Abstract
Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an extremely rare disease of unknown origin. It is a fibrosing chronic inflammatory proces that effects intracranial dura mater.
The clinical picture can be heterogeneous including headache, ataxia, seizures and cranial nerve palsy. Cranial nerve palsy in 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 findigs include elevated C-reactive protein and elevated blood sedimentation.
Findings i n cerebrospinal fluid usually show a chronic aseptic proces. Magnetic resonance imaging (MR) 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 progresssive and includes frequent recurrences.
Corticosteroid therapy should be the first approach in IHCP, useful is a combination with azathioprine, cyclosphoshamide or methotrexate. Resistant cases can profit from radiotherapy or surgery.
We report two cases from our own patinent base through which we demonstrate the course, diagnostics and therapy of this disorder.