RT Journal Article
SR Electronic
T1 A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase
JF BMJ Case Reports
FD BMJ Publishing Group Ltd
SP bcr0620092016
DO 10.1136/bcr.06.2009.2016
VO 2010
A1 Kiyotaka Nakamagoe
A1 Ai Hosaka
A1 Yuzuru Kondo
A1 Eiichi Ishikawa
A1 Akira Tamaoka
YR 2010
UL http://casereports.bmj.com/content/2010/bcr.06.2009.2016.abstract
AB This report concerns a 53-year-old male patient with idiopathic hypertrophic cranial pachymeningitis who presented with multiple cranial nerve palsies (I, II, III, IV, V, VI). Brain magnetic resonance imaging showed diffuse thickening and gadolinium enhancement of the cerebral dura mater. A biopsy of the cerebral dura mater showed granulomatous vasculitis with histiocyte infiltration. Although both the serum rheumatoid factor (RF) and matrix metalloproteinase-3 (MMP-3) were high, the patient showed no signs of arthritis. He was anti-cyclic citrullinated peptide antibody negative, which makes the presence of comorbid chronic rheumatoid arthritis (RA) unlikely. The aetiology of the pachymeningitis was unknown, which led to the diagnosis of idiopathic hypertrophic cranial pachymeningitis. Steroid pulse therapy successfully diminished the patient’s pachymeningitis and lowered both RF and MMP-3. High values of RF suggest the possible involvement of an autoimmune mechanism, and the MMP value may be an important indicator of the aetiology of pachymeningitis with granulomatous vasculitis.