Childhood Stiff-Person Syndrome Improved with Rituximab
نویسندگان
چکیده
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.
منابع مشابه
Novel use of dual immunomodulation for treating stiff-person syndrome, cerebellar variant.
Stiff-person syndrome is an autoimmune syndrome defined by muscle spasms and rigidity of proximal and axial muscles. Glutamic acid decarboxylase is the autoantigen found in 60%–80% of stiff person syndrome patients. Stiff person syndrome, cerebellar variant was characterized in a subpopulation of patients (5 of 38) with stiff person syndrome and concomitant cerebellar dysfunction and confirmed ...
متن کاملTreatment of stiff person syndrome with rituximab.
This case report is about the novel use of the anti-CD20 antibody, rituximab, in the treatment of a 41 year old woman with stiff person syndrome. She was admitted to hospital as an emergency with prolonged and painful extensor spasms affecting the neck and back, arms, and legs. The disease had progressed despite a favourable initial response to conventional treatment with intravenous immunoglob...
متن کاملSuccessful treatment with rituximab in a patient with stiff-person syndrome complicated by dysthyroid ophthalmopathy.
We report a patient with stiff-person syndrome and insulin-dependent diabetes mellitus with anti-glutamic acid decarboxylase (GAD) antibody, who suddenly complained of diplopia due to dysthyroid ophthalmopathy. Therapeutic efficacy of plasmapheresis and high-dose intravenous immunoglobulin was transient. After starting administration of rituximab, the patient showed obvious improvement of muscl...
متن کاملLong-Lived Plasma Cells and Memory B Cells Produce Pathogenic Anti-GAD65 Autoantibodies in Stiff Person Syndrome
Stiff person syndrome (SPS) is a rare, neurological disorder characterized by sudden cramps and spasms. High titers of enzyme-inhibiting IgG autoantibodies against the 65 kD isoform of glutamic acid decarboxylase (GAD65) are a hallmark of SPS, implicating an autoimmune component in the pathology of the syndrome. Studying the B cell compartment and the anti-GAD65 B cell response in two monozygot...
متن کاملRecent Advances and Review on Treatment of Stiff Person Syndrome in Adults and Pediatric Patients
Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic vari...
متن کامل