• Hubert de Boysson, Grégoire Boulouis, Nelly Dequatre, Sophie Godard, Antoine Néel, Caroline Arquizan, Olivier Detante, Coralie Bloch-Queyrat, Mathieu Zuber, Emmanuel Touzé, Boris Bienvenu, Achille Aouba, Loïc Guillevin, Olivier Naggara, Christian Pagnoux, and French Vasculitis Study Group.
• From the Departments of Internal Medicine (H.d.B., B.B., A.A.) and Department of Neurology, Caen University Hospital, University of Caen-Normandie, INSERM U919, France (E.T.); Department of Neuroradiology, Centre Hospitalier Sainte-Anne, University Paris-Descartes, INSERM UMR 894, France (G.B., O.N.); Department of Neurology, Lille University Hospital, France (N.D.); Department of Neurology, Angers University Hospital, France (S.G.); Department of Internal Medicine, Nantes University Hospital, France (A.N.); Department of Neurology, Montpellier University Hospital, INSERM UMR 894, France (C.A.); Department of Neurology, Grenoble Alpes University Hospital, France (O.D.); Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France (C.B.-Q.); Department of Vascular Neurology-Centre Hospitalier Saint Joseph, Université Paris-Descartes, INSERM UMR S 919, France (M.Z.); Department of Internal Medicine, Centre Hospitalier Cochin, Paris, France (L.G.); and the Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto (C.P.). deboysson-h@chu-caen.fr.
• Stroke. 2016 Sep 1; 47 (9): 2401-4.

Background And PurposeWe aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system.MethodsWe retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort.ResultsThe 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0.008; 9 [90%] with seizures versus 22 [29%], P<0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12-130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification.ConclusionsPatients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.© 2016 American Heart Association, Inc.

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