Paradoxical reaction to increased doses of intrathecal baclofen in a patient with Leigh syndrome.

In clinical practice, intrathecal baclofen (ITB) therapy is used to control spasticity. After initial placement of the ITB pump, clinicians incrementally increase the dose until effectiveness in alleviating spasms and spasticity is optimized. However, this case describes a 4-year-old male with Leigh syndrome who developed a paradoxical worsening of spasticity and pain with incremental increase of his ITB pump. In this rare genetic disease with a poor prognosis, an ITB pump was trialed and implanted and titrated upwards with initial improvement. However, his spasticity and pain then began to worsen with each dosage increase. Subsequently, his symptoms improved significantly when the dose was weaned. This is the first case that describes this paradoxical reaction in a pediatric population and discusses recommendations about how clinicians should safely titrate the pump for patient care.