Journal of Neurology

, Volume 256, Issue 3, pp 299–304

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

REVIEW

Abstract

The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener’s granulomatosis. One patient developed de novo 6th nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging with CT and MRI and the laboratory provided no clues suggesting active vasculitis. However, in both patients the neuropathies fully recovered in response to standard induction protocols of vasculitis. In the absence of organspecific proof of vasculitis, these treatment decisions were guided by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs.

Key words

ANCA systemic vasculitis abducens nerve palsy vagal nerve palsy Wegener’s granulomatosis cranial neuropathy 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE (1993) Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 33(1):4–9PubMedCrossRefGoogle Scholar
  2. 2.
    Burns TM, Schaublin GA, Dyck PJ (2007) Vasculitic neuropathies. Neurol Clin 25(1):89–113PubMedCrossRefGoogle Scholar
  3. 3.
    De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, Gross WL, Luqmani R, Jayne DR (2005) Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibodyassociated vasculitis. Arthritis Rheum 52(8):2461–2469PubMedCrossRefGoogle Scholar
  4. 4.
    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG (1994) Nomenclature of systemic vasculitides – proposal of an international consensus conference. Arthritis Rheum 39:189–195Google Scholar
  5. 5.
    Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K, D’Cruz D, Guillevin L, Harper L, Jayne D, Luqmani R, Mooney J, Scott D on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group (2007) BSR and BHPR guidelines for the management of adults with ANCA associeated vasculitis. Rheumatology 46:1–11CrossRefGoogle Scholar
  6. 6.
    Jennette JC, Falk RJ (1997) Smallvessel vasculitis. N Engl J Med 337(21):1512–1523PubMedCrossRefGoogle Scholar
  7. 7.
    Cattaneo L, Chierici E, Pavone L, Grasselli C, Manganelli P, Buzio C, Pavesi G (2007) Peripheral neuropathy in Wegener’s granulomatosis, Churg- Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry 78(10):1119–1123PubMedCrossRefGoogle Scholar
  8. 8.
    Schaublin GA, Michet JR Jr, Dyck PJ, Burns TM (2005) An update on the classification and treatment of vasculitis neuropathy. Lancet Neurol 4(12):853–865PubMedCrossRefGoogle Scholar
  9. 9.
    Daderian AD, Chayasirisobhon S (2000) An unusual case of multiple cranial nerve palsies in Wegener’s granulomatosis. J Natl Med Assoc 92(9):455–457PubMedGoogle Scholar
  10. 10.
    Gorson KC (2007) Vasculitis neuro pathies: an update. Neurologist 13(1):12–19PubMedCrossRefGoogle Scholar
  11. 11.
    Nishino H, Rubino FA (1993) Horner’s syndrome in Wegener’s granulomatosis: report of four cases. J Neurol Neurosurg Psychiatry 56(8):897–899PubMedCrossRefGoogle Scholar
  12. 12.
    Newman NJ, Slamovits TL, Friedland S, Wilson WB (1995) Neuro-ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener’s granulomatosis. Am J Ophthalmol 120(5):613–621PubMedGoogle Scholar
  13. 13.
    Heuss D, Schlotter-Weigel B, Sommer C; German centers for neuromuscular disease (2003) Diagnosis and therapy of vasculitic neuropathy. Consensus statement of the German centers for neuromuscular disease. Fortschr Neurol Psychiatr 71(4):172–186PubMedCrossRefGoogle Scholar
  14. 14.
    Sakurazawa M, Katsumata T, Kunugi S, Katsura K, Sakamoto S, Katayama Y (2007) Case report of Wegener’s granulomatosis presenting with multiple cranial nerve palsy and hypertrophic cranial pachymeningitis. Rinsho Shinkeigaku 47(2–3):85–89PubMedGoogle Scholar
  15. 15.
    Kamimura T, Shimazaki H, Morita M, Nakano I, Okazaki H, Minota S (2006) Limited Wegener’s granulomatosis manifested by abducens nerve palsy resulting from pachymeningitis. J Clin Rheumatol 12(5):259–260PubMedCrossRefGoogle Scholar
  16. 16.
    Tsuda H, Ishikawa H, Majima T, Sawada U, Mizutani T (2005) Isolated oculomotor nerve palsy in Churg- Strauss syndrome. Intern Med 44(6):638–640PubMedCrossRefGoogle Scholar
  17. 17.
    Shiotani A, Mukobayashi C, Oohata H, Yamanishi T, Hara T, Itoh H, Nishioka S (1997) Wegener’s granulomatosis with dural involvement as the initial clinical manifestation. Intern Med 36(7):514–518PubMedCrossRefGoogle Scholar
  18. 18.
    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 (4):209–212CrossRefGoogle Scholar
  19. 19.
    Spranger M, Schwab S, Meinck HM, Tischendorf M, Sis J, Breibart A, Andrassy K (1997) Meningeal involvement in Wegener’s granulomatosis confirmed and monitored by positive circulating antineutrophil cytoplasm in cerebral fluid. Neurology 48:263–265PubMedGoogle Scholar
  20. 20.
    Jacobi D, Maillot F, Mommet C, Arsene S, Cottier JP, Lamisse F, Guillevin L (2005) P-ANCA cranial pachymeningitis: a case report. Clin Rheumatol 24(2):174–177PubMedCrossRefGoogle Scholar
  21. 21.
    Nagashima T, Maguchi S,Terayama Y, Horimoto M, Nemoto M, Nunomura M, Mori M, Seki T, Matsukawa S, Itoh T, Nagashima K (2000) P-ANCA-positive Wegener’s granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature. Neuropathology 20(1):23–30PubMedCrossRefGoogle Scholar
  22. 22.
    Akahoshi M, Yoshimoto G, Nakashima H, Miyake K, Inoue Y, Tanaka Y, Tsukamoto H, Horiuchi T, Otsuka T, Harada M (2004) MPO-ANCA-positive Wegener’s granulomatosis presenting with hypertrophic cranial pachymeningitis: case report and review of the literature. Mod Rheumatol 14(2): 179–183PubMedCrossRefGoogle Scholar
  23. 23.
    Lopez-Rodriguez R, Garcia-Gonzalez J, Campos-Franco J, Mallo-Gonzalez N, Alende-Sixto MR (2007) Neurological involvement in Wegener’s granulomatosis: report of one case. Rev Med Chil 135(7):913–916PubMedGoogle Scholar
  24. 24.
    Sugiyama Y, Shimizu M, Hoshi A, Aoki T, Matsuura Y, Tochikubo S, Shimizu S, Muroi A, Honma M, Yamamoto T (1999) An old man presenting with fluctuating bilateral multiple cranial nerve palsies and positive test for perinuclear antineutrophil cytoplasmic antibody. No To Shinkei 51(9):825–832PubMedGoogle Scholar
  25. 25.
    von Bierbrauer A, Pelzer C, Fischer V (2008) Severe chronic headache with depression induced by pachymeningitis in Wegener’s granulomatosis. Dtsch Med Wochenschr 133(5):185–188PubMedCrossRefGoogle Scholar
  26. 26.
    Salvarani C, et al. (2008) Primary central nervous system vasculitis with prominent leptomeningeal enhancement. Arthritis & Rhematism 58:595–603CrossRefGoogle Scholar
  27. 27.
    Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, Lesavre P, Ludemann J, Rasmussen N, Sinico RA, Wiik A, van der Woude FJ (1998) Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int 53:743–753PubMedCrossRefGoogle Scholar
  28. 28.
    Nowack R, Grab I, Flores-Suarez LF, Schnulle P, Yard B, van der Woude FJ (2001) ANCA-titres, even of IgG subclasses, and soluble CD14 fail to predict relapses in patients with ANCA-associated vasculitis. Nephrol Dial Transplant 16:1631–1637PubMedCrossRefGoogle Scholar
  29. 29.
    Sanders JS, Huitma MG, Kallenberg CG, Stegeman CA (2006) Prediction of relapses in PR3-ANCA-associated vasculitis by assessing responses of ANCA titres to treatment. Rheumatology (Oxford) 45(6):724–729PubMedCrossRefGoogle Scholar
  30. 30.
    Hogan SL, Falk RJ, Chin H, Cai J, Jennette CE, Jennette JC, Nachman PH (2005) Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 143(9):621–631PubMedGoogle Scholar
  31. 31.
    Morinaga A, Ono K, Komai K, Yamada M (2007) Microscopic polyangiitis presenting with temporal arteritis and multiple cranial neuropathies. J Neurol Sci 256(1–2):81–83PubMedCrossRefGoogle Scholar
  32. 32.
    Knight A, Ekbom A, Brandt L, Askling J (2006) Increasing incidence of Wegener’s granulomatosis in Sweden, 1975– 2001. J Rheumatol 33(10):2060–2063PubMedGoogle Scholar
  33. 33.
    Mahr AD, Neogi T, Merkel PA (2006) Epidemiology of Wegener’s granulomatosis: lessons from descriptive studies and analyses of genetic and environmental risk determinants. Clin Exp Rheumatol 24(Suppl 41):S82–S91PubMedGoogle Scholar
  34. 34.
    Mohammad AJ, Jacosson LT, Mahr AD, Sturfelt G, Segelmark M (2007) Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg Strauss Syndrome within a defined population in southern Sweden. Rheumatology 46(8):1329–1337PubMedCrossRefGoogle Scholar
  35. 35.
    Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert J-W, Dadoniene J, Ekstrand A, Gaskin G, Gregorini G, de Groot K, Hagen EC, Mirapeix E, Pettersson E, Siegert C, Sinico A, Tesar V, Westman K, Pusey C and the European Vasculitis Study Group (EUVAS) (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349(1):36–44PubMedCrossRefGoogle Scholar
  36. 36.
    Jayne DRW, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L, Mirapeix E, Savage COS, Sinico RA, Stegeman CO, Westman KW, van der Woude FJ, de Lind van Wijngaarden RAF, Pusey CD; on behalf of the European Vasculitis Study Group (2007) Randomized Trial of Plasma Exchange or High-Dosage Methylprednisolone as Adjunctive Therapy for Severe Renal Vasculitis. J Am Soc Nephrol 18:2180–2188PubMedCrossRefGoogle Scholar

Copyright information

© Steinkopff-Verlag 2009

Authors and Affiliations

  1. 1.Nephrology/Dialysis centre LindauLindauGermany
  2. 2.Neurologische GemeinschaftspraxisRavensburgGermany
  3. 3.Ear, Nose & Throat DepartmentCopenhagenDenmark

Personalised recommendations