J Korean Med Sci. 2001 Oct;16(5):683-688. English.
Published online Apr 24, 2009.
Copyright © 2001 The Korean Academy of Medical Sciences
Case Report

Idiopathic Hypertrophic Spinal Pachymeningitis: A Case Report

Sung Hye Park, C Jin Whang, Moonjun Sohn, Yeun Chul Oh, Chae Hyuk Lee and Yoon Joon Whang
    • Department of Pathology, Neurosurgery, Ilsan Paik Hospital, Inje University Medical College, Goyang, Korea.

Abstract

Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a typical case of spinal IHP occurring in a long segment of cervical and thoracic dura from C6 to T8. The patient was 56-yr-old female, who had been suffered from pain on her upper back and both arms for 3 months and recent onset motor weakness of both legs. During the 9 months of follow-up period, she experienced the improvement of her neurologic symptoms with combined therapy of partial excision and corticosteroid medication. Since early surgical intervention and subsequent pulse ste-roid therapy are mandatory for this disease to avoid irreversible damage of nervous system, the identification of this unique disease entity is essential on frozen diagnosis. A few cases have been reported in Korean literature.

Keywords
Meningitis; Idiopathic Hypertrophic Pachymeningitis; Spine; Immunohistochemical Study; Chronic Nonspecific Inflammation; Granulomatous Disease, Chronic


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