Skip to main content
Erschienen in: Clinical Neuroradiology 1/2019

05.09.2017 | Original Article

Neuroradiologic Characteristics of Primary Angiitis of the Central Nervous System According to the Affected Vessel Size

verfasst von: Christian Thaler, Ann-Katrin Kaufmann-Bühler, Tserenchunt Gansukh, Amarjargal Gansukh, Simon Schuster, Henrike Bachmann, Götz Thomalla, Tim Magnus, Jakob Matschke, Jens Fiehler, Susanne Siemonsen

Erschienen in: Clinical Neuroradiology | Ausgabe 1/2019

Einloggen, um Zugang zu erhalten

Abstract

Introduction

Magnetic resonance imaging (MRI) has an important impact in diagnosing primary angiitis of the central nervous system (PACNS). However, neuroradiologic findings may vary immensely, making an easy and definite diagnosis challenging.

Methods

In this retrospective, single center study, we analyzed neuroradiologic findings of patients with PACNS diagnosed at our hospital between 2009 and 2014. Furthermore, we classified patients according to the affected vessel size and compared imaging characteristics between the subgroups.

Results

Thirty-three patients were included (mean age 43 [±15.3] years, 17 females) in this study. Patients with positive angiographic findings were classified as either medium or large vessel PACNS and presented more ischemic lesions (p < 0.001) and vessel wall enhancement (p = 0.017) compared to patients with small vessel PACNS. No significant differences were detected for the distribution of contrast-enhancing lesions (parenchymal or leptomeningeal), hemorrhages, or lesions with mass effect. Twenty-five patients underwent brain biopsy. Patients with medium or large vessel PACNS were less likely to have positive biopsy results.

Discussion

It is essential to differentiate between small and medium/large vessel PACNS since results in MRI, digital subtraction angiography and brain biopsy may differ immensely. Since image quality of MR scanners improves gradually and brain biopsy may often be nonspecific or negative, our results emphasize the importance of MRI/MRA in the diagnosis process of PACNS.
Literatur
1.
Zurück zum Zitat Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988;67:20-39. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988;67:20-39.
2.
Zurück zum Zitat Hajj-Ali RA, Calabrese LH. Primary angiitis of the central nervous system. Autoimmun Rev. 2013;12:463–6.CrossRef Hajj-Ali RA, Calabrese LH. Primary angiitis of the central nervous system. Autoimmun Rev. 2013;12:463–6.CrossRef
3.
Zurück zum Zitat Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009;66:704–9.CrossRefPubMed Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009;66:704–9.CrossRefPubMed
4.
Zurück zum Zitat Ay H, Sahin G, Saatci I, Söylemezoğlu F, Saribaş O. Primary angiitis of the central nervous system and silent cortical hemorrhages. AJNR Am J Neuroradiol. 2002;23:1561–3.PubMed Ay H, Sahin G, Saatci I, Söylemezoğlu F, Saribaş O. Primary angiitis of the central nervous system and silent cortical hemorrhages. AJNR Am J Neuroradiol. 2002;23:1561–3.PubMed
5.
Zurück zum Zitat Kumar RS, Singh A, Rathore C, Kesavadas C. Primary angiitis of central nervous system: Tumor-like lesion. Neurol India. 2010;58:147–9.CrossRefPubMed Kumar RS, Singh A, Rathore C, Kesavadas C. Primary angiitis of central nervous system: Tumor-like lesion. Neurol India. 2010;58:147–9.CrossRefPubMed
6.
Zurück zum Zitat Néel A, Pagnoux C. Primary angiitis of the central nervous system. Clin Exp Rheumatol. 2009;27(1 Suppl 52):95–107. Néel A, Pagnoux C. Primary angiitis of the central nervous system. Clin Exp Rheumatol. 2009;27(1 Suppl 52):95–107.
7.
Zurück zum Zitat de Boysson H, Zuber M, Naggara O, Neau JP, Gray F, Bousser MG, Crassard I, Touzé E, Couraud PO, Kerschen P, Oppenheim C, Detante O, Faivre A, Gaillard N, Arquizan C, Bienvenu B, Néel A, Guillevin L, Pagnoux C; French Vasculitis Study Group and the French NeuroVascular Society. Primary angiitis of the central nervous system: Description of the first fifty-two adults enrolled in the french cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol. 2014;66:1315–26.CrossRefPubMed de Boysson H, Zuber M, Naggara O, Neau JP, Gray F, Bousser MG, Crassard I, Touzé E, Couraud PO, Kerschen P, Oppenheim C, Detante O, Faivre A, Gaillard N, Arquizan C, Bienvenu B, Néel A, Guillevin L, Pagnoux C; French Vasculitis Study Group and the French NeuroVascular Society. Primary angiitis of the central nervous system: Description of the first fifty-two adults enrolled in the french cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol. 2014;66:1315–26.CrossRefPubMed
8.
Zurück zum Zitat Küker W, Gaertner S, Nägele T, Dopfer C, Schoning M, Fiehler J, Rothwell PM, Herrlinger U. Vessel wall contrast enhancement: A diagnostic sign of cerebral vasculitis. Cerebrovasc Dis. 2008;26:23–9.CrossRefPubMedPubMedCentral Küker W, Gaertner S, Nägele T, Dopfer C, Schoning M, Fiehler J, Rothwell PM, Herrlinger U. Vessel wall contrast enhancement: A diagnostic sign of cerebral vasculitis. Cerebrovasc Dis. 2008;26:23–9.CrossRefPubMedPubMedCentral
9.
Zurück zum Zitat Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012;380(9843):767–77.CrossRefPubMed Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012;380(9843):767–77.CrossRefPubMed
10.
Zurück zum Zitat Salvarani C, Brown RD Jr, Christianson TJ, Huston J 3rd, Giannini C, Miller DV, Hunder GG. Adult primary central nervous system vasculitis treatment and course; analysis of one hundred sixty-three patients. Arthritis Rheumatol. 2015;67:1637–45.CrossRefPubMed Salvarani C, Brown RD Jr, Christianson TJ, Huston J 3rd, Giannini C, Miller DV, Hunder GG. Adult primary central nervous system vasculitis treatment and course; analysis of one hundred sixty-three patients. Arthritis Rheumatol. 2015;67:1637–45.CrossRefPubMed
11.
Zurück zum Zitat Celluci T, Tyrrell PN, Sheikh S, Benseler SM. Childhood primary angiitis of the central nervous system: Identifying disease trajectories and early risk factors for persistently higher disease acitivity. Arthritis Rheumatol. 2012;64:1665–72.CrossRef Celluci T, Tyrrell PN, Sheikh S, Benseler SM. Childhood primary angiitis of the central nervous system: Identifying disease trajectories and early risk factors for persistently higher disease acitivity. Arthritis Rheumatol. 2012;64:1665–72.CrossRef
12.
Zurück zum Zitat Park MS, Marlin AE, Gaskill SJ. Angiography-negative primary angiitis of the central nervous system in childhood. J Neurosurg Pediatr. 2014;13:62–7.CrossRefPubMed Park MS, Marlin AE, Gaskill SJ. Angiography-negative primary angiitis of the central nervous system in childhood. J Neurosurg Pediatr. 2014;13:62–7.CrossRefPubMed
14.
Zurück zum Zitat Salvarani C, Brown RD Jr, Christianson T, Miller DV, Giannini C, Huston J 3rd, Hunder GG. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: Description of 163 patients. Medicine (Baltimore). 2015;94(21):e738.CrossRef Salvarani C, Brown RD Jr, Christianson T, Miller DV, Giannini C, Huston J 3rd, Hunder GG. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: Description of 163 patients. Medicine (Baltimore). 2015;94(21):e738.CrossRef
15.
16.
Zurück zum Zitat Boulouis G, de Boysson H, Zuber M, Guillevin L, Meary E, Costalat V, Pagnoux C, Naggara O, French Vasculitis Group. Primary Angiitis of the central nervous system: Magnetic resonance imaging spectrum of Parenchymal, meningeal, and vascular lesions at baseline. Stroke. 2017;48(5):1248–55.CrossRefPubMed Boulouis G, de Boysson H, Zuber M, Guillevin L, Meary E, Costalat V, Pagnoux C, Naggara O, French Vasculitis Group. Primary Angiitis of the central nervous system: Magnetic resonance imaging spectrum of Parenchymal, meningeal, and vascular lesions at baseline. Stroke. 2017;48(5):1248–55.CrossRefPubMed
17.
Zurück zum Zitat Torres J, Loomis C, Cucchiara B, Smith M, Messé S. Diagnostic yield and safety of brain biopsy for suspected primary central nervous system angiitis. Stroke. 2016;47:2127–9.CrossRefPubMed Torres J, Loomis C, Cucchiara B, Smith M, Messé S. Diagnostic yield and safety of brain biopsy for suspected primary central nervous system angiitis. Stroke. 2016;47:2127–9.CrossRefPubMed
18.
Zurück zum Zitat de Boysson H, Boulouis G, Aouba A, Bienvenu B, Guillevin L, Zuber M, Touzé E, Naggara O, Pagnoux C. Adult primary angiitis of the central nervous system: Isolated small-vasculitis represents distinct disease pattern. Rheumatology (Oxford). 2017;56(3):439–44. de Boysson H, Boulouis G, Aouba A, Bienvenu B, Guillevin L, Zuber M, Touzé E, Naggara O, Pagnoux C. Adult primary angiitis of the central nervous system: Isolated small-vasculitis represents distinct disease pattern. Rheumatology (Oxford). 2017;56(3):439–44.
19.
Zurück zum Zitat Amlie-Lefond C. Catheter angiogram in central nervous system vasculitis: Still first among equals. Dev Med Child Neurol. 2010;52:792.CrossRefPubMed Amlie-Lefond C. Catheter angiogram in central nervous system vasculitis: Still first among equals. Dev Med Child Neurol. 2010;52:792.CrossRefPubMed
20.
Zurück zum Zitat Cosottini M, Canovetti S, Pesaresi I, Desideri I, Pizzanelli C, Catarsi E, Puglioli M, Tavoni A, Bonuccelli U, Bartolozzi C. 3‑T magnetic resonance angiography in primary angiitis of the central nervous system. J Comput Assist Tomogr. 2013;37(4):493–8.CrossRefPubMed Cosottini M, Canovetti S, Pesaresi I, Desideri I, Pizzanelli C, Catarsi E, Puglioli M, Tavoni A, Bonuccelli U, Bartolozzi C. 3‑T magnetic resonance angiography in primary angiitis of the central nervous system. J Comput Assist Tomogr. 2013;37(4):493–8.CrossRefPubMed
21.
Zurück zum Zitat Mandell DM, Matouk CC, Farb RI, Krings T, Agid R, terBrugge K, Willinsky RA, Swartz RH, Silver FL, Mikulis DJ. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: Preliminary results. Stroke. 2012;43:860–2.CrossRefPubMed Mandell DM, Matouk CC, Farb RI, Krings T, Agid R, terBrugge K, Willinsky RA, Swartz RH, Silver FL, Mikulis DJ. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: Preliminary results. Stroke. 2012;43:860–2.CrossRefPubMed
22.
Zurück zum Zitat Obusez EC, Hui F, Hajj-Ali RA, Cerejo R, Calabrese LH, Hammad T, Jones SE. High-resolution MRI vessel wall imaging: Spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system Vasculitis. AJNR Am J Neuroradiol. 2014;35:1527–32.CrossRefPubMed Obusez EC, Hui F, Hajj-Ali RA, Cerejo R, Calabrese LH, Hammad T, Jones SE. High-resolution MRI vessel wall imaging: Spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system Vasculitis. AJNR Am J Neuroradiol. 2014;35:1527–32.CrossRefPubMed
23.
Zurück zum Zitat Pfefferkorn T, Linn J, Habs M, Opherk C, Cyran C, Ottomeyer C, Straube A, Dichgans M, Nikolaou K, Saam T. Black blood MRI in suspected large artery primary Angiitis of the central nervous system. J Neuroimaging. 2013;23:379–83.CrossRefPubMed Pfefferkorn T, Linn J, Habs M, Opherk C, Cyran C, Ottomeyer C, Straube A, Dichgans M, Nikolaou K, Saam T. Black blood MRI in suspected large artery primary Angiitis of the central nervous system. J Neuroimaging. 2013;23:379–83.CrossRefPubMed
24.
Zurück zum Zitat van der Kolk AG, Zwanenburg JJ, Brundel M, Biessels GJ, Visser F, Luijten PR, Hendrikse J. Intracranial vessel wall imaging at 7.0-T MRI. Stroke. 2011;42:2478–84.CrossRef van der Kolk AG, Zwanenburg JJ, Brundel M, Biessels GJ, Visser F, Luijten PR, Hendrikse J. Intracranial vessel wall imaging at 7.0-T MRI. Stroke. 2011;42:2478–84.CrossRef
25.
Zurück zum Zitat Saam T, Hatsukami TS, Takaya N, Chu B, Underhill H, Kerwin WS, Cai J, Ferguson MS, Yuan C. The vulnerable, or high-risk, atherosclerotic plaque: Noninvasive MR imaging for characterization and assessment. Radiology. 2007;244:64–77.CrossRefPubMed Saam T, Hatsukami TS, Takaya N, Chu B, Underhill H, Kerwin WS, Cai J, Ferguson MS, Yuan C. The vulnerable, or high-risk, atherosclerotic plaque: Noninvasive MR imaging for characterization and assessment. Radiology. 2007;244:64–77.CrossRefPubMed
Metadaten
Titel
Neuroradiologic Characteristics of Primary Angiitis of the Central Nervous System According to the Affected Vessel Size
verfasst von
Christian Thaler
Ann-Katrin Kaufmann-Bühler
Tserenchunt Gansukh
Amarjargal Gansukh
Simon Schuster
Henrike Bachmann
Götz Thomalla
Tim Magnus
Jakob Matschke
Jens Fiehler
Susanne Siemonsen
Publikationsdatum
05.09.2017
Verlag
Springer Berlin Heidelberg
Erschienen in
Clinical Neuroradiology / Ausgabe 1/2019
Print ISSN: 1869-1439
Elektronische ISSN: 1869-1447
DOI
https://doi.org/10.1007/s00062-017-0622-8

Weitere Artikel der Ausgabe 1/2019

Clinical Neuroradiology 1/2019 Zur Ausgabe

Information

Information

Update Radiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.