Long-term lack of progression after initial treatment of idiopathic hypertrophic pachymeningitis

Abstract

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare inflammatory disease which is sometimes difficult to diagnose and can lead to misinterpretations of the clinical and imaging findings. The main clinical manifestations are headache, ataxia and cranial nerve palsy. In most of the reported patients continuous medication is needed to avoid disease recurrence. We present a female patient with an 8-year follow-up, no clinical regression and no need for any further medical treatment. Even though most patients with IHCP experience recurrence after diagnosis and initial treatment there were no clinical or imaging signs of relapse in our patient. Our patient is still not under any medical or surgical treatment due to the lack of any significant symptoms.