Nervous System Vasculitis

  • M. Habib Bouhaouala
  • Mehdi Charfi
  • Wassim Saîd
  • Slim El Fourti
  • Lotfi Hendaoui
Part of the Medical Radiology book series (MEDRAD)


The central nervous system (CNS) and the peripheral nervous system (PNS) represent the major target organs in systemic vasculitis and their involvement is often present at the onset of the disease. CNS vasculitis affects typically the middle cerebral artery territory. The diagnosis remains largely dependent on MRI, angiography and biopsy. CSF analysis is also an integral part of the diagnostic process. On MRI, CNS vasculitis is suspected in view of a multiple and bilateral infarctions of different ages in the cortico-subcortical junction with intra- and extra-axial hemorrhages. Etiological diagnosis is oriented by extraneurological signs, imaging findings, topography of the lesions and biopsy. PNS vasculitis occurs more frequently in medium-sized and small vessels. The diagnosis is based on anamnesis, physical examination, electromyography and electro-physiological nerve conduction studies. The confirmation of diagnosis depends on tissue biopsy.


Peripheral Nervous System Systemic Vasculitis Etiological Diagnosis Middle Cerebral Artery Territory Central Nervous System Manifestation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Autret A, De Toffol B, Saudeau D, Fetissof F, Guilmot JL (1992) Manifestations neurologiques des vascularites. EMC (Elsevier Masson SAS, Paris), Neurologie, 17-170-A-10Google Scholar
  2. Cacoub P, Musset L, Piette JC (1998) Cryoglobulinémies. EMC (Elsevier Masson SAS, Paris), Akos Encyclopédie pratique de médecine, 5-0455Google Scholar
  3. Caselli RJ, Hunder GG (1994) Neurologic complications of giant cell (temporal) arteritis. Semin Neurol 14:349–353PubMedCrossRefGoogle Scholar
  4. Chakravarty K (1997) Vasculitis by organ systems. Baillieres Clin Rheum 2:357–393CrossRefGoogle Scholar
  5. Davies DJ (2005) Small vessel vasculitis. Cardiovasc Pathol 14:335–346PubMedCrossRefGoogle Scholar
  6. Defuentes G, Mangouka L, Chenilleau MC, Potet J, Cremades S, Perrier E, Garcin JM, Berets O (2008) Thromboang Acite de Buerger. EMC (Elsevier Masson SAS, Paris), Podologie, 27-110-A-20Google Scholar
  7. Do TH, Fisch C, Evoy F (2004) Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol 25:382–388PubMedGoogle Scholar
  8. Du-Boutin LTH, Wechsler B (2009) Neuro-Behçet. EMC (Elsevier Masson SAS, Paris), Neurologie, 17-169-A-10Google Scholar
  9. Ellis SG, Verity MA (1979) Central nervous system involvement in systemic lupus erythematosus: a review of neuropathologic findings in 57 cases 1955–77. Semin Arthritis Rheum 8:212–221PubMedCrossRefGoogle Scholar
  10. Guillevin L, Durand-Gasselin B (1999) Principales vascularites nécrosantes systémiques. EMC (Elsevier SAS, Paris), Appareil locomoteur, 14-245-F-10Google Scholar
  11. Guillevin L, Pagnoux C, Cevallos R et al (2006) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430CrossRefGoogle Scholar
  12. Jayne D (2009) The diagnosis of vasculitis. Best Pract Res Clin Rheum 23:445–453CrossRefGoogle Scholar
  13. Johnson RT, Richardson EP (1968) The neurological manifestations of systemic lupus erythematosus. Medicine (Baltimore) 47:337–369CrossRefGoogle Scholar
  14. Joseph FG, Scolding NJ (2002) Cerebral vasculitis: a practical approach. Pract Neurol 2:80–93CrossRefGoogle Scholar
  15. Karni A, Sadeh M, Blatt I, Goldhammer Y (1991) Cogan’s syndrome complicated by lacunar brain infarcts. J Neurol Neurosurg Psychiatry 54:169–171PubMedCrossRefGoogle Scholar
  16. Khasnis A, Langford CA (2009) Update on vasculitis. J Allergy Clin Immunol 123:1226–1236PubMedCrossRefGoogle Scholar
  17. Langford CA (2010) Vasculitis. J Allergy Clin Immunol 2:S216–S225CrossRefGoogle Scholar
  18. Laraki R (2008) Maladie de Takayasu. EMC (Elsevier Masson SAS, Paris), Angéiologie, 19-1520Google Scholar
  19. Lopez-Yunez AM, Garg BR (2001) Noninfectious cerebral vasculitis in children. Semin Cerebrovasc Dis Stroke 1:249–263CrossRefGoogle Scholar
  20. Moore PM, Richardson B (1998) Neurology of the vasculitides and connective tissue diseases. J Neurol Neurosurg Psychiatry 65:10–22PubMedCrossRefGoogle Scholar
  21. Moritani T, Hiwatashi A, Shrier DA, Wang HZ, Numaguchi Y, Westesson PLA (2004) CNS vasculitis and vasculopathy efficacy and usefulness of diffusion-weighted echoplanar MRI. J Clin Imaging 28:261–270CrossRefGoogle Scholar
  22. Mrabet D, Meddeb N, Ajlani H, Sahli H, Sellami S (2007) Cerebral vasculitis in a patient with rheumatoid arthritis. Rev Rhum 74:301–304CrossRefGoogle Scholar
  23. Nader J, Bogousslavsky J, Del Mar Saez De Ocariz M (1996) Vasculites cérébrales infectieuses. EMC (Elsevier Masson SAS, Paris), Neurologie 17-046-B-05Google Scholar
  24. Piette AM, Arnaud L, Blétry O (2006) Maladie de Takayasu. EMC (Elsevier Masson SAS, Paris), Cardiologie, 11-610-A-10Google Scholar
  25. Ponge T, JH Barrier (1999) Maladie de Horton. EMC (Elsevier Masson SAS, Paris), Akos Encyclopédie Pratique de Médecine 5-0365, Angéiologie, 19-1810Google Scholar
  26. Puechal X (2007) Vascularites associées aux anticorps anticytoplasme des polynucléaires. Rev Rhum 74:824–832CrossRefGoogle Scholar
  27. Rossi CM, Comite GD (2009) The clinical spectrum of the neurological involvement in vasculitides. J Neurol Sci 285:13–21PubMedCrossRefGoogle Scholar
  28. Silvera S, Vignaux O, Legman p (2007) Imagerie de l’atteinte ORL et cérébrale de la maladie de Wegener. Presse Med 36:913–921PubMedCrossRefGoogle Scholar
  29. Watts RA, Mooney J, Lane SE, Scott DG (2004) Rheumatoid vasculitis: becoming extinct. J Rheum 43:920–923CrossRefGoogle Scholar
  30. Wechsler B, Du-Boutin LTH, Amoura Z (2009) Maladie de Behçet. EMC (Elsevier Masson SAS, Paris), Appareil locomoteur, 14-206-B-10Google Scholar
  31. Zuber M, Blustajn J, Arquizan C, Trystram D, Mas JL, Meder JF (1999) Angiitis of the CNS. J Neuroradiol 26:101–117PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • M. Habib Bouhaouala
    • 1
  • Mehdi Charfi
    • 1
  • Wassim Saîd
    • 1
  • Slim El Fourti
    • 1
  • Lotfi Hendaoui
    • 2
  1. 1.Department of Medical Imaging, Medical School of TunisInterior Security Forces Teaching HospitalLa MarsaTunisia
  2. 2.Department of Medical ImagingImaging Guided Interventions Mongi Slim University HospitalLa MarsaTunisia

Personalised recommendations