Childhood Stiff-Person Syndrome Improved with Rituximab

INTRODUCTION: Stiff-person syndrome (SPS) is manifested by fluctuating rigidity of axial musculature with painful episodic spasms due to simultaneous co-contraction of agonist and antagonist muscles. We present a case report and video illustrating response to treatment with rituximab. MATERIALS AND...

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Detalles Bibliográficos
Autores principales: Fekete, R., Jankovic, J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2012
Materias:
Published online: May, 2012
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3383307/
https://www.ncbi.nlm.nih.gov/pubmed/22740823
http://dx.doi.org/10.1159/000339446
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author Fekete, R.
Jankovic, J.
author_facet Fekete, R.
Jankovic, J.
author_sort Fekete, R.
collection PubMed
description INTRODUCTION: Stiff-person syndrome (SPS) is manifested by fluctuating rigidity of axial musculature with painful episodic spasms due to simultaneous co-contraction of agonist and antagonist muscles. We present a case report and video illustrating response to treatment with rituximab. MATERIALS AND METHODS: Case description and video are provided. A literature search for other reports of treatment with rituximab was performed. RESULTS: Nine cases in addition to our case were described. Substantial clinical benefit was reported in 7/9 (78%) cases. Four out of 9 (44%) cases displayed persistent anti-glutamic acid decarboxylase (GAD) antibody positivity. CONCLUSION: Rituximab is an important treatment strategy in SPS. The persistence of anti-GAD antibody positivity even with clinical remission remains to be elucidated.
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spelling pubmed-33833072012-06-27 Childhood Stiff-Person Syndrome Improved with Rituximab Fekete, R. Jankovic, J. Case Rep Neurol Published online: May, 2012 INTRODUCTION: Stiff-person syndrome (SPS) is manifested by fluctuating rigidity of axial musculature with painful episodic spasms due to simultaneous co-contraction of agonist and antagonist muscles. We present a case report and video illustrating response to treatment with rituximab. MATERIALS AND METHODS: Case description and video are provided. A literature search for other reports of treatment with rituximab was performed. RESULTS: Nine cases in addition to our case were described. Substantial clinical benefit was reported in 7/9 (78%) cases. Four out of 9 (44%) cases displayed persistent anti-glutamic acid decarboxylase (GAD) antibody positivity. CONCLUSION: Rituximab is an important treatment strategy in SPS. The persistence of anti-GAD antibody positivity even with clinical remission remains to be elucidated. S. Karger AG 2012-05-31 /pmc/articles/PMC3383307/ /pubmed/22740823 http://dx.doi.org/10.1159/000339446 Text en Copyright © 2012 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.
spellingShingle Published online: May, 2012
Fekete, R.
Jankovic, J.
Childhood Stiff-Person Syndrome Improved with Rituximab
title Childhood Stiff-Person Syndrome Improved with Rituximab
title_full Childhood Stiff-Person Syndrome Improved with Rituximab
title_fullStr Childhood Stiff-Person Syndrome Improved with Rituximab
title_full_unstemmed Childhood Stiff-Person Syndrome Improved with Rituximab
title_short Childhood Stiff-Person Syndrome Improved with Rituximab
title_sort childhood stiff-person syndrome improved with rituximab
topic Published online: May, 2012
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3383307/
https://www.ncbi.nlm.nih.gov/pubmed/22740823
http://dx.doi.org/10.1159/000339446
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