• Hubert de Boysson, Jean-Jacques Parienti, Jérôme Mawet, Caroline Arquizan, Grégoire Boulouis, Cécilia Burcin, Olivier Naggara, Mathieu Zuber, Emmanuel Touzé, Achille Aouba, Marie-Germaine Bousser, Christian Pagnoux, and Anne Ducros.
• From the Department of Internal Medicine (H.d.B., A.A.), Caen University Hospital; University of Caen-Normandie (H.d.B., E.T., A.A); Biostatistics and Clinical Research Unit (J.-J.P.), Caen University Hospital; Emergency Headache Centre (J.M., C.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris; Department of Neurology (C.A., A.D.), Montpellier University Hospital; Department of Neuroradiology (G.B., O.N.), Sainte-Anne Hospital, Paris-Descartes University; INSERM UMR 894 (O.N.); Department of Vascular Neurology (M.Z.), Saint Joseph Hospital; Université Paris-Descartes (M.Z.), INSERM UMR S 919, Paris; Department of Neurology (E.T.), Caen University Hospital; University of Caen-Normandie (E.T.), INSERM U919; Department of Neurology (M.-G.B.), Lariboisière Hospital, Assistance Publique des Hôpitaux de Paris, France; and Division of Rheumatology (C.P.), Vasculitis Clinic, Mount Sinai Hospital, Toronto, Ontario, Canada. deboysson-h@chu-caen.fr.
• Neurology. 2018 Oct 16; 91 (16): e1468-e1478.

ObjectivesTo further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS).MethodsWe compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled).ResultsPatients with RCVS were predominantly female (p < 0.0001), with migraines (p < 0.0001), and were more often exposed to vasoactive substances (p < 0.0001) or postpartum (p = 0.002) than patients with PACNS. Headache, especially thunderclap headache, was more frequent in RCVS (both p < 0.0001). Thunderclap headache was absent in only 6% of patients with RCVS and was mainly recurrent (87%) and provoked (77%) mostly by sexual intercourse, exertion, or emotion. All other neurologic symptoms (motor deficit, seizure, cognitive disorder, or vigilance impairment, all p < 0.0001) were more frequent in PACNS. At admission, brain CT or MRI was abnormal in all patients with PACNS and in 31% of patients with RCVS (p < 0.0001). Acute ischemic stroke was more frequent in PACNS than in RCVS (p < 0.0001). Although intracerebral hemorrhage was more frequent in PACNS (p = 0.006), subarachnoid hemorrhage and vasogenic edema predominated in RCVS (p = 0.04 and p = 0.01, respectively). Multiple small deep infarcts, extensive deep white matter lesions, tumor-like lesions, or multiple gadolinium-enhanced lesions were observed only in PACNS, whereas cervical artery dissection was found only in RCVS.ConclusionsOur study confirms that careful analysis of clinical context, headache features, and patterns of brain lesions can distinguish PACNS and RCVS within the first few days of admission in most cases. However, diagnosis remains challenging in a few cases.© 2018 American Academy of Neurology.

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