[Atypical headache and facial pain as a result of hypertrophic pachymeningitis in C-ANCA-positive Wegener's granulomatosis].
BACKGROUND
Wegener's granulomatosis (WG) is a systemic vasculitis involving the nervous system in 20-54% of cases; lesions of peripheral nerves are commonest, while manifestation in the central nervous system (CNS) is rarer. Focal hypertrophic pachymeningitis is a very rare complication of WG. This inflammatory thickening and fibrosis of the dura mater is always associated with headaches, whereas cranial nerve lesions, cerebellar symptoms or epileptic seizures occur more rarely.


METHODS
A 67-year-old patient, in whom WG had been diagnosed 2 years earlier and who had been treated with immunosuppressants since then, complained of continuous severe, mainly left-sided headache and facial pain for weeks. Cranial MRI showed thickening of the left tentorium cerebelli with obvious contrast enhancement and led to the diagnosis of hypertrophic pachymeningitis. The inflammatory parameters and the C-ANCA (antineutrophil cytoplasmic antibodies) in the serum were raised and CANCA were detectable in the cerebrospinal fluid. The headaches subsided with several days of intravenous high-dose corticosteroids and a simultaneous increase in the immunosuppressive basic medication. On a follow-up MRI after 3 months, the magnetic resonance changes were less apparent, i. e., the hypertrophic pachymeningitis was resolving; C-ANCA were now no longer detectable in the cerebrospinal fluid.


CONCLUSIONS
With newly occurring, unusually severe and persistent headaches in the presence of WG, the very rare complication of hypertrophic pachymeningitis should be considered.
Kuhn
Jens
J
Neurologische Klinik, Kliniken der Stadt Köln, Krankenhaus Merheim, Köln. Jkuhn11@aol.com
Weber
Manfred
M
Hedde
Jan-Peter
JP
Bewermeyer
Heiko
H
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000134:cerebrospinal fluid*; D004359:Drug Therapy, Combination; D005157:Facial Pain / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006261:Headache / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007275:Injections, Intravenous; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000209:etiology*; D013997:Time Factors
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents