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Effectiveness of Intrathecal Baclofen for Intractable Stiffperson Syndrome: a Case Report
Bruce Zhang1, Richard Lau1, David van Why2,3
1Department of Physical Medicine and Rehabilitation, Temple University Hospital, Philadelphia, PA, USA.
Journal of Rehabilitation Medicine. Clinical Communications
|April 22, 2021
Summary
Intrathecal baclofen therapy offers significant functional improvement for individuals with stiff-person syndrome. This treatment effectively manages intractable spasms, restoring mobility and independence.
Area of Science:
- Neurology
- Pharmacology
Background:
- Stiff-person syndrome (SPS) is a rare neurological disorder characterized by debilitating muscle rigidity and spasms.
- Intrathecal baclofen is an established adjuvant therapy for managing intractable spasticity in neurological conditions.
Observation:
- A 38-year-old female patient with severe SPS presented with non-ambulatory status due to intractable spasms.
- Initial management with oral baclofen, diazepam, and intravenous immunoglobulin provided only transient symptom relief.
Findings:
- A trial of intrathecal baclofen (50 μg bolus) significantly reduced spasms, enabling functional transfers and ambulation.
- Permanent intrathecal baclofen delivery, titrated to 186 μg/day, allowed discontinuation of oral medications and achieved full ambulation without assistive devices.
Implications:
- This case highlights the efficacy of intrathecal baclofen in refractory stiff-person syndrome, improving patient function and quality of life.
- Intrathecal baclofen therapy represents a viable and effective treatment option for severe, treatment-resistant SPS cases.
