Pachymeningitis in mixed connective tissue disease.

P achymeningitis is a rare illness which can be shown by magnetic resonance imaging (MRI) to be a thickening of the intracranial dura mater, when associated with an infectious, malignant, or rheumatic systematic disease. ‘‘Idiopathic hypertrophic cranial pachymeningitis’’ is also noticeable, and is based on a process of chronic inflammation whose cause is unclear. Typical symptoms are chronic headaches, facial pain, cerebella ataxia, cranial nerve failure, neuro-ophthalmic complications, including papilloedema. We report on a 64 year old woman who presented with monthly progressive cephalgia, subfebrile temperatures, loss of weight, Raynaud’s phenomenon, proximal myasthenia, arthritis, photosensitivity, and prickling paraesthesia. From this we diagnosed a mixed connective tissue disease, fulfilling at least three American College of Rheumatology (ACR) criteria, with a secondary vasculitis (pachymeningitis and polyneuropathy) and pulmonary disease (neutrophilic alveolitis). A cerebral MRI scan showed initially and after 3 months clear thickening and enrichment of contrast dye in all meningeal structures (pachymeningitis), as well as widened ventricles I–III, with periventricular capping in the form of a