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Case Reports
. 2012 May;4(2):92-6.
doi: 10.1159/000339446. Epub 2012 May 31.

Childhood stiff-person syndrome improved with rituximab

R Fekete  1 , J Jankovic
Affiliations
Case Reports

Childhood stiff-person syndrome improved with rituximab

R Fekete et al. Case Rep Neurol. 2012 May.

Abstract

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.

Keywords: Myoclonus; Rituximab; Stiff-person syndrome.

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