Intrathecal Baclofen Therapy for the Treatment of Spasticity in Sjögren-Larsson Syndrome. (January 2017)

• Record Type: Journal Article
• Authors: Hidalgo, Eveline Teresa; Orillac, Cordelia; Hersh, Andrew; Harter, David H.; Rizzo, William B.; Weiner, Howard L.
• Is Part Of: Journal of child neurology. Volume 32:Number 1(2017:Jan.)
• Page Start: 100
• Page End: 103
• Publication Date: 2017-01
• Subjects: Sjögren-Larsson syndrome -- intrathecal baclofen therapy -- spasticity -- baclofen pump -- Modified Ashworth Scale; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073816671440
• Languages: English
• ISSNs: 0883-0738

Abstract

Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjögren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjögren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients. One patient experienced skin irritation 15 months after surgery, necessitating pump repositioning. No infection occurred. Our report shows that intrathecal baclofen therapy can have a positive therapeutic effect on spasticity in patients with Sjögren-Larsson syndrome, and therefore may be a promising addition to current treatments.