Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Headache as a manifestation of SAPHO syndrome with a lesion extending to the dura mater, parietal bone, and temporal muscle

Miho Uematsu, M.D.1)2), Shinsuke Tobisawa, M.D.1), Masahiro Nagao, M.D.1), Shiro Matsubara, M.D.1), Toshio Mizutani, M.D.3) and Makoto Shibuya, M.D.4)

1)Department of Neurology, Tokyo Metropolitan Neurological Hospital
2)Department of Neurology, Kyoto City Hospital
3)Department of Pathology, Tokyo Metropolitan Neurological Hospital
4)Department of Pathology, Tokyo Medical University Ibaraki Medical Center

A 50-year-old woman with a history of palmoplantar pustulosis, femur osteomyelitis, and sterno-costoclavicular hyperostosis presented with a chronic severe left temporal headache that had progressed during the previous year. Her CRP level was elevated. Cranial images showed Gadolinium-enhancement of the left temporal muscle, left parietal bone and dura mater. 99mTc-HMDP scintigram showed increased uptake in the left parietal bone, left sterno-costo-clavicular joint, right femoral head and intervertebral joints. Biopsy of the lesion demonstrated 1) proliferation of connective tissue in both perimysium and endomysium of the temporal muscle with mild inflammatory cell infiltration within the interstitium, 2) marked infiltration of granulocytes to the bone marrow of the parietal bone, 3) necrosis and moderate fibrosis in the interstitium with inflammatory cell infiltration in the parietal bone, and 4) moderate fibrosis and slight infiltration of inflammatory cells in the dura mater. The patient was diagnosed with a cranial lesion of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome. There was a moderate response to treatment with intravenous steroid pulse therapy and subsequent methotrexate. In a case of headache accompanied by inflammatory response, palmoplantar pustulosis and joint lesions such as hyperostosis, the possibility of a rare cranial manifestation of SAPHO syndrome should be considered.
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(CLINICA NEUROL, 52: 106|110, 2012)
key words: SAPHO syndrome, cranial lesion, temporal muscle, acquired hyperostosis syndrome

(Received: 12-May-11)