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Case Reports in Neurological Medicine
Volume 2017, Article ID 2580620, 3 pages
Case Report

Difficult to Treat Focal, Stiff Person Syndrome of the Left Upper Extremity

1University of Central Florida College of Medicine, 6850 Lake Nona Boulevard, Orlando, FL 32827, USA
2Department of Internal Medicine, Orlando VA Medical Center, 13800 Veterans Way, Orlando, FL 32827, USA

Correspondence should be addressed to Nathan E. Esplin; moc.liamg@nilpsen

Received 1 July 2017; Revised 17 September 2017; Accepted 12 October 2017; Published 25 October 2017

Academic Editor: Pablo Mir

Copyright © 2017 Nathan E. Esplin et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Background. Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle rigidity. It is a disorder of reduced GABA activity leading to increased muscle tone and often painful spasms. It generally presents in the axial musculature but rarely can involve only one limb, typically a lower extremity. In rare cases it can be paraneoplastic which generally resolves on treatment of the underlying neoplasm. Case Report. A 46-year-old male with a history of Hodgkin’s Lymphoma in remission presented with left upper extremity pain secondary to a diagnosis of Stiff Person Syndrome limited to his left upper extremity. He had previously benefitted from plasmapheresis and was on diazepam and baclofen at home with relatively good control of his symptoms. SPS had previously been diagnosed with EMG and anti-GAD-65 antibody titers and was confirmed by an elevated anti-GAD-65 antibody titer. He was treated with plasmapheresis and maximum doses of medical treatment including botulinum toxin with only transient mild improvement in his symptoms. Conclusion. This case represents a case of a rare disease that was refractory to all known therapies. It outlines the need for further understanding of this disorder in order to provide better symptomatic treatment or potentially more definitive care.