A juvenile case of idiopathic hypertrophic pachymeningitis involved cavernous sinus and proximal trigeminal nerve. Issue 2 (12th December 2019)

• Record Type: Journal Article
• Authors: Bian, Zhihong; Omote, Yoshio; Tadokoro, Koh; Ikegami, Ken; Osakada, Yosuke; Takemoto, Mami; Hishikawa, Nozomi; Yamashita, Toru; Ohta, Yasuyuki; Abe, Koji
• Is Part Of: Neurology and clinical neuroscience. Volume 8:Issue 2(2020:Mar.)
• Page Start: 82
• Page End: 85
• Publication Date: 2019-12-12
• Subjects: cavernous sinus -- diplopia -- idiopathic hypertrophic pachymeningitis -- juvenile -- trigeminal nerve; Neurology -- Periodicals; Neurosciences -- Periodicals; 616.8
• DOI: 10.1111/ncn3.12355
• Languages: English
• ISSNs: 2049-4173

Abstract

Abstract: Idiopathic hypertrophic pachymeningitis (HP) is a rare inflammatory disorder characterized by local or diffuse thickening of the cranial or spinal dura mater, which is primarily reported in adults. Here, we report a juvenile idiopathic HP case presenting diplopia and multiple cranial nerve palsies. Although cranial magnetic resonance imaging (MRI) shows evident lesions in cavernous sinus and preganglionic segment of trigeminal nerve, only cerebrospinal fluid (CSF) showed elevated protein (55 mg/dL) and myelin basic protein (MBP, 355 pg/mL) but negative systemic inflammatory markers. Steroid therapy greatly improved the symptom and the CSF data in 1 month.