A Case of Idiopathic Hypertrophic Cranial Pachymeningitis Associated with Tolosa-Hunt Syndrome

ABSTRACT

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disease, which causes chronic progressive inflammation and thickening of the basal dura mater and produces multiple cranial neuropathies, headache, ataxia, and seizure. A 40 year-old man presented with a continuous, diffuse, dull headache with left periorbital pain, exophthalmos, ptosis and loss of visual perception. Brain MRI revealed thickened, highly enhanced pachymeninges in all supratentorial areas in association with the left cavernous sinus. A subsequent meningeal biopsy showed non-specific chronic inflammations of the pachymeninges. The patient dramatically responded to steroid therapy. To our knowledge, this is an extremely rare case of IHCP associated with Tolosa-Hunt syndrome. (J Korean Neurol Assoc 19(1)56~59, 2001