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Author Sylaja, P. N. ♦ Cherian, P. J. ♦ Das, C. K. ♦ Radhakrishnan, V. V. ♦ Radhakrishnan, K.
Source Sree Chitra Tirunal Institute for Medical Sciences & Technology
Content type Text
Publisher Neurology India
File Format PDF
Language English
Subject Domain (in DDC) Technology ♦ Medicine & health ♦ Diseases
Subject Domain (in MeSH) Nervous System Diseases ♦ Diseases
Subject Keyword Neurology
Abstract Idiopathic hypertrophic cranial pachymeningitis is a rare form of ribrosing 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.
Education Level UG and PG
Learning Resource Type Article
Educational Framework Medical Council of India (MCI)
Journal NEUROLOGY INDIA
Volume Number 50
Issue Number 1
Page Count 7
Starting Page 53
Ending Page 59