Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of hypertrophic spinal pachymeningitis associated with MPO-ANCA

Yusuke Yakushiji, M.D.1), Kazuhiro Kurohara, M.D.1), Shuji Toda, M.D.2), Masamitsu Abe, M.D.3) and Yasuo Kuroda, M.D.1)

Departments of Internal Medicine1), Pathology2) and Neurosurgery3), Saga Medical School

A 71-year-old man was admitted to our hospital, because of subacute progressive back pain and thoracic transverse myelopathy. Magnetic resonance imaging showed thickening with gadolinium enhancement of dura mater at the T1-T6 vertebrate levels. A dura biopsy specimen revealed fibrous thickening of the dura with the inflammatory changes, and diagnosis of hypertrophic spinal pachymeningitis was made. The cerebrospinal fluid (CSF) contained high titer of myeloperoxydase anti-neutrophil cytoplasmic antidody (MPO-ANCA), indicating that the antibody synthesized intrathecally. After treatment with oral prednisolone, 60 mg daily for a month, thickness of the dura and the CSF MPO-ANCA improved dramatically. This is the first report of hypertrophic pachymeningitis associated with MPO-ANCA localized exclusively in the spine. This report suggests that intrathecal MPO-ANCA synthesis is an index of disease activity as well as a diagnostic hallmark. Early corticosteroid therapy is recommended in this disorder.

(CLINICA NEUROL, 42: 873|877, 2002)
key words: hypertrophic pachymeningitis, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), magnetic resonance imaging, corticosteroid, cerebrospinal fluid

(Received: 1-May-02)