Abstract | OBJECTIVE: METHODS: We encountered six patients with idiopathic hypertrophic cranial pachymeningitis and analyzed their clinical presentations, radiological findings, and treatment. RESULTS: In the six patients, the main manifestations were cranial nerve palsies and headache. Three associations were present, namely optic neuropathy, Tolosa-Hunt syndrome, and diabetes insipidus. Gadolinium-enhanced magnetic resonance imaging was diagnostic, showing intense dural enhancement in a linear or nodular pattern. The responses to corticosteroid therapy were better for patients who exhibited linear, rather than nodular, dural enhancement. For one patient, surgical decompression of the superior orbital fissure provided lasting relief. The course of the disease followed one of three patterns, i.e., sustained remission, relapse with corticosteroid independence, or relapse with corticosteroid dependence. Pulse corticosteroid therapy provided significant relief, while reducing the daily corticosteroid requirement and avoiding side effects, for a corticosteroid-dependent relapsing patient. CONCLUSION:
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Authors | N Hatano, S Behari, T Nagatani, M Kimura, K Ooka, K Saito, J Yoshida |
Journal | Neurosurgery
(Neurosurgery)
Vol. 45
Issue 6
Pg. 1336-42; discussion 1342-4
(Dec 1999)
ISSN: 0148-396X [Print] United States |
PMID | 10598701
(Publication Type: Case Reports, Journal Article)
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Chemical References |
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Topics |
- Adrenal Cortex Hormones
(administration & dosage)
- Aged
- Diagnosis, Differential
- Dura Mater
(pathology)
- Female
- Humans
- Hypertrophy
- Image Enhancement
- Magnetic Resonance Imaging
- Male
- Meningitis
(diagnosis, drug therapy, pathology)
- Middle Aged
- Neurologic Examination
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