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Neuroradiologie Scan 2015; 05(02): 125-150
DOI: 10.1055/s-0034-1391774
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© Georg Thieme Verlag KG Stuttgart · New York

Spektrum der Bildgebungsbefunde bei ZNS-Vaskulitis[1]

Imaging spectrum of CNS vasculitis
Ahmed A. K. Abdel Razek
,
Hortensia Alvarez
,
Stephen Bagg
,
Sherif Refaat
,
Mauricio Castillo
Further Information

Publication History

Publication Date:
01 April 2015 (online)

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Zusammenfassung

Kennzeichen der zerebralen Vaskulitis ist eine Entzündung der Gefäßwände, von der Blutgefäße jeder Größe betroffen sein können. Die Pathogenese der Vaskulitis ist noch immer zum großen Teil ungeklärt. Erkranken können große (Takayasu-Arteriitis, Riesenzellarteriitis), mittelgroße (Kawasaki-Krankheit, Polyarteriitis nodosa), kleine (Immunglobulin-A-Vaskulitis, mikroskopische Polyangiitis, Granulomatose mit Polyangiitis, eosinophile Granulomatose mit Polyangiitis) oder variabel große Gefäße (Behçet-Krankheit, Cogan-Syndrom). Die primäre Angiitis des Zentralnervensystems ist eine idiopathische Erkrankung, die nicht mit einer generalisierten Entzündung einhergeht und den reversiblen zerebralen Vasokonstriktionssyndromen ähnelt. Die Vaskulitis kann Folge einer systemischen Erkrankung, einer Infektion oder eines Malignoms sein, aber auch durch den Konsum von Medikamenten oder Drogen oder durch eine Strahlentherapie ausgelöst werden. Die Bildgebungsbefunde reichen von leichten ischämischen Veränderungen bis hin zu deutlich sichtbarem Infarkt, Blutungen und einem Ödem der weißen Substanz; nach Kontrastmittelgabe kann eine Anreicherung zu beobachten sein. Die Hirnarterien können ein perlschnurartiges Aussehen annehmen und unterschiedlich stark ausgeprägte Stenosen und Verschlüsse aufweisen. Nach Kontrastmittelgabe kann eine Kontrastmittelanreicherung der Gefäßwände zu beobachten sein. Die Interpretation der Bildgebungsbefunde in Zusammenhang mit dem klinischen Bild und den Laborergebnissen hilft bei der Diagnose einer Vaskulitis des Zentralnervensystems.

Abstract

Cerebral vasculitis is characterized by inflammation of the walls of blood vessels and may affect vessels of any size. The pathogenesis of vasculitis remains poorly understood. Vasculitis may affect large vessels (Takayasu arteritis, giant cell arteritis), medium-sized vessels (Kawasaki disease, polyarteritis nodosa), small vessels (immunoglobulin A vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis), or variable-sized vessels (Behçet disease, Cogan syndrome). Primary angiitis of the central nervous system (CNS) is an idiopathic disorder with no evidence of generalized inflammation that may simulate reversible cerebral vasoconstriction syndromes. Vasculitis may be secondary to systemic disease, infection, malignancy, drug use, or radiation therapy. Imaging findings vary from small ischemic changes to frank infarction, hemorrhage, and white matter edema and may show contrast material enhancement. The cerebral arteries may demonstrate a beaded appearance with variable degrees of stenosis, occlusion, and contrast enhancement of the vessel wall. Correlation of imaging findings with clinical presentation and laboratory test results helps establish the diagnosis of CNS vasculitis.

1 © 2014 The Radiological Society of North America. All rights reserved. Originally puplished in English in RadioGraphics 2014; 34: 873 – 894. Online published in 10.1148 /rg.344135028. Translated and reprinted with permission of RSNA. RSNA is not responsible for any inaccuracy or error arising from the translation from English to German.


 

  • Literatur

  • 1 Garg A. Vascular brain pathologies. Neuroimaging Clin N Am 2011; 21: 897-926, ix
    CrossrefGoogle Scholar
  • 2 Bouhaouala MH, Charfi M, Saîd W et al. Nervous system vasculitis. In: Hendaoui L, Stanson AW, Bouhaouala MH, Joffre F, eds. Systemic vasculitis: imaging features. Medical Radiology/Diagnostic Imaging series. Berlin: Springer; 2012: 415-423
    CrossrefGoogle Scholar
  • 3 Néel A, Pagnoux C, Guillevin L et al. Central nervous system vasculitides: an update [in French]. Rev Med Interne 2012; 33: 381-389
    CrossrefGoogle Scholar
  • 4 Alba MA, Espígol-Frigolé G, Prieto-González S et al. Central nervous system vasculitis: still more questions than answers. Curr Neuropharmacol 2011; 9: 437-448
    CrossrefGoogle Scholar
  • 5 Berlit P. Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord 2010; 3: 29-42
    CrossrefPubMedGoogle Scholar
  • 6 Hajj-Ali RA, Calabrese LH. Central nervous system vasculitis. Curr Opin Rheumatol 2009; 21: 10-18
    CrossrefGoogle Scholar
  • 7 Rossi CM, Di Comite G. The clinical spectrum of the neurological involvement in vasculitides. J Neurol Sci 2009; 285: 13-21
    CrossrefGoogle Scholar
  • 8 Schedel J, Kuchenbuch S, Schoelmerich J et al. Cerebral lesions in patients with connective tissue diseases and systemic vasculitides: Are there specific patterns?. Ann N Y Acad Sci 2010; 1193: 167-175
    CrossrefGoogle Scholar
  • 9 Drier A, Bonneville F, Haroche J et al. Central nervous system involvement in systemic diseases: spectrum of MRI findings [in French]. J Neuroradiol 2010; 37: 255-267
    CrossrefGoogle Scholar
  • 10 Küker W. Cerebral vasculitis: imaging signs revisited. Neuroradiology 2007; 49: 471-479
    CrossrefPubMedGoogle Scholar
  • 11 Pipitone N, Versari A, Salvarani C. Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 2008; 47: 403-408
    Google Scholar
  • 12 Küker W. Imaging of cerebral vasculitis. Int J Stroke 2007; 2: 184-190
    CrossrefPubMedGoogle Scholar
  • 13 Jennette JC, Falk RJ, Andrassy K et al. Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 1994; 37: 187-192
    CrossrefPubMedGoogle Scholar
  • 14 Jennette JC, Falk RJ, Bacon PA et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11
    CrossrefGoogle Scholar
  • 15 Poels MM, Ikram MA, Vernooij MW. Improved MR imaging detection of cerebral microbleeds more accurately identifies persons with vasculopathy. AJNR Am J Neuroradiol 2012; 33: 1553-1556
    CrossrefGoogle Scholar
  • 16 Wintermark M, Sesay M, Barbier E et al. Comparative overview of brain perfusion imaging techniques. Stroke 2005; 36: e83-e99
    CrossrefGoogle Scholar
  • 17 Demaerel P, De Ruyter N, Maes F et al. Magnetic resonance angiography in suspected cerebral vasculitis. Eur Radiol 2004; 14: 1005-1012
    CrossrefGoogle Scholar
  • 18 Swartz RH, Bhuta SS, Farb RI et al. Intracranial arterial wall imaging using high-resolution 3-tesla contrast-enhanced MRI. Neurology 2009; 72: 627-634
    CrossrefGoogle Scholar
  • 19 O’Brien WT Sr, Vagal AS, Cornelius RS. Applications of computed tomography angiography (CTA) in neuroimaging. Semin Roentgenol 2010; 45: 107-115
    CrossrefGoogle Scholar
  • 20 Cianfoni A, Colosimo C, Basile M et al. Brain perfusion CT: principles, technique and clinical applications. Radiol Med (Torino) 2007; 112: 1225-1243
    CrossrefGoogle Scholar
  • 21 Pacheco CastellanosM del C, Mínguez VegaM, Martínez CaballeroA et al. Early diagnosis of large vessel vasculitis: usefulness of positron emission tomography with computed tomography. Rheumatol Clin 2013; 9: 65-68
    Google Scholar
  • 22 De Miguel E, Roxo A, Castillo C et al. The utility and sensitivity of colour Doppler ultrasound in monitoring changes in giant cell arteritis. Clin Exp Rheumatol 2012; 30: 34-S38
    Google Scholar
  • 23 Mavrogeni S, Dimitroulas T, Chatziioannou SN et al. The role of multimodality imaging in the evaluation of Takayasu arteritis. Semin Arthritis Rheum 2013; 42: 401-412
    CrossrefGoogle Scholar
  • 24 Khandelwal N, Kalra N, Garg MK et al. Multidetector CT angiography in Takayasu arteritis. Eur J Radiol 2011; 77: 369-374
    CrossrefGoogle Scholar
  • 25 Garg SK, Mohan S, Kumar S. Diagnostic value of 3D contrast-enhanced magnetic resonance angiography in Takayasu’s arteritis: a comparative study with digital subtraction angiography. Eur Radiol 2011; 21: 1658-1666
    CrossrefGoogle Scholar
  • 26 Abdel RazekA, Saad E, Soliman N et al. Assessment of vascular disorders of the upper extremity with contrast-enhanced MR angiography: pictorial review. Jpn J Radiol 2010; 28: 87-94
    CrossrefGoogle Scholar
  • 27 Koenigkam-Santos M, Sharma P, Kalb B et al. Magnetic resonance angiography in extracranial giant cell arteritis. J Clin Rheumatol 2011; 17: 306-310
    CrossrefGoogle Scholar
  • 28 Geiger J, Bley T, Uhl M et al. Diagnostic value of T2-weighted imaging for the detection of superficial cranial artery inflammation in giant cell arteritis. J Magn Reson Imaging 2010; 31: 470-474
    CrossrefGoogle Scholar
  • 29 Stanzani L, Fusi L, Gomitoni A et al. A case of posterior reversible encephalopathy during polyarteritis nodosa vasculitis. Neurol Sci 2008; 29: 163-167
    CrossrefGoogle Scholar
  • 30 Provenzale JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. AJNR Am J Neuroradiol 1996; 17: 1119-1126
    Google Scholar
  • 31 Okanishi T, Enoki H. Transient subcortical highsignal lesions in Kawasaki syndrome. Pediatr Neurol 2012; 47: 295-298
    CrossrefGoogle Scholar
  • 32 Alves NR, Magalhães CM, Almeida Rde F et al. Prospective study of Kawasaki disease complications: review of 115 cases. Rev Assoc Med Bras 2011; 57: 295-300
    CrossrefGoogle Scholar
  • 33 Garzoni L, Vanoni F, Rizzi M et al. Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature. Rheumatology (Oxford) 2009; 48: 1524-1529
    CrossrefGoogle Scholar
  • 34 Zhang HL, Wu J. Posterior reversible encephalopathy syndrome associated with Henoch-Schönlein purpura. Pediatr Emerg Care 2010; 26: 966
    Google Scholar
  • 35 Ku BD, Shin HY. Multiple bilateral non-hemorrhagic cerebral infarctions associated with microscopic polyangiitis. Clin Neurol Neurosurg 2009; 111: 904-906
    CrossrefGoogle Scholar
  • 36 Yamashiro K, Mori A, Shimada Y et al. Gradient echo T2*-weighted magnetic resonance imaging revealing cerebral microbleeds in a patient with microscopic polyangiitis complicated by cerebrovascular disease. J Stroke Cerebrovasc Dis 2012; 21: e7-e9
    Google Scholar
  • 37 Chen NC, Lai PH, Fang HC et al. Microscopic polyangiitis with an initial presentation of pontine infarction. Am J Med Sci 2012; 344: 163-165
    CrossrefGoogle Scholar
  • 38 Yong TY, Li JY, Amato L et al. Pituitary involvement in Wegener’s granulomatosis. Pituitary 2008; 11: 77-84
    CrossrefGoogle Scholar
  • 39 Spísek R, Kolouchová E, Jensovský J et al. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis. Clin Rheumatol 2006; 25: 739-742
    CrossrefGoogle Scholar
  • 40 Razek AA, Castillo M. Imaging appearance of granulomatous lesions of head and neck. Eur J Radiol 2010; 76: 52-60
    CrossrefGoogle Scholar
  • 41 Razek AA, Huang BY. Lesions of the petrous apex: classification and findings at CT and MR imaging. RadioGraphics 2012; 32: 151-173
    CrossrefGoogle Scholar
  • 42 Mencacci NE, Bersano A, Cinnante CM et al. Intracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome: case report and review of the literature. J Neurol Sci 2011; 301: 107-111
    CrossrefGoogle Scholar
  • 43 Wolf J, Bergner R, Mutallib S et al. Neurologic complications of Churg-Strauss syndrome: a prospective monocentric study. Eur J Neurol 2010; 17: 582-588
    CrossrefPubMedGoogle Scholar
  • 44 Tokumaru AM, Obata T, Kohyama S et al. Intracranial meningeal involvement in Churg-Strauss syndrome. AJNR Am J Neuroradiol 2002; 23: 221-224
    Google Scholar
  • 45 Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8: 192-204
    CrossrefPubMedGoogle Scholar
  • 46 Borhani HaghighiA, Sarhadi S, Farahangiz S. MRI findings of neuro-Behcet’s disease. Clin Rheumatol 2011; 30: 765-770
    CrossrefGoogle Scholar
  • 47 Siva A, Saip S. The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol 2009; 256: 513-529
    CrossrefGoogle Scholar
  • 48 Antonios N, Silliman S. Cogan syndrome: an analysis of reported neurological manifestations. Neurologist 2012; 18: 55-63
    CrossrefGoogle Scholar
  • 49 Albayram MS, Wityk R, Yousem DM et al. The cerebral angiographic findings in Cogan syndrome. AJNR Am J Neuroradiol 2001; 22: 751-754
    Google Scholar
  • 50 Hajj-Ali RA, Calabrese LH. Primary angiitis of the central nervous system. Autoimmun Rev 2013; 12: 463-466
    CrossrefGoogle Scholar
  • 51 Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis: an update. Curr Opin Rheumatol 2012; 24: 46-52
    CrossrefGoogle Scholar
  • 52 Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet 2012; 380: 767-777
    CrossrefGoogle Scholar
  • 53 Zuccoli G, Pipitone N, Haldipur A et al. Imaging findings in primary central nervous system vasculitis. Clin Exp Rheumatol 2011; 29: S104-S109
    Google Scholar
  • 54 Ay H, Sahin G, Saatci I et al. Primary angiitis of the central nervous system and silent cortical hemorrhages. AJNR Am J Neuroradiol 2002; 23: 1561-1563
    Google Scholar
  • 55 Aviv RI, Benseler SM, Silverman ED et al. MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol 2006; 27: 192-199
    Google Scholar
  • 56 Singhal AB, Hajj-Ali RA, Topcuoglu MA et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011; 68: 1005-1012
    CrossrefGoogle Scholar
  • 57 Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012; 11: 906-917
    CrossrefGoogle Scholar
  • 58 Marder CP, Donohue MM, Weinstein JR et al. Multimodal imaging of reversible cerebral vasoconstriction syndrome: a series of 6 cases. AJNR Am J Neuroradiol 2012; 33: 1403-1410
    CrossrefGoogle Scholar
  • 59 Velez A, McKinney JS. Reversible cerebral vasoconstriction syndrome: a review of recent research. Curr Neurol Neurosci Rep 2013; 13: 319
    CrossrefGoogle Scholar
  • 60 Lee M, Zaharchuk G, Guzman R et al. Quantitative hemodynamic studies in moyamoya disease: a review. Neurosurg Focus 2009; 26: E5
    Google Scholar
  • 61 Bacigaluppi S, Dehdashti AR, Agid R et al. The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease: a review. Neurosurg Focus 2009; 26: E3
    Google Scholar
  • 62 Sawada T, Yamamoto A, Miki Y et al. Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels. Neuroradiology 2012; 54: 1089-1097
    CrossrefGoogle Scholar
  • 63 Sibbitt WL Jr, Brooks WM, Kornfeld M et al. Magnetic resonance imaging and brain histopathology in neuropsychiatric systemic lupus erythematosus. Semin Arthritis Rheum 2010; 40: 32-52
    CrossrefGoogle Scholar
  • 64 Goh YP, Naidoo P, Ngian GS. Imaging of systemic lupus erythematosus. I. CNS, cardiovascular, and thoracic manifestations. Clin Radiol 2013; 68: 181-191
    CrossrefGoogle Scholar
  • 65 Hasiloglu ZI, Albayram S, Tasmali K et al. A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement. Rheumatol Int 2012; 32: 805-807
    CrossrefGoogle Scholar
  • 66 Damasceno A, França MC Jr, Zanardi VA et al. Brainstem involvement in Sjögren’s syndrome-related sensory neuronopathy. J Neuroimaging 2010; 20: 397-399
    CrossrefGoogle Scholar
  • 67 Razek AA. Diffusion-weighted magnetic resonance imaging of head and neck. J Comput Assist Tomogr 2010; 34: 808-815
    CrossrefGoogle Scholar
  • 68 Wartolowska K, Hough MG, Jenkinson M et al. Structural changes of the brain in rheumatoid arthritis. Arthritis Rheum 2012; 64: 371-379
    CrossrefGoogle Scholar
  • 69 Kurne A, Karabudak R, Karadag O et al. An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis. Rheumatol Int 2009; 29: 1349-1353
    CrossrefGoogle Scholar
  • 70 Muscal E, Brey RL. Antiphospholipid syndrome and the brain in pediatric and adult patients. Lupus 2010; 19: 406-411
    CrossrefGoogle Scholar
  • 71 Amaral TN, Marques NetoJF, Lapa AT et al. Neurologic involvement in scleroderma en coup de sabre. Autoimmune Dis 2012; 2012: 719685
    Google Scholar
  • 72 Mohan S, Jain KK, Arabi M et al. Imaging of meningitis and ventriculitis. Neuroimaging Clin N Am 2012; 22: 557-583
    CrossrefGoogle Scholar
  • 73 Patkar D, Narang J, Yanamandala R et al. Central nervous system tuberculosis: pathophysiology and imaging findings. Neuroimaging Clin N Am 2012; 22: 677-705
    CrossrefGoogle Scholar
  • 74 Handique SK. Viral infections of the central nervous system. Neuroimaging Clin N Am 2011; 21: 777-794, vii
    CrossrefGoogle Scholar
  • 75 Baskin HJ, Hedlund G. Neuroimaging of herpesvirus infections in children. Pediatr Radiol 2007; 37: 949-963
    CrossrefGoogle Scholar
  • 76 Corr PD. Imaging of cerebrovascular and cardiovascular disease in AIDS patients. AJR Am J Roentgenol 2006; 187: 236-241
    CrossrefGoogle Scholar
  • 77 Patsalides AD, Wood LV, Atac GK et al. Cerebrovascular disease in HIV-infected pediatric patients: neuroimaging findings. AJR Am J Roentgenol 2002; 179: 999-1003
    CrossrefGoogle Scholar
  • 78 Razek AA, Castillo M. Imaging lesions of the cavernous sinus. AJNR Am J Neuroradiol 2009; 30: 444-452
    CrossrefGoogle Scholar
  • 79 Abdel RazekAA, Watcharakorn A, Castillo M. Parasitic diseases of the central nervous system. Neuroimaging Clin N Am 2011; 21: 815-841, viii
    CrossrefGoogle Scholar
  • 80 Song DK, Boulis NM, McKeever PE et al. Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. AJNR Am J Neuroradiol 2002; 23: 239-242
    Google Scholar
  • 81 Tamrazi B, Almast J. Your brain on drugs: imaging of drug-related changes in the central nervous system. RadioGraphics 2012; 32: 701-719
    CrossrefGoogle Scholar
  • 82 Geibprasert S, Gallucci M, Krings T. Addictive illegal drugs: structural neuroimaging. AJNR Am J Neuroradiol 2010; 31: 803-808
    CrossrefPubMedGoogle Scholar
  • 83 Rauch PJ, Park HS, Knisely JP et al. Delayed radiation-induced vasculitic leukoencephalopathy. Int J Radiat Oncol Biol Phys 2012; 83: 369-375
    CrossrefGoogle Scholar