Idiopathic Hypertrophic Cranial Pachymeningitis – Two Case Reports

Czech version

Authors: Z. Pavelek ¹; P. Ryška ²; J. Žižka ²; S. Plíšek ³; M. Vališ ¹
Authors‘ workplace: Neurologická klinika, LF UK a FN Hradec Králové ¹; Radiologická klinika, LF UK a FN Hradec Králové ²; Klinika infekčních nemocí, LF UK a FN Hradec Králové ³
Published in: Cesk Slov Neurol N 2016; 79/112(5): 604-607
Category: Case Report

Overview

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an extremely rare disease of unknown origin. It is a fibrosing chronic inflammatory process that affects intracranial dura mater. The clinical picture can be heterogeneous including headache, ataxia, seizures and cranial nerve palsy. Cranial nerve palsy is caused by compression of the exit zone of the nerve roots by hypertrophic basal pachymeningitis. IHCP can even imitate transient ischemic attack. Common haematological abnormal findings include elevated C-reactive protein and elevated blood sedimentation. Findings in cerebrospinal fluid usually show a chronic aseptic process. Magnetic resonance imaging (MRI) is the most important diagnostic method. MRI finds a diffusely thickened dura that enhances after paramagnetic contrast injection. The course of disease is chronic and progressive and includes frequent recurrences. Corticosteroid therapy should be the first approach in IHCP, useful is a combination with azathioprine, cyclophosphamide or methotrexate. Resistant cases can profit from radiotherapy or surgery. We report two cases from our own patient base through which we demonstrate the course, diagnostics and therapy of this disorder.

Key words:
pachymeningitis – magnetic resonance imaging – thickening of dura mater

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

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