Idiopathic hypertrophic pachymeningitis - case report and literature review.

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare pathological state, with still unclear aetiopathogenesis. We present a case of a 63-year-old woman with cranial variety of that disease. The manifestations of the disease included headaches, paresis of VI, IX, X nerves and cerebellar ataxia. The disease was diagnosed with magnetic resonance imaging (MRI) and histopathological assessment of the pachymeninx biopsy specimen. The MRI revealed significant thickening of the cranial base pachymeninx, compressing the pons and medulla oblongata. MRI examinations could be misinterpreted as extensive meningioma of the skull base. Dura mater biopsy revealed however inflammation with abundant lymphocytic infiltrations. Clinical improvement was obtained after the application of corticosteroids. We noted the subsidence of all symptoms of the disease, as well as radiological improvement, manifested through substantial regression of the described changes in the pachymeninx. The patient has been presented in the context of 65 cases of idiopathic hypertrophic pachymeningitis, described in the literature of English-speaking countries in the last five years. Recently, the importance of the autoimmunogenic background of IHPM has been underlined. In that respect IHPM has become an interdisciplinary problem. Its diagnosis and treatment requires not only radiologists, neurologists, pathomorphologists and neurosurgeons, but also specialists in internal medicine, including immunologists, allergologists and rheumatologists as well - in other words, physicians that rarely take part in the processes of diagnosing and treating intracranial pathologies.