Idiopathic hypertrophic cranial pachymeningitis manifested by transient ischemic attack.

BACKGROUND: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a very infrequent disorder that causes a localized or diffuse thickening of the dura mater. The main clinical features of IHCP at presentation are headache, cranial nerve involvement, ataxia, and seizures. Transient ischemic attacks (TIAs) have not previously been reported as a main clinical manifestation. We present a case with TIA as the main clinical manifestation and investigated the treatment of the case. CASE REPORT: A 33-year-old male complaining of recurrent left hemiparesis for 22 days was admitted into our hospital. The clinical characteristics and CT, MRI, and pathological data were investigated carefully. IHCP was diagnosed and pulse corticosteroid therapy was used to treat this case. Headache, neck pain and recurrent episodes of weakness and numbness in the left limbs were the main clinical manifestations. CT and MRI revealed thickening of the dura along the falx and tentorium. Meningeal biopsy showed diffused thickening of the dura, consisting of collagen in concentric form with inflammatory cell infiltrating and fibrinoid necrosis, confirmed to be dura mater by histopathological examination. Treatment with an antiplatelet drug, low molecular heparin and herbs did not prevent the attacks. Pulse corticosteroid therapy was used and good efficiency was achieved.
CONCLUSIONS: Recurrent episodes of TIAs can be the main clinical manifestations of IHCP, and pulse corticosteroid therapy was effective.