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Pachymeningitis 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.1–6 “Idiopathic hypertrophic cranial pachymeningitis” is also noticeable, and is based on a process of chronic inflammation whose cause is unclear.4 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 hydrocephalus (fig 1). Because of the hydrocephalus, it was neurologically advisable not to perform a cerebrospinal fluid puncture. There was no indication of tuberculosis (chest x ray examination, tuberculin test, sputum culture) or any other infection. The neoplasia investigation was also negative. An anamnestic investigation showed that there was no need to use ibuprofen or sulindac.
When the situation became life threatening we initiated immunosuppressive treatment with cyclophosphamide (100 mg/day), as well as intravenous steroids (100 mg) initially. The initial intravenous cortisone treatment relieved the headache symptoms. Follow up examinations for up to 1 year (every 3 months) showed remission of clinical and MRI findings with this treatment (fig 2). Stable partial remission occurred after 8 months, and oral administration of cyclophosphamide was stopped. Treatment to preserve remission was changed to azathioprine (100 mg). The prednisone dose was finally reduced to 5 mg.
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