Idiopathic hypertrophic cranial pachymeningitis associated with hydrocephalus and myocarditis: remarkable steriod-induced remission of hypertrophic dura mater.
We describe a patient with idiopathic hypertrophic cranial pachymeningitis (IHCP) associated with hydrocephalus and myocarditis in whom steroids caused remission of the hypertrophic dura and enlarged ventricles. Myocarditis induced complete atrioventricular block that responded to steroids initially but later required permanent pacemaker implantation. Serum ANA and anti-dsDNA antibody were positive. Our case suggests that IHCP and myocarditis may share a common autoimmune etiology.
Tanaka
M
M
Department of Medicine and Clinical Science, Kyoto University Graduate School of Medicine, Japan.
Suda
M
M
Ishikawa
Y
Y
Fujitake
J
J
Fujii
H
H
Tatsuoka
Y
Y
D000368:Aged; D001706:Biopsy; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001927:Brain Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D003287:Contrast Media; D004388:Dura Mater / Q000000981:diagnostic imaging / Q000473:pathology; D019786:Gadolinium DTPA; D006650:Histocompatibility Testing; D006801:Humans; D006849:Hydrocephalus / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D009205:Myocarditis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D009942:Organometallic Compounds; D010138:Pacemaker, Artificial; D004369:Pentetic Acid / Q000031:analogs & derivatives; D014057:Tomography, X-Ray Computed
D003287:Contrast Media; D009942:Organometallic Compounds; D004369:Pentetic Acid; D019786:Gadolinium DTPA