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Atypical Type of Idiopathic Hypertrophic Pachymeningitis with Predominant Leptomeningeal Enhancement

J Mult Scler > Volume 4(2); 2013 > Article
Journal of Multiple Sclerosis 2013;4(2):57-60.
Published online September 1, 2013.
연수막 조영증강이 동반된 비특이적인 특발성 비후성 경수막염
이성욱, 백설희, 정진만, 권도영, 박문호
고려대학교 의과대학 안산병원 신경과학교실
Atypical Type of Idiopathic Hypertrophic Pachymeningitis with Predominant Leptomeningeal Enhancement
Sung-Woog Lee, Seol-Hee Baek, Jin-Man Jung, Do-Young Kwon, Moon Ho Park
Department of Neurology, Korea University Ansan Hospital, Korea University College of Medicine, Ansan, Korea
Abstract
"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 neurologicalsymptoms attributed to meningeal involvement and increased intracranial pressure. IHP is diagnosedby magnetic resonance image (MRI) with gadolinium-enhancement and brain biopsy, whichcan reveal an inflammatory change and diffuse fibrosis of dura matter. It has been reported that IHPmight fairly well respond to corticosteroid treatment or immunosuppressant. However, we presenthere on a case of atypical type of IHP with predominantly leptomeningeal enhancement and poor responseto steroid and immunosuppressive agent. Journal of Multiple Sclerosis 4(2):57-60, 2013"
Key Words: Idiopathic hypertrophic pachymeningitis, Cyclophosphamide, Leptomeningeal enhancement


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