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Logo of bmjcrInstructions for authorsCurrent ToCBMJ Case Reports
BMJ Case Rep. 2010; 2010: bcr06.2009.2016.
Published online Feb 25, 2010. doi:  10.1136/bcr.06.2009.2016
PMCID: PMC3027825
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A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase
Kiyotaka Nakamagoe,1 Ai Hosaka,1 Yuzuru Kondo,2 Eiichi Ishikawa,3 and Akira Tamaoka1
1Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan
2Department of Pathology, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan
3Department of Neurosurgery, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tennodai 1-1-1, Tsukuba, Ibaraki, 305-8575, Japan
Correspondence to Kiyotaka Nakamagoe, Nakamagoek/at/
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.
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