Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja A rare, brain inflammatory disease characterized by thickening of the dura mater of the cranium or spine with at least two histiopatholgical features of IgG4-related disease: dense lymphoplasmacytic infiltrate, storiform fibrosis, and/or obliterative phlebitis. Patients typically have non-specific CSF findings, and might be without systemic involvement or serum IgG4 elevation. Clinical manifestation are caused by mechanical compression of nerve or vascular structure, leading to functional deficit, most commonly headache, cranial nerve palsies, vision problems and motor weakness. Disease data Klasyfikacja Clinical subtype Synonimy Idiopathic hypertrophic pachymeningitis Idiopatyczne przerostowe zapalenie opony twardej Kod ORPHA 449427 Kod OMIM - Kod ICD10 G03.9 Kod ICD11 - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl