Guillaume Taieb1, Alberto Duran-Peña2, Nicolas Menjot de Chamfleur3, Antoine Moulignier4, Eric Thouvenot5, Thibaut Allou6, Arnaud Lacour7, Khe Hoang-Xuan2, Jean Pelletier8, Pierre Labauge6. 1. Department of Neurology, CHU Montpellier, Hopital Guy de Chauliac, 80 avenue Augustin Fliche, 34295, Montpellier Cedex 5, France. taiebguillaume@gmail.com. 2. Department of Neurology, Mazarin, Goupe Hospitalier Pitié Salpêtrière, Paris, France. 3. Department of Neuroradiology, CHU Montpellier, Hopital Guy de Chauliac, Montpellier, France. 4. Department of Neurology, Fondation Rothschild, Paris, France. 5. Department of Neurology, CHU Nîmes, Hôpital Caremeau, Nîmes, France. 6. Department of Neurology, CHU Montpellier, Hopital Guy de Chauliac, 80 avenue Augustin Fliche, 34295, Montpellier Cedex 5, France. 7. Department of Neurology, CHU De Lille, Lille, France. 8. APHM, Hôpital de la Timone, Pôle de Neurosciences Cliniques, Service de Neurologie, Marseille, France.
Abstract
INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: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.
Entities:
Keywords:
Blood-brain barrier; CLIPPERS; Curvilinear enhancement; Linear enhancement; Punctate enhancement
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