The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)

Author:

Caria Filomena1,Zedde Marialuisa2,Gamba Massimo3,Bersano Anna4,Rasura Maurizia5,Adami Alessandro6,Piantadosi Carlo7,Quartuccio Luca8,Azzini Cristiano9,Melis Maurizio10,Luisa Delodovici Maria11,Dallocchio Carlo12,Gandolfo Carlo13,Cerrato Paolo14,Motto Cristina15,Melis Fabio16,Chiti Alberto17,Gentile Mauro18,Bignamini Valeria19,Morotti Andrea20,Maria Lotti Enrico21,Toriello Antonella22,Costa Paolo23,Silvestrelli Giorgio24,Zini Andrea25,De Giuli Valeria1,Poli Loris1,Paciaroni Maurizio26,Lodigiani Corrado27,Marcheselli Simona28,Sanguigni Sandro29,Del Sette Massimo30,Monaco Serena31,Lochner Piergiorgio32,Zanferrari Carla33,Anticoli Sabrina34,Padovani Alessandro1,Pezzini Alessandro1ORCID,

Affiliation:

1. Dipartimento di Scienze Cliniche e Sperimentali, Clinica Neurologica, Università degli Studi di Brescia, Brescia, Italy

2. SC Neurologia, Azienda Unità Sanitaria Locale – IRCCS di Reggio Emilia, Reggio Emilia, Italy

3. Stroke Unit, Neurologia Vascolare, Spedali Civili di Brescia, Brescia, Italy

4. UO Malattie Cerebrovascolari, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milan, Italy

5. Stroke Unit, Azienda Ospedaliera Sant’Andrea, Università “La Sapienza”, Rome, Italy

6. Stroke Center, Dipartimento di Neurologia, Ospedale Sacro Cuore Negrar, Verona, Italy

7. UOC Neurologia, Azienda Ospedaliera “San Giovanni-Addolorata”, Rome, Italy

8. Clinica di Reumatologia, Dipartimento di Area Medica, Azienda Ospedaliero-Universitaria “Santa Maria della Misericordia”, Udine, Italy

9. UO di Neurologia, Dipartimento di Neuroscienze e Riabilitazione, Azienda Ospedaliero-Universitaria di Ferrara, Ferrara, Italy

10. SC Neurologia and Stroke Unit, Dipartimento Neuroscienze e Riabilitazione, Azienda Ospedaliera “G. Brotzu”, Cagliari, Italy

11. Unità di Neurologia, Ospedale di Circolo, Università dell’Insubria, Varese, Italy

12. Dipartimento di Area Medica, UOC Neurologia, ASST Pavia, Voghera, Italy

13. Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili, Università di Genova, Genoa, Italy

14. Dipartimento di Neuroscienze, Stroke Unit, Università di Torino, Torino, Italy

15. Stroke Unit, Dipartmento di Scienze Neurologiche, Azienda Ospedaliera “Niguarda Ca’ Granda”, Milan, Italy

16. SS NeuroVascolare Ospedale Maria Vittoria, ASL Città di Torino, Torino, Italy

17. UO Neurologia, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy

18. UO Neurologia – Stroke Unit, Ospedale “Santa Maria della Misericordia”, Rovigo, Italy

19. Stroke Unit, U.O Neurologia, Ospedale “S. Chiara”, Trento, Italy

20. UC Malattie Cerebrovascolari, IRCCS Fondazione Mondino, Pavia, Italy

21. Unità Operativa Complessa Neurologia, AUSL Romagna, Ravenna, Italy

22. Unità Operativa Complessa Neurologia, Azienda Ospedaliera Universitaria “San Giovanni di Dio e Ruggi d’Aragona”, Salerno, Italy

23. UO Neurologia, Istituto Ospedaliero Poliambulanza, Brescia, Italy

24. Stroke Unit, Dipartimento di Neuroscienze, Ospedale Carlo Poma, Mantova, Italy

25. Stroke Unit, Clinica Neurologica, Nuovo Ospedale Civile “S. Agostino Estense”, AUSL Modena, Italy

26. Stroke Unit and Divisione di Medicina Cardiovascolare, Università di Perugia, Italy

27. Centro Trombosi e Malattie Emorragiche, Humanitas Clinical and Research Center, IRCCS, Rozzano-Milano, Italy

28. Neurologia d’Urgenza and Stroke Unit, Humanitas Clinical and Research Center, IRCCS, Rozzano-Milano, Italy

29. Dipartmento di Neurologia, Ospedale “Madonna del Soccorso”, San Benedetto del Tronto, Italy

30. Unità di Neurologia, E.O. Ospedali Galliera, Genova, Italy

31. Stroke Unit, Ospedale Civico, Palermo, Italy

32. Dipartimento di Neurologia, Saarland University Medical Center, Homburg, Germany

33. UOC Neurologia, ASST Melegnano-Martesana, Vizzolo Predabissi, Italy

34. Stroke Unit, Dipartimento di Emergenza-Urgenza, Ospedale “S. Camillo-Forlanini”, Rome, Italy

Abstract

Introduction To describe clinical, neuroimaging, and laboratory features of a large cohort of Italian patients with reversible cerebral vasoconstriction syndrome. Methods In the setting of the multicenter Italian Project on Stroke at Young Age (IPSYS), we retrospectively enrolled patients with a diagnosis of definite reversible cerebral vasoconstriction syndrome according to the International Classification of Headache Disorders (ICHD)-3 beta criteria (6.7.3 Headache attributed to reversible cerebral vasoconstriction syndrome, imaging-proven). Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of reversible cerebral vasoconstriction syndrome without typical causes (“idiopathic reversible cerebral vasoconstriction syndrome”) were compared with those of reversible cerebral vasoconstriction syndrome related to putative causative factors (“secondary reversible cerebral vasoconstriction syndrome”). Results A total of 102 patients (mean age, 47.2 ± 13.9 years; females, 85 [83.3%]) qualified for the analysis. Thunderclap headache at presentation was reported in 69 (67.6%) patients, and it typically recurred in 42 (60.9%). Compared to reversible cerebral vasoconstriction syndrome cases related to putative etiologic conditions (n = 21 [20.6%]), patients with idiopathic reversible cerebral vasoconstriction syndrome (n = 81 [79.4%]) were significantly older (49.2 ± 13.9 vs. 39.5 ± 11.4 years), had more frequently typical thunderclap headache (77.8% vs. 28.6%) and less frequently neurological complications (epileptic seizures, 11.1% vs. 38.1%; cerebral infarction, 6.1% vs. 33.3%), as well as concomitant reversible brain edema (25.9% vs. 47.6%). Conclusions Clinical manifestations and putative etiologies of reversible cerebral vasoconstriction syndrome in our series are slightly different from those observed in previous cohorts. This variability might be partly related to the coexistence of precipitating conditions with a putative etiologic role on disease occurrence.

Publisher

SAGE Publications

Subject

Clinical Neurology,General Medicine

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