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KMID : 1022820130040020057
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Journal of Multiple Sclerosis and Neuroimmunology
2013 Volume.4 No. 2 p.57 ~ p.60
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Atypical Type of Idiopathic Hypertrophic Pachymeningitis with Predominant Leptomeningeal Enhancement
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Lee Sung-Woog
Baek Seol-Hee
Jung Jin-Man
Kwon Do-Young
Park Moon-Ho
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Abstract
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Idiopathic hypertrophic pachymeningitis (IHP) is an uncommon chronic inflammatory disorder that causes a localized or diffuse fibrous thickening of the dura matter. It presents as diverse neurological symptoms attributed to meningeal involvement and increased intracranial pressure. IHP is diagnosed by magnetic resonance image (MRI) with gadolinium-enhancement and brain biopsy, which can reveal an inflammatory change and diffuse fibrosis of dura matter. It has been reported that IHP might fairly well respond to corticosteroid treatment or immunosuppressant. However, we present here on a case of atypical type of IHP with predominantly leptomeningeal enhancement and poor response to steroid and immunosuppressive agent.
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KEYWORD
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Idopathic hypertrophic pachymeningitis, Cyclophospharmide, Leptomenigeal enhancement
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