Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options

Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic... AbstractOBJECTIVE:Idiopathic hypertrophic cranial pachymeningitis is a rare disease, of undetermined pathogenesis, that is characterized by inflammation and fibrosis of the dura mater.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:Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurosurgery Oxford University Press

Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options

Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options

Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options Norikazu Hatano, M.D., Sanjay Behan, M.D., Tetsuya Nagatani, M.D., Masaaki Kimura, M.D., Keiji Ooka, M.D., Kiyoshi Saito, M.D., Jun Yoshida, M .D. Department of Neurosurgery (NH, TN, KS, )Y), Nagoya University School of Medicine, Nagoya, )apan; Department of Neurosurgery (SB), Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India; Department of Neurosurgery (MK), Nagoya Daini Red Cross Hospital, Nagoya, Japan; and Department of Neurosurgery (KO), Tokai Central Hospital, Kagamihara, japan O B JE C T IV E : Idiopathic hypertrophic cranial pachym eningitis is a rare disease, of undeterm ined pathogenesis, that is characterized by inflam m ation and fibrosis of the dura mater. M E T H O D S : W e encountered six patients w ith idiopathic hypertrophic cranial pachym eningitis and analyzed their clin ical presentations, radiological findings, and treatm ent. 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. C O N C L U S IO N : Idiopathic hypertrophic cranial pachym eningitis exhibits varied clin ica l...
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Publisher
Oxford University Press
Copyright
© Published by Oxford University Press.
ISSN
0148-396X
eISSN
1524-4040
D.O.I.
10.1097/00006123-199912000-00014
Publisher site
See Article on Publisher Site

Abstract

AbstractOBJECTIVE:Idiopathic hypertrophic cranial pachymeningitis is a rare disease, of undetermined pathogenesis, that is characterized by inflammation and fibrosis of the dura mater.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:Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression.

Journal

NeurosurgeryOxford University Press

Published: Dec 1, 1999

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