Childhood Stiff-Person Syndrome Improved with Rituximab

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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Bibliographic Details
Main Authors: R. Fekete, J. Jankovic
Format: Article
Language:English
Published: Karger Publishers 2012-05-01
Series:Case Reports in Neurology
Subjects:
Rituximab
Stiff-person syndrome
Myoclonus
Online Access:http://www.karger.com/Article/FullText/339446
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spelling doaj-7a0c70b9e39e4537bd92a58abc9742122020-11-25T00:27:39ZengKarger PublishersCase Reports in Neurology1662-680X2012-05-0142929610.1159/000339446339446Childhood Stiff-Person Syndrome Improved with RituximabR. FeketeJ. JankovicIntroduction: 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.http://www.karger.com/Article/FullText/339446RituximabStiff-person syndromeMyoclonus
collection DOAJ
language English
format Article
sources DOAJ
author R. Fekete
J. Jankovic
spellingShingle R. Fekete
J. Jankovic
Childhood Stiff-Person Syndrome Improved with Rituximab
Case Reports in Neurology
Rituximab
Stiff-person syndrome
Myoclonus
author_facet R. Fekete
J. Jankovic
author_sort R. Fekete
title Childhood Stiff-Person Syndrome Improved with Rituximab
title_short 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_sort childhood stiff-person syndrome improved with rituximab
publisher Karger Publishers
series Case Reports in Neurology
issn 1662-680X
publishDate 2012-05-01
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.
topic Rituximab
Stiff-person syndrome
Myoclonus
url http://www.karger.com/Article/FullText/339446
work_keys_str_mv AT rfekete childhoodstiffpersonsyndromeimprovedwithrituximab
AT jjankovic childhoodstiffpersonsyndromeimprovedwithrituximab
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