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

J Korean Neurol Assoc > Volume 19(1); 2001 > Article

Journal of the Korean Neurological Association 2001;19(1): 56-59.

Tolosa-Hunt 증후군을 동반한 특발성 비후성 두개 경막염 1예

김은주, 박경필 ·김대성 ·정대수 ·박규현 ·박동준*·엄부섭**

부산대학교 의과대학 신경과학교실,신경외과학교실*,안과학교실**

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

Eun-Joo Kim, M.D., Kyung-Pil Park, M.D., Dae-Seong Kim, M.D., Dae-Soo Jung, M.D.,Kyu-Hyun Park, M.D., Dong-June Park, M.D.*, Boo-Sup Oum, M.D.**

Department of Neurology, Neurosurgery* & Ophthalmology**, Pusan National University School of Medicine

Abstract

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disease, which causes chronic progressive inflamma-tion 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, 2001Key Words : Idiopathic hypertrophic cranial pachymeningitis, Tolosa-Hunt syndrome