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A juvenile case of idiopathic hypertrophic pachymeningitis involved cavernous sinus and proximal trigeminal nerve
Author(s) -
Bian Zhihong,
Omote Yoshio,
Tadokoro Koh,
Ikegami Ken,
Osakada Yosuke,
Takemoto Mami,
Hishikawa Nozomi,
Yamashita Toru,
Ohta Yasuyuki,
Abe Koji
Publication year - 2020
Publication title -
neurology and clinical neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
0ISSN - 2049-4173
DOI - 10.1111/ncn3.12355
Subject(s) - medicine , cavernous sinus , diplopia , magnetic resonance imaging , cerebrospinal fluid , trigeminal nerve , dura mater , pathology , extraocular muscles , nerve root , anatomy , radiology , surgery
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.