Disclosures: Bruce Zhang, MD: Nothing to disclose

Case Description: A 38-year-old woman with past medical history of type I diabetes mellitus, Stiff-Person-Syndrome (confirmed with serum anti-glutamic acid decarboxylase antibodies) presented to acute rehabilitation for intractable painful muscle spasms causing significant ADL dysfunction related to her Stiff-Person-Syndrome. Her exam was notable for severe and painful muscle spasms diffusely, but particularly in her left lower extremity. Although symptoms were described as episodic in nature, they were triggered by light palpation during exam. The patient had multiple acute care admissions for episodes related to her condition and was treated with serial up-titration of oral baclofen and diazepam as well as cycles of IVIG with only transient symptom control. Her spontaneous spasms made her non-ambulatory and limited her to wheelchair tolerance.

Setting: Tertiary care rehabilitation center.

Patient: 38-year-old woman with Stiff-Person-Syndrome.

Assessment/Results: Following up-titration of PO diazepam to 20 mg Q6H and PO baclofen to 15 mg TID while in inpatient rehabilitation, the patient agreed to explore intrathecal baclofen therapy. She underwent a single bolus trial of 50 mcg via a lumbar puncture at the L3-L4 interspace. This resulted in a dramatic decrease in spontaneous spasms for several hours. She demonstrated modified independence on transfers and ambulation. The patient subsequently was implanted with an intrathecal delivery system. Her intrathecal baclofen was eventually titrated to 100 mcg per day with simple continuous delivery. She was capable of being weaned completely off her oral baclofen and diazepam. She attained complete functional independence with ambulation without the need for assistive devices. Her course was complicated only by a transient post-lumbar puncture headache after implantation.

Discussion: The patient demonstrated noticeable improvement in symptoms related to Stiff-Person Syndrome after intrathecal baclofen pump placement and was able to wean her oral medications.

Conclusion: Intrathecal baclofen can be a viable alternative treatment option for symptomatic control of refractory Stiff-Person-Syndrome cases.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/intrathecal-baclofen-as-effective-therapy-for-intractable-stiff-person-syndrome-with-associated-dramatic-functional-improvement-a-case-report/