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Chapter 20 - Treatment of Stiff-Person Syndrome with Botulinum Toxin

Published online by Cambridge University Press:  02 November 2023

By
Delaram Safarpour  and
Bahman Jabbari
Edited by
Daniel Truong ,
Dirk Dressler ,
Mark Hallett ,
Christopher Zachary  and
Mayank Pathak
Daniel Truong
Affiliation:
University of California, Riverside
Dirk Dressler
Affiliation:
Hannover Medical School
Mark Hallett
Affiliation:
National Institutes of Health (NIH)
Christopher Zachary
Affiliation:
University of California, Irvine
Mayank Pathak
Affiliation:
Truong Neuroscience Institute
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Summary

Stiff-person syndrome (SPS), formerly termed stiff-man syndrome, is characterized by muscular rigidity and episodic spasms mainly involving the trunk and lower limbs. The typical form is an autoimmune disease characterized by progressive axial rigidity, predominantly involving the paraspinal and abdominal muscles, along with hyperlordosis of the lumbar spine, spontaneous or stimulus sensitive disabling muscle spasms of the abdominal wall, lower extremities and other proximal muscles. Ninety-five percent of patients had GAD-65 and 89% had islet cell antibodies (ICA). A sizeable number of patients also have other antibodies such as those against glycine receptor and glycine transporter 2. SPS associated with neoplasms tends to involve the upper limbs and neck and cranial nerves. Patients with carcinoma of the breast or lung (oat cell carcinoma) may develop SLS with high titers of anti-amphiphysin antibodies. This chapter illustrates the use of botulinum toxin (BoNT) in the treatment of SPS, along with anatomical illustrations demonstrating the typical involved musculature, and approach to injection with botulinum neurotoxin, with tabulated dosing recommendations for the various BoNT formulations.

Keywords

Stiff personstiff manstiff limbbotulinum toxinBoNTmuscle rigidityGAD antibody
Type
Chapter
Information
Manual of Botulinum Toxin Therapy , pp. 174 - 179
Publisher: Cambridge University Press
Print publication year: 2023

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References

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