METHODS

A 43-year-old man was admitted to a hospital because of an exacerbation of severe headache, which for three years had been refractory to any treatment. Headache had led to chronic use of analgesics and to drug-treated depression. As a result he had to quit his job the previous year and had since then been receiving a disability pension. At that time diagnostic procedures done elsewhere, including cranial computer tomography and laboratory tests had not shown any pathological results. At the current admission the patient was in a reduced general condition and complained of severe headache and depression. However, the physical examination, including neurological tests, was normal.

METHODS

Cranial magnetic resonance imaging (MRI) revealed general thickening and pronounced contrast enhancement of all meningeal structures (pachymeningitis). Laboratory tests indicated renal failure and proteinuria, as well as elevation of anti-neutrophilic cytoplasmic antibodies against proteinase 3 (PR3-ANCA).

METHODS

The MRI findings and laboratory results established the diagnosis of a severe pachymeningitis secondary to vasculitis of the central nervous system as part of Wegener's granulomatosis. Immunosuppressive therapy with cyclophosphamide and glucocorticoids resulted in the severe headaches subsiding within a few days. The patient was able to stop taking analgesics and antidepressants. However, renal function improved only partially.

CONCLUSIONS

Pachymeningitis, as demonstrated on MRI, is a rare manifestation of inflammatory cerebrovascular involvement in Wegener's granulomatosis. Pachymeningitis should be considered in patients with severe and persistent headache of unknown aetiology, as well as in patients with Wegener's granulomatosis with recent onset of headache.