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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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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1725338538193453056 |