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Utility of Botulinum Injections in Stiff-Person Syndrome

Stiff-person syndrome (SPS) is an uncommon neurological disorder characterized by significant rigidity and muscle spasms primarily affecting the truncal and proximal musculature. Furthermore, a wide-based gait with functional impairment is generally seen. High-dose benzodiazepines or baclofen are wi...

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Autores principales: Conners, L. M., Betcher, A., Shahinian, A., Janda, P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6800926/
https://www.ncbi.nlm.nih.gov/pubmed/31687237
http://dx.doi.org/10.1155/2019/9317916
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author Conners, L. M.
Betcher, A.
Shahinian, A.
Janda, P.
author_facet Conners, L. M.
Betcher, A.
Shahinian, A.
Janda, P.
author_sort Conners, L. M.
collection PubMed
description Stiff-person syndrome (SPS) is an uncommon neurological disorder characterized by significant rigidity and muscle spasms primarily affecting the truncal and proximal musculature. Furthermore, a wide-based gait with functional impairment is generally seen. High-dose benzodiazepines or baclofen are widely considered the optimal initial therapy; however, major adverse effects often preclude adequate dosing. Refractory cases may be treated with intravenous immunoglobulins (IVIG), plasma exchange, or B-cell depletion with rituximab, although these are also associated with major, sometimes fatal, adverse reactions. Several reports have validated the safety and utility of botulinum injections in this setting, yet botulinum remains markedly underutilized in this cohort. Below, a case report and review of the literature show botulinum can decrease pain and stiffness, improve gait and balance, and decrease dependence on powerful systemic treatments in this group.
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spelling pubmed-68009262019-11-04 Utility of Botulinum Injections in Stiff-Person Syndrome Conners, L. M. Betcher, A. Shahinian, A. Janda, P. Case Rep Neurol Med Case Report Stiff-person syndrome (SPS) is an uncommon neurological disorder characterized by significant rigidity and muscle spasms primarily affecting the truncal and proximal musculature. Furthermore, a wide-based gait with functional impairment is generally seen. High-dose benzodiazepines or baclofen are widely considered the optimal initial therapy; however, major adverse effects often preclude adequate dosing. Refractory cases may be treated with intravenous immunoglobulins (IVIG), plasma exchange, or B-cell depletion with rituximab, although these are also associated with major, sometimes fatal, adverse reactions. Several reports have validated the safety and utility of botulinum injections in this setting, yet botulinum remains markedly underutilized in this cohort. Below, a case report and review of the literature show botulinum can decrease pain and stiffness, improve gait and balance, and decrease dependence on powerful systemic treatments in this group. Hindawi 2019-10-07 /pmc/articles/PMC6800926/ /pubmed/31687237 http://dx.doi.org/10.1155/2019/9317916 Text en Copyright © 2019 L. M. Conners et al. https://creativecommons.org/licenses/by/4.0/ 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.
spellingShingle Case Report
Conners, L. M.
Betcher, A.
Shahinian, A.
Janda, P.
Utility of Botulinum Injections in Stiff-Person Syndrome
title Utility of Botulinum Injections in Stiff-Person Syndrome
title_full Utility of Botulinum Injections in Stiff-Person Syndrome
title_fullStr Utility of Botulinum Injections in Stiff-Person Syndrome
title_full_unstemmed Utility of Botulinum Injections in Stiff-Person Syndrome
title_short Utility of Botulinum Injections in Stiff-Person Syndrome
title_sort utility of botulinum injections in stiff-person syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6800926/
https://www.ncbi.nlm.nih.gov/pubmed/31687237
http://dx.doi.org/10.1155/2019/9317916
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