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2017 ; 13
(2
): 155-161
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Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis
#MMPMID28271643
Jang Y
; Lee ST
; Jung KH
; Chu K
; Lee SK
J Clin Neurol
2017[Apr]; 13
(2
): 155-161
PMID28271643
show ga
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 tapered. 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 prominent
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.