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자료유형
학술저널
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대한신경과학회 Journal of Clinical Neurology Journal of Clinical Neurology 제13권 제2호
발행연도
2017.1
수록면
155 - 161 (7page)

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Background and Purpose Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are ta¬pered. Here we report three HP cases that were successfully treated with rituximab (RTX). Methods From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m2). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. Results All three patients were recurrence-free of neurologic symptoms and exhibited promi¬nent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. Conclusions We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.

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