Intrathecal Baclofen Therapy for the Treatment of Spasticity in Sjögren-Larsson Syndrome

Eveline Teresa Hidalgo, Cordelia Orillac, Andrew Hersh, David H. Harter, William B. Rizzo, Howard L. Weiner

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)100-103
Number of pages4
JournalJournal of Child Neurology
Issue number1
StatePublished - Jan 1 2017


  • Modified Ashworth Scale
  • Sjögren-Larsson syndrome
  • baclofen pump
  • intrathecal baclofen therapy
  • spasticity

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology


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