Final Diagnosis -- Primary Wegener’s granulomatosis of the meninges


Primary Wegener's granulomatosis of the meninges.


Wegener's granulomatosis (WG) is a systemic disease of unknown origin characterized by necrotizing granulomatous vasculitis which involves primarily the respiratory tract and the kidney. Recently, the disease has been associated with the HLA-DPB1 locus (5). The onset of the disease is typically in the 4th decade of life. WG involves the nervous system in 20-54% of cases, predominantly as mononeuritis multiplex, while manifestation in the central nervous system (CNS) is less common (2% - 8%) (6). Primary manifestation in the nervous system is rare. Several case reports demonstrated patients suffering from palsies of multiple cranial nerves without pulmonary manifestations (3,9). Atypical cases of WG presented as ischemic stroke (10) as subarachnoid hemorrhage (2) or pituitary disease (9). In one patient a Wegener's lesion formed around the cervical spinal cord similarly to the case presented here (7). This patient, however, had elevated c-ANCA which resolved after high dose corticosteroid therapy. A number of reports demonstrated secondary spread of WG to the meninges. In these cases inflammatory thickening and fibrosis of the dura mater is usually associated with headaches, whereas cranial nerve lesions, cerebellar symptoms or epileptic seizures occur only rarely (1,4,7). In addition, a case of diffuse involvement of the meninges from Wegener's granulomatosis followed by chronic aseptic meningitis with communicating hydrocephalus and papillary edema was reported (8). Differential diagnosis in the case presented here included tuberculosis which was excluded microbiologically and sarcoidosis for which the histology was not typical.

This is the first case of WG presenting as thickening of the dura in the area of the medulla oblongata, followed by cranial nerve palsy (VI and XII), ataxia, headache, personality changes and hydrocephalus.

In the woman presented here, it is noteworthy that serological tests for p- and c-ANCA, ANA, DNA, ENA were negative over the complete observation period of 1.5 years. The improvement of neurological symptoms and complete regression of the lesion in MRI 1.5 years after surgery (Figures 11, 12) after low dose medication with prednisolone (10 mg p.d.), however, support the histological diagnosis.


  1. von Bierbrauer A, Pelzer C, Fischer V (2008) Severe chronic headache with depression induced by pachymeningitis in Wegener's granulomatosis. Dtsch Med Wochenschr 133:185-8
  2. Cruz DN, Segal AS (1997). A patient with Wegener's granulomatosis presenting with a subarachnoid hemorrhage: case report and review of CNS disease associated with Wegener's granulomatosis. Am J Nephrol 17:181-6.
  3. Daderian AD, Chayasirisobhon S (2000). An unusual case of multiple cranial nerve palsies in Wegener's granulomatosis. J Natl Med Assoc 92:455-7.
  4. Di Comite G,Bozzolo EP, Praderio L, Tresoldi M, Sabbadini MG (2006). Meningeal involvement in Wegener's granulomatosis is associated with localized disease. Clin Exp Rheumatol 24:S60-4
  5. Heckmann M, Holle JU, Arning L, Knaup S, Hellmich B, Nothnagel M, Jagiello P, Gross WL, Epplen JT, Wieczorek S (2008) The Wegener's granulomatosis quantitative trait locus on chromosome 6p21.3 as characterised by tagSNP genotyping. Ann Rheum Dis 67:972-9
  6. Kuhn J, Weber M, Hedde JP, Bewermeyer H (2005) Atypical headache and facial pain as a result of hypertrophic pachymeningitis in C-ANCA-positive Wegener's granulomatosis. Med Klin (Munich) 100:209-12.
  7. McCarthy PJ, Arend WP, Kleinschmidt-DeMasters BK. (2001) 32 year old female with dural mass encircling cervical spinal cord. Brain Pathol. 11: 483-4, 487.
  8. Scarrow AM, Segal R, Medsger TA Jr, Wasko MC (1998) Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener's granulomatosis: case report and literature review. Neurosurgery 43:1470-3
  9. Spísek R, Kolouchová E, Jensovsky J, Rusina R, Fendrych P, Plas J, Bartunková J (2006). Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis. Clin Rheumatol 25:739- 42
  10. Wawrzynska L, Wiatr E, Chabowski M, Bestry I, Kurzyna M, Torbicki A (2008) Wegener's granulomatosis presenting with ischaemic stroke. Pneumonol Alergol Pol 76:180-5.

Contributed by Malgorzata Kolodziej, Ludwig Benes, Christopher Nimsky, Hans-Eckart Schaefer, Axel Pagenstecher

Case IndexCME Case StudiesFeedbackHome