J Korean Neurol Assoc.  2001 Jan;19(1):56-59.

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

Affiliations
  • 1Department of Neurology, Pusan National University School of Medicine.
  • 2Department of Neurosurgery, Pusan National University School of Medicine.
  • 3Department of Ophthalmology, Pusan National University School of Medicine.

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

Keyword

Idiopathic hypertrophic cranial pachymeningitis; Tolosa-Hunt syndrome

MeSH Terms

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
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