssb infusion, they were seen with persistent good control of spasticity symptoms and improved control of dystonia symptoms thus resulting in a decrease in their oral anti-spasticity medications.At maximum therapeutic benefit, the patient was maintained at 586g/day ssb with scheduled tizanidine 8 mg/day.Unfortunately, they suffered both tip migration and pump pocket infection approximately 2 months following supraspinal catheter tip re-implantation and had multiple operations for wound washout and replacement of the programmable pump, which ultimately failed.The ensuing post-operative course was further complicated by wound dehiscence, and the pump was explanted.There was one patient who unfortunately had multiple complications which ultimately led to pump removal.At 2-months following reimplantation of the catheter tip above the foramen magnum, the patient in case 6 presented to hospital with baclofen withdrawal.They subsequently had multiple operations for replacement of the catheter tip, pump pocket wound washout, and pump replacement.See attached literature article.
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Please note that this date is based off of the date that the article was accepted for publication as the event dates were not p rovided in the published literature.Other relevant device(s) are: product id: 8780, serial/lot #: unknown, product type: catheter.Citation: wu j, ceverha b, yeh by, bedell kk.Supraspinal baclofen for the treatment of secondary generalized dystonia: a case series.J pediatr rehabil med.2022;15(1):145-150.10.3233/prm-210044.If information is provided in the future, a supplemental report will be issued.
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