Case Series: Intrathecal Baclofen Therapy in Stiff-Person Syndrome

Area of Science:

• Neurology
• Neurosurgery
• Rehabilitation Medicine

Background:

• Stiff-person syndrome (SPS) causes axial rigidity and muscle spasms, significantly impacting daily activities, particularly ambulation.
• Medically refractory spasticity is a hallmark of SPS, posing challenges for patient management.

Purpose of the Study:

• To evaluate the efficacy and outcomes of intrathecal baclofen (ITB) infusion for managing spasticity in patients with stiff-person syndrome.
• To assess the impact of ITB on pain, spasm frequency, and ambulation in SPS patients.

Main Methods:

• Retrospective case series of nine patients diagnosed with SPS treated in a spasticity clinic.
• Included patients with medically refractory spasticity who underwent ITB screening and/or chronic infusion.
• Outcome measures included pain, spasm frequency, lower extremity spasticity (Modified Ashworth Scale), and walking performance.

Main Results:

• Seven of nine patients received chronic ITB infusion, showing improved spasticity scores (MAS) at early and late follow-up.
• Five patients reported reduced pain, and ambulatory patients maintained stable walking performance.
• Four patients experienced manageable complications related to ITB implantation.

Conclusions:

• Intrathecal baclofen (ITB) infusion is an effective treatment for medically intractable spasticity in stiff-person syndrome.
• ITB therapy can lead to significant symptom reduction, including decreased spasticity and pain, without compromising ambulation.