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Case report

Effectiveness of intrathecal baclofen for intractable stiff-person syndrome: A case report

Bruce Zhang, Richard Lau, David Van Why, Michael Saulino
Physical Medicine and Rehabilitation, Temple University Hospital, Philadelphia, USA.
DOI: 10.2340/20030711-1000052

Abstract

Background: Intrathecal baclofen is considered an adjuvant therapy for patients with intractable spasms due to stiff-person syndrome. There is increasing evidence to support the use of intrathecal baclofen in the management of symptomatic stiff-person syndrome, with improvement in function.
Case report: A 38-year-old woman with stiff-person syndrome initially presented to inpatient rehabilitation for intractable muscle spasms. The symptoms made her non-ambulatory and limited her tolerance to wheelchair use for mobility. The patient underwent uptitration of oral baclofen and diazepam, with concurrent intravenous immunoglobulin cycles, leading to transient symptom relief. She agreed to explore intrathecal baclofen therapy. An initial trial of a single bolus of 50 μg intrathecal baclofen resulted in a significant decrease in spontaneous spasms, enabling modified independence in transfers and ambulation. The patient was subsequently implanted with a permanent intrathecal delivery system. To date, the intrathecal baclofen had been titrated to 186 μg per day with simple continuous delivery. The patient was weaned off oral baclofen. She attained complete functional independence with ambulation without the need for assistive devices, and has had no lasting post-procedural complications to date.
Conclusion: This case report adds to the increasing evidence of cases of refractory stiff-person syndrome managed successfully using intrathecal
baclofen therapy.

Lay Abstract

"Stiff-person-syndrome" is a rare disease that causes debilitating muscle spasms and pain. Patients often cannot walk as a result. The disease is believed to be autoimmune; for unclear reasons the body attacks a part of the central nervous system that normally works to calm the muscles. Sometimes medications that we normally take by mouth can be introduced into the human body directly through the central nervous system. Baclofen is one such medicine that can calm muscle spasms but often has intolerable side effects when taken by mouth. By introducing Baclofen in a controlled fashion directly into the central nervous system through a technique called "intrathecal delivery," we can offer "stiff-person-syndrome" patients symptom relief without affecting the rest of the body. In this report, we describe a 38 year old woman with "stiff-person-syndrome" that responded very well to intrathecal baclofen. She was able to walk without assistance after treatment.

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