Klinische Monatsblätter für Augenheilkunde
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Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a chronic inflammatory disease of unknown origin that is morphologically characterized by a local fibrotic thickening of the dura mater with a sterile lymphocytic infiltrate. Patients usually present with chronic headache, ataxia and cranial nerve palsies. Because of the great diversity of symptoms and the fact that IHCP can be associated with many other disorders, there is often a long time between the occurrence of clinical features and making the diagnosis. During this time symptoms can further increase. In addition, this is the third case report describing bone involvement in this disorder. ⋯ Chronic headache, ataxia and cranial nerve palsies in combination with inflammation and dural thickening should call to mind idiopathic hypertrophic cranial pachymeningitis. In principle, every ophthalmological or neurological symptom can be associated with IHCP.