Les vascularites du système nerveux central : mise au point |
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Authors: | A Néel C Pagnoux L Guillevin M Hamidou |
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Affiliation: | 1. Service de médecine interne, hôpital Hôtel-Dieu, CHU de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes cedex 01, France;2. Pôle de médecine interne, centre national de référence maladies auto-immunes et systémiques rares (vascularites et sclérodermie), hôpital Cochin, AP–HP, faculté de médecine, université Paris-Descartes, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France |
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Abstract: | Central nervous system (CNS) vasculitides are a heterogeneous group of diseases that can lead to severe disability and death. Given the poor performance of available diagnostic procedures (magnetic resonance imaging, angiography and brain biopsy), the diagnosis of isolated CNS vasculitis is challenging. Differential diagnosis includes secondary CNS vasculitides (infection, cancer, drug exposure or systemic inflammatory disorders) and several non-inflammatory cerebral vasculopathies, such as the reversible cerebral vasoconstriction syndrome. Despite recent advances in neuroradiology and the publication of large retrospective case series, primary angiitis of the CNS remains a heterogeneous and challenging nosological entity. Prospective interdisciplinary studies are necessary to improve our approach to these patients. |
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Keywords: | Vascularite cé ré brale Syndrome de vasoconstriction cé ré brale ré versible |
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