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

on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalCephalalgia
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Vasoconstriction
Stroke
Primary Headache Disorders
Neuroimaging
Headache Disorders
Brain Edema
Cerebral Infarction
Headache
Epilepsy

Keywords

  • Reversible cerebral vasoconstriction syndrome (RCVS)
  • stroke
  • thunderclap headache

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

The clinical spectrum of reversible cerebral vasoconstriction syndrome : The Italian Project on Stroke at Young Age (IPSYS). / on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators.

In: Cephalalgia, 01.01.2019.

Research output: Contribution to journalArticle

on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators. / The clinical spectrum of reversible cerebral vasoconstriction syndrome : The Italian Project on Stroke at Young Age (IPSYS). In: Cephalalgia. 2019.
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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.",
keywords = "Reversible cerebral vasoconstriction syndrome (RCVS), stroke, thunderclap headache",
author = "{on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators} and Filomena Caria and Marialuisa Zedde and Massimo Gamba and Anna Bersano and Maurizia Rasura and Alessandro Adami and Carlo Piantadosi and Luca Quartuccio and Cristiano Azzini and Maurizio Melis and {Luisa Delodovici}, Maria and Carlo Dallocchio and Carlo Gandolfo and Paolo Cerrato and Cristina Motto and Fabio Melis and Alberto Chiti and Mauro Gentile and Valeria Bignamini and Andrea Morotti and {Maria Lotti}, Enrico and Antonella Toriello and Paolo Costa and Giorgio Silvestrelli and Andrea Zini and {De Giuli}, Valeria and Loris Poli and Maurizio Paciaroni and Corrado Lodigiani and Simona Marcheselli and Sandro Sanguigni and {Del Sette}, Massimo and Serena Monaco and Piergiorgio Lochner and Carla Zanferrari and Sabrina Anticoli and Alessandro Padovani and Alessandro Pezzini",
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T1 - The clinical spectrum of reversible cerebral vasoconstriction syndrome

T2 - The Italian Project on Stroke at Young Age (IPSYS)

AU - on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators

AU - Caria, Filomena

AU - Zedde, Marialuisa

AU - Gamba, Massimo

AU - Bersano, Anna

AU - Rasura, Maurizia

AU - Adami, Alessandro

AU - Piantadosi, Carlo

AU - Quartuccio, Luca

AU - Azzini, Cristiano

AU - Melis, Maurizio

AU - Luisa Delodovici, Maria

AU - Dallocchio, Carlo

AU - Gandolfo, Carlo

AU - Cerrato, Paolo

AU - Motto, Cristina

AU - Melis, Fabio

AU - Chiti, Alberto

AU - Gentile, Mauro

AU - Bignamini, Valeria

AU - Morotti, Andrea

AU - Maria Lotti, Enrico

AU - Toriello, Antonella

AU - Costa, Paolo

AU - Silvestrelli, Giorgio

AU - Zini, Andrea

AU - De Giuli, Valeria

AU - Poli, Loris

AU - Paciaroni, Maurizio

AU - Lodigiani, Corrado

AU - Marcheselli, Simona

AU - Sanguigni, Sandro

AU - Del Sette, Massimo

AU - Monaco, Serena

AU - Lochner, Piergiorgio

AU - Zanferrari, Carla

AU - Anticoli, Sabrina

AU - Padovani, Alessandro

AU - Pezzini, Alessandro

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Reversible cerebral vasoconstriction syndrome (RCVS)

KW - stroke

KW - thunderclap headache

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