Purpose: To determine if rituximab, an anti-CD20 monoclonal antibody, reduces cerebrospinal fluid (CSF) B-cell expansion in opsoclonus-myoclonus syndrome (OMS) and results in clinical improvement.
Methods: Sixteen children with OMS and increased % CD20 B-cells in CSF received 4 rituximab infusions (375 mg/m IV) as add-on therapy to corticotropin (ACTH), intravenous immunoglobulins, or both, and were reevaluated 6 months later. Outcome measures were clinical (motor function, behavior, sleep) and immunologic (CSF and blood immunophenotype and Ig levels). Controls were 16 age-matched and sex-matched children, who did not have OMS.
Results: After rituximab, 81% of OMS had a lower motor severity score, and 44% improved one severity category. Mean total score decreased by 44% (P = 0.0005). Rituximab reduced rage score, nighttime awakenings, and the number of children with opsoclonus, action myoclonus, drooling, gait ataxia, and rage. Despite a 51% reduction in ACTH dose, 9 of 11 children on ACTH did not relapse. The percentage of CSF CD19 (and CD20) B-cells was lowered in all children (undetectable in 6), with a 90% reduction in the group mean (P = 0.00003). CSF B-cells were no longer expanded compared with controls. In blood, CD19 B-cells decreased (-90%, P = 0.0003), as did the CSF:blood CD19 B-cell ratio (P = 0.00003). Serum IgM fell by 69% (below reference range), with no statistically significant change in IgG or IgA.
Conclusions: Rituximab seems efficacious and safe as adjunctive therapy for OMS. Selective targeting of CSF B lymphocytes represents a novel and valuable paradigm shift in the therapy for centrally mediated paraneoplastic disorders.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1097/01.mph.0000212991.64435.f0 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Amyotrophic lateral sclerosis in a patient with chronic lymphocytic leukaemia and drug related sarcoid-like reaction.
BMC Neurol
January 2025
Department of Neurology, Wessex Neurological Centre, University Hospital Southampton, Southampton, UK.
Article Synopsis
- Sarcoid-like reaction (SLR) is an immune response affecting lymph nodes and organs, which doesn't fulfill the criteria for systemic sarcoidosis, and can be associated with certain diseases like Chronic lymphocytic leukaemia (CLL) and Amyotrophic lateral sclerosis (ALS).
- A 60-year-old male patient with treated CLL developed ALS symptoms following exposure to Venetoclax and Rituximab, presenting with rashes and weakness that progressed over a year.
- Diagnosis complications included atypical signs and symptoms, leading to misdiagnosis of neurosarcoidosis and challenges in treatment, despite initial interventions with prednisolone and infliximab.
Successful Switch to Obinutuzumab in a Rituximab-Intolerant Child with Difficult-to-Treat Idiopathic Nephrotic Syndrome.
J Clin Med
January 2025
Department of Paediatrics, Nephrology and Hypertension, Medical University of Gdansk, 80-210 Gdansk, Poland.
: Idiopathic nephrotic syndrome (INS) is the most common cause of nephrotic syndrome in children. A hallmark of the disease is the rapid remission of proteinuria following a high dose of steroids. Recurrent disease or steroid dependence are common, leading to a high steroid burden and the introduction of steroid sparing therapy.
View Article and Find Full Text PDFAssessment of dysphagia and its associations in patients with secondary progressive multiple sclerosis.
Mult Scler Relat Disord
December 2024
Multiple Sclerosis Research Center, Neuroscience Institute, Tehran University of Medical Sciences, Sina Hospital, Hasan Abad Sq., Tehran, Iran. Electronic address:
In this cross-sectional study in patients with secondary progressive multiple sclerosis receiving anti-CD20 monoclonal antibodies, we assessed complaint of dysphagia and evaluated their association with sex, age, disease duration, disability severity, and disease-modifying therapies (DMTs). The validated Persian version of Dysphagia in Multiple Sclerosis (DYMUS) questionnaire was used. A total of 66 patients were included.
View Article and Find Full Text PDFFrom Fibrosis to Granuloma: Drug Induced Systemic Sarcoidosis-Like Reaction After Rituximab in a Patient with Primary Sjögren's Syndrome.
Eur J Case Rep Intern Med
December 2024
Clínica de Medicina, Serviço de Medicina Interna, Centro Hospitalar Universitário de Santo António, Porto, Portugal.
Unlabelled: Sarcoidosis is a multisystemic syndrome characterized by non-caseous granulomatous inflammation, although necrotizing sarcoid granulomatosis is considered part of the spectrum of the disease. Drug induced sarcoidosis-like reaction (DISR) is a systemic granulomatous reaction, which is histopathologically identical to primary sarcoidosis - mostly described after the use of biologics like tumour necrosis factor alpha antagonists but also anti-CD20 (rituximab). The authors present the very rare case of a woman with a primary Sjögren's syndrome (pSS) started on rituximab for disease control, which evolved with a 3-year indolent progressive systemic sarcoid reaction.
View Article and Find Full Text PDFBackground: Rituximab is already a standard part of the treatment of patients with Waldenström's macroglobulinemia. However, a small proportion of patients develop intolerance to rituximab during administration or the treatment is not very effective. In these patients, we are faced with the question of whether another anti-CD20 monoclonal antibody can be used and what result will be achieved.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!