A Case of Idiopathic Hypertrophic Cranial Pachymeningitis Associated with Tolosa-Hunt Syndrome.
- Author:
Eun Joo KIM
1
;
Kyung Pil PARK
;
Dae Seong KIM
;
Dae Soo JUNG
;
Kyu Hyun PARK
;
Dong June PARK
;
Boo Sup OUM
Author Information
1. Department of Neurology, Pusan National University School of Medicine.
- Publication Type:Case Report
- Keywords:
Idiopathic hypertrophic cranial pachymeningitis;
Tolosa-Hunt syndrome
- MeSH:
Adult;
Ataxia;
Biopsy;
Brain;
Cavernous Sinus;
Cranial Nerve Diseases;
Dura Mater;
Exophthalmos;
Headache;
Humans;
Inflammation;
Magnetic Resonance Imaging;
Meningitis*;
Rare Diseases;
Seizures;
Tolosa-Hunt Syndrome*;
Visual Perception
- From:Journal of the Korean Neurological Association
2001;19(1):56-59
- CountryRepublic of Korea
- Language:Korean
-
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