Skip to main content
SpringerLink
Log in

Churg-Strauss-Syndrom mit dem klinischen Bild eines Guillain-Barré-Syndroms

A patient with Churg-Strauss syndrome presenting as Guillain-Barré syndrome

  • Kasuistiken
  • Published:
Der Nervenarzt Aims and scope Submit manuscript

Zusammenfassung

Ein 74-jähriger Mann entwickelte in einer Lungenklinik eine symmetrische, aufsteigende Tetraparese, die klinisch und neurophysiologisch zunächst als ein Guillain-Barré-Syndrom (GBS) eingestuft wurde. Ein erster Therapiezyklus mit 7s-Globulinen blieb erfolglos. Die Symptomatik verlief progredient bis zur Gangunfähigkeit. Im weiteren Verlauf fiel laborchemisch eine Eosinophilie (41%) auf. Wegen der Kombination des neu aufgetretenen Asthma bronchiale und Eosinophilie wurde der Verdacht auf ein Churg-Strauss-Syndroms (CSS) gestellt. Trotz hochdosierter Kortikoidmedikation besserte sich die Symptomatik nicht. Erst eine Immunsuppression mit Cyclophosphamid bewirkte eine deutliche Rückbildung der Paresen und Sensibilitätsstörungen.

Als Differenzialdiagnose bei einer rasch aufsteigenden, symmetrischen Neuropathie muss an die Untergruppe der nekrotisierenden Vaskulitiden gedacht werden. Das frühe Erkennen dieses Krankheitsbildes ist wichtig, weil es in der Regel gut auf eine rechtzeitige immunsuppressive Behandlung anspricht, jedoch bei Therapieverzögerung tödlich verlaufen kann.

Summary

A 74-year-old man presented in a pulmonary clinic with symmetrically ascending tetraparesis. Physical and neurophysiological examinations suggested Guillain-Barré syndrome. The patient was treated with an initial course of 7s immunoglobulins without success. His state worsened until he was unable to walk. Severe eosinophilia (41%) was later noted in the differential white blood cell count. Combined with the onset of asthma-like symptoms, this prompted us to suspect Churg-Strauss syndrome. Despite treatment with high-dose corticoids, the palsy did not improve. It was only after immunosuppression with cyclophosphamide that the patient began to recover. The subgroup of necrotising vasculitides must be considered as differential diagnosis of rapidly progressive, symmetrical neuropathy with ascending course. Early identification and treatment are essential, since early immunosuppressive therapy is often successful, whereas delayed initiation of treatment may lead to a fatal outcome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3

Literatur

  1. Abu-Shakra M, Smythe H, Lewtas J et al. (1994) Outcome of polyarteritis nodosa and Churg-Strauss syndrome. An analysis of twenty-five patients. Arthritis Rheum 37: 1798–1803

    Article  PubMed  CAS  Google Scholar 

  2. Bazzi P, Tancredi L, Scarpini E et al. (2000) Severe polyneuropathy in a patient with Churg-Strauss syndrome. J Peripher Nerv Syst 5: 106–110

    Article  PubMed  CAS  Google Scholar 

  3. Churg J, Strauss L (1951) Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 27: 277–301

    PubMed  CAS  Google Scholar 

  4. Davies L, Spies JM, Pollard JD, McLeod JG (1996) Vasculitis confined to peripheral nerves. Brain 119: 1441–1448

    Article  PubMed  Google Scholar 

  5. Dyck PJ (1988) Detection, chrarcterisation and staging of polyneuropathie assessed in diabetics. Muscle Nerve 11: 21–32

    Article  PubMed  CAS  Google Scholar 

  6. Engelhardt A, Lorler H, Neundorfer B (1993) Immunohistochemical findings in vasculitic neuropathies. Acta Neurol Scand 87: 318–321

    PubMed  CAS  Google Scholar 

  7. Fauci AS, Katz P, Haynes BF, Wolff SM (1979) Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 301: 235–238

    Article  PubMed  CAS  Google Scholar 

  8. Fujimura H, Lacroix C, Said G (1991) Vulnerability of nerve fibres to ischaemia. A quantitative light and electron microscope study. Brain 114: 1929–1942

    Article  PubMed  Google Scholar 

  9. Hattori N, Ichimura M, Nagamatsu M et al. (1999) Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain 122: 427–439

    Article  PubMed  Google Scholar 

  10. Hawke SH, Davies L, Pamphlett R et al. (1991) Vasculitic neuropathy. A clinical and pathological study. Brain 114: 2175–2190

    Article  PubMed  Google Scholar 

  11. Kaneki T, Kawashima A, Hayano T et al. (1998) Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer. J Gastroenterol 33: 112–116

    Article  PubMed  CAS  Google Scholar 

  12. Masi AT, Hunder GG, Lie JT et al. (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33: 1094–1100

    Article  PubMed  CAS  Google Scholar 

  13. Noth I, Strek ME, Leff AR (2003) Churg-Strauss syndrome. Lancet 361: 587–594

    Article  PubMed  Google Scholar 

  14. Nusslein HG, Baenkler HW, Stommer P et al. (1986). Polyneuropathy in allergic granulomatosis and angiitis (Churg-Strauss syndrome). Nervenarzt 57: 140–144

    PubMed  CAS  Google Scholar 

  15. Ohno S, Suzuki T, Hara K et al. (1990) A case of suspected allergic granulomatosis and angiitis with a rapid clinical course of paraplegia. Arerugi 39: 701–707

    PubMed  CAS  Google Scholar 

  16. Rosenthal G, Schneck M, Lifshitz T (2002) Branch retinal vein occlusion in Churg-Strauss syndrome. Clin Experiment Ophthalmol 30: 381–382

    Article  PubMed  Google Scholar 

  17. Sehgal M, Swanson JW, DeRemee RA, Colby TV (1995) Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc 70: 337–341

    Article  PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Klinik für Neurologie, Georg-August-Universität, Robert-Koch-Straße 40, 37075, Göttingen, Deutschland

    M. Djukic, H. Schmidt & R. Nau

  2. Klinik für Neurologie, Klinikum Kassel, Kassel, Deutschland

    C. Mazurek

  3. Abteilung Neuropathologie, Georg-August-Universität, Göttingen, Deutschland

    F. König

  4. Abteilung Pathologie, Georg-August-Universität, Göttingen, Deutschland

    S. Schweyer

Authors
  1. M. Djukic
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H. Schmidt
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. C. Mazurek
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. F. König
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Schweyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. R. Nau
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to M. Djukic.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Djukic, M., Schmidt, H., Mazurek, C. et al. Churg-Strauss-Syndrom mit dem klinischen Bild eines Guillain-Barré-Syndroms. Nervenarzt 79, 457–461 (2008). https://doi.org/10.1007/s00115-007-2377-y

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00115-007-2377-y

chlüsselwörter

Keywords

Search

Navigation