Publication: Idiopathic hypertrophic cranial pachymeningitis
Issued Date
2002-01-01
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ISSN
00283886
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2-s2.0-0036225714
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Mahidol University
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SCOPUS
Bibliographic Citation
Neurology India. Vol.50, No.1 (2002), 53-59
Suggested Citation
Arada Rojana-udomsart, Teeratorn Pulkes, Kaseansom Viranuwatti, Jiraporn Laothamatas, Suchart Phudhichareonrat, Rawiphan Witoonpanich Idiopathic hypertrophic cranial pachymeningitis. Neurology India. Vol.50, No.1 (2002), 53-59. doi:10.1016/j.jocn.2006.11.020 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/20579
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Title
Idiopathic hypertrophic cranial pachymeningitis
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Abstract
Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. The diagnosis of idiopathic hypertrophic cranial pachymeningitis was based on neuroimaging findings of thickened enhancing dura, exclusion of known causes and histopathologic findings compatible with nonspecific inflammation in the meningeal biopsies. Corticosteroid therapy was effective in all cases in inducing a complete or partial remission of the neurologic symptoms and signs. We describe the clinical, radiological and pathological features of idiopathic hypertrophic cranial pachymeningitis and discuss the relationship of this entity with other inflammatory fibrosclerotic disorders to explain the pathogenesis. A high index of suspicion, prompt confirmation of the diagnosis by meningeal biopsy, and early institution and long-term maintenance of steroid therapy may help to prevent irreversible neurologic sequelae, especially blindness.