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

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 dia...

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Veröffentlicht in:Cephalalgia 2019-09, Vol.39 (10), p.1267-1276
Hauptverfasser: Caria, Filomena, Zedde, Marialuisa, Gamba, Massimo, Bersano, Anna, Rasura, Maurizia, Adami, Alessandro, Piantadosi, Carlo, Quartuccio, Luca, Azzini, Cristiano, Melis, Maurizio, Luisa Delodovici, Maria, Dallocchio, Carlo, Gandolfo, Carlo, Cerrato, Paolo, Motto, Cristina, Melis, Fabio, Chiti, Alberto, Gentile, Mauro, Bignamini, Valeria, Morotti, Andrea, Maria Lotti, Enrico, Toriello, Antonella, Costa, Paolo, Silvestrelli, Giorgio, Zini, Andrea, De Giuli, Valeria, Poli, Loris, Paciaroni, Maurizio, Lodigiani, Corrado, Marcheselli, Simona, Sanguigni, Sandro, Del Sette, Massimo, Monaco, Serena, Lochner, Piergiorgio, Zanferrari, Carla, Anticoli, Sabrina, Padovani, Alessandro, Pezzini, Alessandro
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container_issue 10
container_start_page 1267
container_title Cephalalgia
container_volume 39
creator Caria, Filomena
Zedde, Marialuisa
Gamba, Massimo
Bersano, Anna
Rasura, Maurizia
Adami, Alessandro
Piantadosi, Carlo
Quartuccio, Luca
Azzini, Cristiano
Melis, Maurizio
Luisa Delodovici, Maria
Dallocchio, Carlo
Gandolfo, Carlo
Cerrato, Paolo
Motto, Cristina
Melis, Fabio
Chiti, Alberto
Gentile, Mauro
Bignamini, Valeria
Morotti, Andrea
Maria Lotti, Enrico
Toriello, Antonella
Costa, Paolo
Silvestrelli, Giorgio
Zini, Andrea
De Giuli, Valeria
Poli, Loris
Paciaroni, Maurizio
Lodigiani, Corrado
Marcheselli, Simona
Sanguigni, Sandro
Del Sette, Massimo
Monaco, Serena
Lochner, Piergiorgio
Zanferrari, Carla
Anticoli, Sabrina
Padovani, Alessandro
Pezzini, Alessandro
description 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.
doi_str_mv 10.1177/0333102419849013
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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.</description><identifier>ISSN: 0333-1024</identifier><identifier>EISSN: 1468-2982</identifier><identifier>DOI: 10.1177/0333102419849013</identifier><identifier>PMID: 31060368</identifier><language>eng</language><publisher>London, England: SAGE Publications</publisher><ispartof>Cephalalgia, 2019-09, Vol.39 (10), p.1267-1276</ispartof><rights>International Headache Society 2019</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c403t-42b399318c56a607aa53f5afc25b8ac8f6932d6ac660fd8034189ffc5267fa3c3</citedby><cites>FETCH-LOGICAL-c403t-42b399318c56a607aa53f5afc25b8ac8f6932d6ac660fd8034189ffc5267fa3c3</cites><orcidid>0000-0001-8629-3315</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://journals.sagepub.com/doi/pdf/10.1177/0333102419849013$$EPDF$$P50$$Gsage$$H</linktopdf><linktohtml>$$Uhttps://journals.sagepub.com/doi/10.1177/0333102419849013$$EHTML$$P50$$Gsage$$H</linktohtml><link.rule.ids>314,776,780,21945,27830,27901,27902,44921,45309</link.rule.ids><linktorsrc>$$Uhttps://journals.sagepub.com/doi/full/10.1177/0333102419849013?utm_source=summon&amp;utm_medium=discovery-provider$$EView_record_in_SAGE_Publications$$FView_record_in_$$GSAGE_Publications</linktorsrc><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31060368$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Caria, Filomena</creatorcontrib><creatorcontrib>Zedde, Marialuisa</creatorcontrib><creatorcontrib>Gamba, Massimo</creatorcontrib><creatorcontrib>Bersano, Anna</creatorcontrib><creatorcontrib>Rasura, Maurizia</creatorcontrib><creatorcontrib>Adami, Alessandro</creatorcontrib><creatorcontrib>Piantadosi, Carlo</creatorcontrib><creatorcontrib>Quartuccio, Luca</creatorcontrib><creatorcontrib>Azzini, Cristiano</creatorcontrib><creatorcontrib>Melis, Maurizio</creatorcontrib><creatorcontrib>Luisa Delodovici, Maria</creatorcontrib><creatorcontrib>Dallocchio, Carlo</creatorcontrib><creatorcontrib>Gandolfo, Carlo</creatorcontrib><creatorcontrib>Cerrato, Paolo</creatorcontrib><creatorcontrib>Motto, Cristina</creatorcontrib><creatorcontrib>Melis, Fabio</creatorcontrib><creatorcontrib>Chiti, Alberto</creatorcontrib><creatorcontrib>Gentile, Mauro</creatorcontrib><creatorcontrib>Bignamini, Valeria</creatorcontrib><creatorcontrib>Morotti, Andrea</creatorcontrib><creatorcontrib>Maria Lotti, Enrico</creatorcontrib><creatorcontrib>Toriello, Antonella</creatorcontrib><creatorcontrib>Costa, Paolo</creatorcontrib><creatorcontrib>Silvestrelli, Giorgio</creatorcontrib><creatorcontrib>Zini, Andrea</creatorcontrib><creatorcontrib>De Giuli, Valeria</creatorcontrib><creatorcontrib>Poli, Loris</creatorcontrib><creatorcontrib>Paciaroni, Maurizio</creatorcontrib><creatorcontrib>Lodigiani, Corrado</creatorcontrib><creatorcontrib>Marcheselli, Simona</creatorcontrib><creatorcontrib>Sanguigni, Sandro</creatorcontrib><creatorcontrib>Del Sette, Massimo</creatorcontrib><creatorcontrib>Monaco, Serena</creatorcontrib><creatorcontrib>Lochner, Piergiorgio</creatorcontrib><creatorcontrib>Zanferrari, Carla</creatorcontrib><creatorcontrib>Anticoli, Sabrina</creatorcontrib><creatorcontrib>Padovani, Alessandro</creatorcontrib><creatorcontrib>Pezzini, Alessandro</creatorcontrib><creatorcontrib>Italian Project on Stroke at Young Age (IPSYS) Investigators</creatorcontrib><creatorcontrib>on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators</creatorcontrib><title>The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)</title><title>Cephalalgia</title><addtitle>Cephalalgia</addtitle><description>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.</description><issn>0333-1024</issn><issn>1468-2982</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNp1kDtPwzAUhS0EoqWwMyGPZQj4kbgOW1XxqFSJSi1Dp8hx7OKSxMVOKpVfj6MWBiSmO5zvfNI9AFxjdIfxaHSPKKUYkRinPE4Rpiegj2PGI5Jycgr6XRx1eQ9ceL9BCCUMsXPQCyWGKON98LV8V1CWpjZSlNBvlWxcW0GroVM75bzJy5Arp3IX8p3wVtraN87Ixtga-n1dOFupB9h5po0ojajh3NlNEMEALBpnPxQUDVzZtl7D8VrB4XS-WC1uL8GZFqVXV8c7AG9Pj8vJSzR7fZ5OxrNIxog2UUxymqYUc5kwwdBIiITqRGhJkpwLyTVLKSmYkIwhXXBEY8xTrWVC2EgLKukADA_erbOfrfJNVhkvVVmKWtnWZ4RQginDFAcUHVDprPdO6WzrTCXcPsMo6xbP_i4eKjdHe5tXqvgt_EwcgOgAeLFW2ca2rg7f_i_8BlN5iGs</recordid><startdate>20190901</startdate><enddate>20190901</enddate><creator>Caria, Filomena</creator><creator>Zedde, Marialuisa</creator><creator>Gamba, Massimo</creator><creator>Bersano, 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Massimo</creator><creator>Monaco, Serena</creator><creator>Lochner, Piergiorgio</creator><creator>Zanferrari, Carla</creator><creator>Anticoli, Sabrina</creator><creator>Padovani, Alessandro</creator><creator>Pezzini, Alessandro</creator><general>SAGE Publications</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-8629-3315</orcidid></search><sort><creationdate>20190901</creationdate><title>The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)</title><author>Caria, Filomena ; Zedde, Marialuisa ; Gamba, Massimo ; Bersano, Anna ; Rasura, Maurizia ; Adami, Alessandro ; Piantadosi, Carlo ; Quartuccio, Luca ; Azzini, Cristiano ; Melis, Maurizio ; Luisa Delodovici, Maria ; Dallocchio, Carlo ; Gandolfo, Carlo ; Cerrato, Paolo ; Motto, Cristina ; Melis, Fabio ; Chiti, Alberto ; Gentile, Mauro ; Bignamini, Valeria ; Morotti, Andrea ; Maria Lotti, Enrico ; Toriello, Antonella ; Costa, Paolo ; Silvestrelli, Giorgio ; Zini, Andrea ; De Giuli, Valeria ; Poli, Loris ; Paciaroni, Maurizio ; Lodigiani, Corrado ; Marcheselli, Simona ; Sanguigni, Sandro ; Del Sette, Massimo ; Monaco, Serena ; Lochner, Piergiorgio ; Zanferrari, Carla ; Anticoli, Sabrina ; Padovani, Alessandro ; Pezzini, Alessandro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c403t-42b399318c56a607aa53f5afc25b8ac8f6932d6ac660fd8034189ffc5267fa3c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Caria, Filomena</creatorcontrib><creatorcontrib>Zedde, Marialuisa</creatorcontrib><creatorcontrib>Gamba, Massimo</creatorcontrib><creatorcontrib>Bersano, Anna</creatorcontrib><creatorcontrib>Rasura, Maurizia</creatorcontrib><creatorcontrib>Adami, 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Academic</collection><jtitle>Cephalalgia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext_linktorsrc</fulltext></delivery><addata><au>Caria, Filomena</au><au>Zedde, Marialuisa</au><au>Gamba, Massimo</au><au>Bersano, Anna</au><au>Rasura, Maurizia</au><au>Adami, Alessandro</au><au>Piantadosi, Carlo</au><au>Quartuccio, Luca</au><au>Azzini, Cristiano</au><au>Melis, Maurizio</au><au>Luisa Delodovici, Maria</au><au>Dallocchio, Carlo</au><au>Gandolfo, Carlo</au><au>Cerrato, Paolo</au><au>Motto, Cristina</au><au>Melis, Fabio</au><au>Chiti, Alberto</au><au>Gentile, Mauro</au><au>Bignamini, Valeria</au><au>Morotti, Andrea</au><au>Maria Lotti, Enrico</au><au>Toriello, Antonella</au><au>Costa, Paolo</au><au>Silvestrelli, Giorgio</au><au>Zini, Andrea</au><au>De Giuli, Valeria</au><au>Poli, Loris</au><au>Paciaroni, Maurizio</au><au>Lodigiani, Corrado</au><au>Marcheselli, Simona</au><au>Sanguigni, Sandro</au><au>Del Sette, Massimo</au><au>Monaco, Serena</au><au>Lochner, Piergiorgio</au><au>Zanferrari, Carla</au><au>Anticoli, Sabrina</au><au>Padovani, Alessandro</au><au>Pezzini, Alessandro</au><aucorp>Italian Project on Stroke at Young Age (IPSYS) Investigators</aucorp><aucorp>on behalf of the Italian Project on Stroke at Young Age (IPSYS) Investigators</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)</atitle><jtitle>Cephalalgia</jtitle><addtitle>Cephalalgia</addtitle><date>2019-09-01</date><risdate>2019</risdate><volume>39</volume><issue>10</issue><spage>1267</spage><epage>1276</epage><pages>1267-1276</pages><issn>0333-1024</issn><eissn>1468-2982</eissn><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.</abstract><cop>London, England</cop><pub>SAGE Publications</pub><pmid>31060368</pmid><doi>10.1177/0333102419849013</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0001-8629-3315</orcidid></addata></record>
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source Sage Journals GOLD Open Access 2024
title The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)
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