, Volume 58, Issue 3, pp 221–235 | Cite as

Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach

  • Guillaume TaiebEmail author
  • Alberto Duran-Peña
  • Nicolas Menjot de Chamfleur
  • Antoine Moulignier
  • Eric Thouvenot
  • Thibaut Allou
  • Arnaud Lacour
  • Khe Hoang-Xuan
  • Jean Pelletier
  • Pierre Labauge
Continuing Education



Cerebral punctate and curvilinear gadolinium enhancements (PCGE) correspond to opacification of small vessel lumen or its perivascular areas in case of blood-brain barrier (BBB) disruption. We will discuss the possible causes of intra-parenchymal central nervous system PCGE.


Our review is based on French database including patients presenting with central nervous system PCGE and literature search using PubMed database with the following keywords: punctate enhancement, linear enhancement, and curvilinear enhancement. Disorders which displayed linear leptomeningeal or periventricular enhancements without intra-parenchymal PCGE are excluded of this review.


Among our 39 patients with PCGE, 16 different diagnoses were established. After combining our PCGE causes with those described in the literature, we propose a practical approach. Besides physiologic post-contrast enhancement of small vessels, three pathologic conditions may exhibit PCGE: (1) small collateral artery network seen in Moyamoya syndrome, (2) small veins congestions related to developmental or acquired venous outflow disturbance, and (3) disorders causing small vessels BBB disruption indicated by T2 and FLAIR hyperintensities in the corresponding areas of PCGE. Disruption of the BBB could be caused by a direct injury of the endothelial cell, as in posterior reversible encephalopathy syndrome, Susac syndrome, and radiochemotherapy-induced injuries, or by an angiocentric cellular infiltrate, as in inflammatory disorders, demyelinating diseases, host immune responses fighting against infections, prelymphoma states, lymphoma, and in CLIPPERS.


PCGE may conceal several causes, including physiological and pathological conditions. Nevertheless, a practical approach could improve its management and limit the indications of brain biopsy to very specific situations.


Punctate enhancement Curvilinear enhancement Linear enhancement Blood-brain barrier CLIPPERS 



Diffusion-weighted images with increased


Apparent diffusion coefficient


Blood-brain barrier


Magnetic resonance imaging


Punctate and curvilinear gadolinium enhancements


Susceptibility-weighted imaging


T1-weighted images


T2-weighted images


Posterior reversible encephalopathy syndrome


Systemic lupus erythematosus


Langerhans cell histiocytosis


Non-Langerhans cell histiocytosis


Primary arteritis of the CNS


Acute demyelinating encephalomyelitis\


Multiple sclerosis


Neuromyelitis optica spectrum disorders


Immune reconstitution inflammatory syndrome


Progressive multifocal leukoencephalopathy


Primary CNS lymphoma


Lymphomatoid granulomatosis


Intravascular lymphoma


Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids



We want to especially thank Mr Benjamin Taieb for his help in figure design.

Compliance with ethical standards

We declare that this manuscript does not contain clinical studies. Anonymized data with a number were used to develop a practical approach.

Conflict of interest

We declare that we have no conflict of interest.


  1. 1.
    Carr JC, Carroll TJ (eds) (2012) Magnetic resonance angiography, principles and applications. Springer, New York, pp 215–216Google Scholar
  2. 2.
    Pittock SJ, Debruyne J, Krecke KN et al (2010) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 133:2626–2634CrossRefPubMedGoogle Scholar
  3. 3.
    Tateishi U, Terae S, Ogata A, Sawamura Y, Suzuki Y, Abe S, Miyasaka K (2001) MR imaging of the brain in lymphomatoid granulomatosis. AJNR Am J Neuroradiol 22:1283–1290PubMedGoogle Scholar
  4. 4.
    Taieb G, Duflos C, Renard D et al (2012) Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 69:847–855PubMedGoogle Scholar
  5. 5.
    Uysal E, Erturk SM, Yildirim H et al (2007) Sensitivity of immediate and delayed gadolinium-enhanced MRI after injection of 0.5 M and 1.0 M gadolinium chelates for detecting multiple sclerosis lesions. AJR Am J Roentgenol 188:697–702CrossRefPubMedGoogle Scholar
  6. 6.
    Friedman DP (1997) Abnormalities of the deep medullary white matter veins: MR imaging findings. AJR Am J Roentgenol 168:1103–1108CrossRefPubMedGoogle Scholar
  7. 7.
    Komiyama M, Nakajima H, Nishikawa M et al (2001) Leptomeningeal contrast enhancement in moyamoya: its potential role in postoperative assessment of circulation through the bypass. Neuroradiology 43:17–23CrossRefPubMedGoogle Scholar
  8. 8.
    Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 29:1043–1049CrossRefPubMedGoogle Scholar
  9. 9.
    Susac JO, Murtagh FR, Egan RA et al (2003) MRI findings in Susac’s syndrome. Neurology 61:1783–1787CrossRefPubMedGoogle Scholar
  10. 10.
    Jennings JE, Sundgren PC, Attwood J et al (2004) Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance. Neuroradiology 46:15–21CrossRefPubMedGoogle Scholar
  11. 11.
    Christoforidis GA, Spickler EM, Recio MV et al (1999) MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment. AJNR Am J Neuroradiol 20:655–669PubMedGoogle Scholar
  12. 12.
    Prayer D, Grois N, Prosch H et al (2004) MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25:880–891PubMedGoogle Scholar
  13. 13.
    Sedrak P, Ketonen L, Hou P et al (2011) Erdheim-Chester disease of the central nervous system: new manifestations of a rare disease. AJNR Am J Neuroradiol 32:2126–2131CrossRefPubMedGoogle Scholar
  14. 14.
    Forbes KP, Collie DA, Parker A (2000) CNS involvement of virus-associated hemophagocytic syndrome: MR imaging appearance. AJNR Am J Neuroradiol 21:1248–1250PubMedGoogle Scholar
  15. 15.
    Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65:1–11CrossRefPubMedGoogle Scholar
  16. 16.
    Chenevier F, Renoux C, Marignier R et al (2009) Primary angiitis of the central nervous system: response to mycophenolate mofetil. J Neurol Neurosurg Psychiatry 80:1159–1161CrossRefPubMedGoogle Scholar
  17. 17.
    Sakaguchi H, Ueda A, Kosaka T et al (2011) Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature. J Neuroinflammation 8:116CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Young NP, Weinshenker BG, Parisi JE et al (2010) Perivenous demyelination: association with clinically defined acute disseminated encephalomyelitis and comparison with pathologically confirmed multiple sclerosis. Brain 133:333–348CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Guttmann CR, Ahn SS, Hsu L et al (1995) The evolution of multiple sclerosis lesions on serial MR. AJNR Am J Neuroradiol 16:1481–1491PubMedGoogle Scholar
  20. 20.
    Pekcevik Y, Izbudak I (2015) Perivascular enhancement in a patient with neuromyelitis optica spectrum disease during an optic neuritis attack. J Neuroimaging 25:686–687CrossRefPubMedGoogle Scholar
  21. 21.
    Post MJ, Thurnher MM, Clifford DB et al (2013) CNS-immune reconstitution inflammatory syndrome in the setting of HIV infection, part 1: overview and discussion of progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome and cryptococcal-immune reconstitution inflammatory syndrome. AJNR Am J Neuroradiol 34:1297–1307CrossRefPubMedGoogle Scholar
  22. 22.
    Wattjes MP, Verhoeff L, Zentjens W et al (2013) Punctate lesion pattern suggestive of perivascular inflammation in acute natalizumab-associated progressive multifocal leukoencephalopathy: productive JC virus infection or preclinical PML-IRIS manifestation? J Neurol Neurosurg Psychiatry 84:1176–1177CrossRefPubMedGoogle Scholar
  23. 23.
    Lescure FX, Moulignier A, Savatovsky J et al (2013) CD8 encephalitis in HIV-infected patients receiving cART: a treatable entity. Clin Infect Dis 57:101–108CrossRefPubMedGoogle Scholar
  24. 24.
    Sanelli PC, Lev MH, Gonzalez RG et al (2001) Unique linear and nodular MR enhancement pattern in schistosomiasis of the central nervous system: report of three patients. AJR Am J Roentgenol 177:1471–1474CrossRefPubMedGoogle Scholar
  25. 25.
    Taieb G, Uro-Coste E, Clanet M et al (2014) A central nervous system B-cell lymphoma arising two years after initial diagnosis of CLIPPERS. J Neurol Sci 344:224–226CrossRefPubMedGoogle Scholar
  26. 26.
    Patsalides AD, Atac G, Hedge U et al (2005) Lymphomatoid granulomatosis: abnormalities of the brain at MR imaging. Radiology 237:265–273CrossRefPubMedGoogle Scholar
  27. 27.
    De Graaff HJ, Wattjes MP, Rozemuller-Kwakkel AJ et al (2013) Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. JAMA Neurol 70:915–918CrossRefPubMedGoogle Scholar
  28. 28.
    Epaliyanage P, King A, Hampton T, Gullan R, Ashkan K (2014) “Brain on fire”: a new imaging sign. J Clin Neurosci 21:2015–2017CrossRefPubMedGoogle Scholar
  29. 29.
    Faivre G, Lagarde J, Choquet S et al (2014) CNS involvement at diagnosis in mantle cell lymphoma with atypical MRI features. J Neurol 261:1018–1020CrossRefPubMedGoogle Scholar
  30. 30.
    Yamamoto A, Kikuchi Y, Homma K et al (2012) Characteristics of intravascular large B-cell lymphoma on cerebral MR imaging. AJNR Am J Neuroradiol 33:292–296CrossRefPubMedGoogle Scholar
  31. 31.
    Liow K, Asmar P, Liow M et al (2000) Intravascular lymphomatosis: contribution of cerebral MRI findings to diagnosis. J Neuroimaging 10:116–118CrossRefPubMedGoogle Scholar
  32. 32.
    Simon NG, Parratt JD, Barnett MH et al (2012) Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry 83:15–22CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Guillaume Taieb
    • 1
    Email author
  • Alberto Duran-Peña
    • 2
  • Nicolas Menjot de Chamfleur
    • 3
  • Antoine Moulignier
    • 4
  • Eric Thouvenot
    • 5
  • Thibaut Allou
    • 1
  • Arnaud Lacour
    • 6
  • Khe Hoang-Xuan
    • 2
  • Jean Pelletier
    • 7
  • Pierre Labauge
    • 1
  1. 1.Department of Neurology, CHU MontpellierHopital Guy de ChauliacMontpellier Cedex 5France
  2. 2.Department of Neurology, MazarinGoupe Hospitalier Pitié SalpêtrièreParisFrance
  3. 3.Department of Neuroradiology, CHU MontpellierHopital Guy de ChauliacMontpellierFrance
  4. 4.Department of NeurologyFondation RothschildParisFrance
  5. 5.Department of Neurology, CHU NîmesHôpital CaremeauNîmesFrance
  6. 6.Department of NeurologyCHU De LilleLilleFrance
  7. 7.APHM, Hôpital de la Timone, Pôle de Neurosciences CliniquesService de NeurologieMarseilleFrance

Personalised recommendations