Fatal reversible cerebral vasoconstriction syndrome: An unusual presentation of a rare disease
Abstract
The reversible cerebral vasoconstriction syndrome is a variable, segmental, and multifocal constriction of brain arteries, usually with a benign course.
We describe the case of a 49-year-old woman who presented with headaches, visual symptoms, and seizures. Three days after admission, vasoconstriction areas were found in at least two vascular territories in two segments of the same arteries. The patient was admitted to the intensive care unit where her blood pressure was monitored and she received medical treatment. Surprisingly, the patient presented an unpredicted evolution in developing malignant cerebral edema on the seventh day after admission. She then suffered brain death and was taken to organ donation. A guided nervous system necropsy was later performed. The pathology discarded vasculitis and exhibited hemorrhage areas in the cerebral convexity.
Herein, we discuss the most relevant aspects of cases with fulminant evolution reported in the literature. The reversible cerebral vasoconstriction syndrome is usually associated with fatal outcomes when patients exhibit focalization, their first neuroimaging typically shows disturbances, and a rapid clinical deterioration occurs. It is crucial to identify factors linked to poor prognosis and set intervention strategies and early prevention.
Downloads
References
Choi HA, Lee MJ, Chung C-S. Cerebral endothelial dysfunction in reversible cerebral vasoconstriction syndrome: A case-control study. J Headache Pain. 2017;18:29. https://doi.org/10.1186/s10194-017-0738-x
Pilato F, Distefano M, Calandrelli R. Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome: Clinical and radiological considerations. Front Neurol. 2020;11:34. https://doi.org/10.3389/fneur.2020.00034
Burton TM, Bushnell CD. Reversible cerebral vasoconstriction syndrome: A diagnostic imaging review. Stroke. 2019;50:2253-8. https://doi.org/10.1161/STROKEAHA.119.024416
Cappelen-Smith C, Calic Z, Cordato D. Reversible cerebral vasoconstriction syndrome: Recognition and treatment. Curr Treat Options Neurol. 2017;19:21. https://doi.org/10.1007/s11940-017-0460-7
Koopman K, Teune LK, Laan M, Uyttenboogaart M, Vroomen PC, De Keyser JD, et al. An often unrecognized cause of thunderclap headache: Reversible cerebral vasoconstriction syndrome. J Headache Pain. 2008;9:389-91. https://doi.org/10.1007/s10194-008-0068-0
Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, part 1: Epidemiology, pathogenesis, and clinical course. Am J Neuroradiol. 2015;36:1392-9. https://doi.org/10.3174/ajnr.A4214
Bouvy C, Ackermans N, Maldonado-Slootjes S, Rutgers MP, Gille M. Reversible cerebral vasoconstriction syndrome revealed by fronto-callosal infarctions. Acta Neurol Belg. 2020;120:1467-9. https://doi.org/10.1007/s13760-020-01319-0
Lee SH, Yun SJ, Choi YH. Reversible cerebral vasoconstriction syndrome presenting as subarachnoid hemorrhage: A rare cause of postpartum seizure. Am J Emerg Med. 2017;35:807.e1-3. https://doi.org/10.1016/j.ajem.2016.12.021
Chen SP, Yang AC, Fuh JL, Wang SJ. Autonomic dysfunction in reversible cerebral vasoconstriction syndromes. J Headache Pain. 2013;14:94. https://doi.org/10.1186/1129-2377-14-94
Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: Reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146:34-44. https://doi.org/10.7326/0003-4819-146-1-200701020-00007
Singhal AB, Hajj-Ali RA, Topcuoglu MA, Fok J, Bena J, Yang D, et al. Reversible cerebral vasoconstriction syndromes: Analysis of 139 cases. Arch Neurol. 2011;68:1005-12. https://doi.org/10.1001/archneurol.2011.68
Robert T, Kawkabani Marchini A, Oumarou G, Uske A. Reversible cerebral vasoconstriction syndrome identification of prognostic factors. Clin Neurol Neurosurg. 2013;115:2351-7. https://doi.org/10.1016/j.clineuro.2013.08.014
Lozupone E, Distefano M, Calandrelli R, Marca GD, Pedicelli A, Pilato F. Reversible cerebral vasoconstriction syndrome: A severe neurological complication in postpartum period. Neurol India.2020;68:192-8. https://doi.org/10.4103/0028-3886.279674
Abadía L, Castañeda C, Méndez JA, Coral J, Zarco LA. Síndrome de vasoconstricción cerebral reversible: revisión de tema. Universitas Médica. 2014;56:226-34.
Topcuoglu MA, Singhal AB. Hemorrhagic reversible cerebral vasoconstriction syndrome: Features and mechanisms. Stroke. 2016;47:1742-7. https://doi.org/10.1161/STROKEAHA.116.013136
Suchdev K, Norris G, Zak I, Mohamed W, Ibrahim M. Fulminant reversible cerebral vasoconstriction syndrome. Neurohospitalist. 2018;8:NP5-8. https://doi.org/10.1177/1941874417692923
Enríquez P, Ariza-Varón M, Enríquez MN, Navarro CE. Síndrome de vasoconstricción cerebral reversible inducido por maniobra de Valsalva: reporte de caso y revisión de la literatura. Acta Neurológica Colombiana. 2020;36:81-6.
Pop A, Carbonnel M, Wang A, Josserand J, Auliac SC, Ayoubi J-M. Posterior reversible encephalopathy syndrome associated with reversible cerebral vasoconstriction syndrome in a patient presenting with postpartum eclampsia: A case report. J Gynecol Obstet Hum Reprod. 2019;48:431-4. https://doi.org/10.1016/j.jogoh.2019.03.019
Singhal AB. Cerebral vasoconstriction syndromes. Top Stroke Rehabil. 2004;11:1-6. https://doi.org/10.1310/ATK7-QTP7-7NE2-5G8X
Kunchok A, Castley HC, Aldous L, Hawke SH, Torzillo E, Parker GD, et al. Fatal reversible cerebral vasoconstriction syndrome. J Neurol Sci. 2018;385:146-50.
Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care. 2005;3:91-7. https://doi.org/10.1385/NCC:3:1:091
Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11:906-17. ttps://doi.org/10.1016/S1474-4422(12)70135-7
Sattar A, Manousakis G, Jensen MB. Systematic review of reversible cerebral vasoconstriction syndrome. Expert Rev Cardiovasc Ther. 2010;8:1417-21. https://doi.org/10.1586/erc.10.124
Williams TL, Lukovits TG, Harris BT, Harker Rhodes C. A fatal case of postpartum cerebral angiopathy with literature review. Arch Gynecol Obstet. 2007;275:67-77. https://doi.org/10.1007/s00404-006-0194-3
Ducros A, Bousser M-G. Reversible cerebral vasoconstriction syndrome. Pract Neurol. 2009;9:256-67. https://doi.org/10.1136/jnnp.2009.187856
Ducros A, Wolff V. The typical thunderclap headache of reversible cerebral vasoconstriction syndrome and its various triggers. Headache. 2016;56:657-73. https://doi.org/10.1111/head.12797
Calado S, Vale-Santos J, Lima C, Viana-Baptista M. Postpartum cerebral angiopathy: Vasospasm, vasculitis or both? Cerebrovasc Dis. 2004;18:340-1. https://doi.org/10.1159/000080976
Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. RCVS(2) score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 2019;92:e639-47. https://doi.org/10.1212/WNL.0000000000006917
Valencia-Mendoza M, Ramírez-Rodríguez N, Vargas-Ávila N, Peña-Ortiz A, Corzo-Villamizar M, Serna-Ramírez L, et al. Fatal reversible cerebral vasoconstriction syndrome: A systematic review of case series and case reports. J Clin Neurosci. 2019;70:183-8. https://doi.org/10.1016/j.jocn.2019.08.014
Some similar items:
- Cristian Antony Ramos-Vera, Juan Camilo Motta, Letter to the editor "Prognostic factors in hospitalized patients diagnosed with SARS-CoV-2 infection, Bogotá, Colombia" , Biomedica: Vol. 41 No. 2 (2021)
- Juan Camilo Motta , Danny Julian Novoa, Carmen Cecilia Gómez, Julian Mauricio Moreno, Lina Vargas, Jairo Pérez, Henry Millán, Álvaro Ignacio Arango, Prognostic factors in hospitalized patients diagnosed with SARS-CoV-2 infection, Bogotá, Colombia , Biomedica: Vol. 40 No. Supl. 2 (2020): SARS-CoV-2 y COVID-19
- Andrés Leonardo González, Ruth Aralí Martínez, Luis Ángel Villar, Clinical evolution of dengue in hospitalized patients , Biomedica: Vol. 28 No. 4 (2008)
- Ignacio Zarante, Liliana Franco, Catalina López, Nicolás Fernández, Frequencies of congenital malformations: assessment and prognosis of 52,744 births in three cities of Colombia , Biomedica: Vol. 30 No. 1 (2010)
- Karen Melissa Ordóñez, Odismar Andrea Hernández, Jorge Alberto Cortés, María José López, Gladys Alfonso, Alejandro Junca, Left-sided infective endocarditis caused by Pseudomonas aeruginosa treated medically , Biomedica: Vol. 30 No. 2 (2010)
- Jairo Lizarazo, Melva Linares, Catalina de Bedout, Ángela Restrepo, Clara Inés Agudelo, Elizabeth Castañeda, Grupo Colombiano para el Estudio de la Criptococosis, Results of nine years of the clinical and epidemiological survey on cryptococcosis in Colombia, 1997-2005 , Biomedica: Vol. 27 No. 1 (2007)
- Álvaro J. Ruiz, Pablo J. Aschner, María Fernanda Puerta, Rafael Alfonso-Cristancho, IDEA Study (International Day for the Evaluation of Abdominal Obesity): Primary care study of the prevalence of abdominal obesity and associated risk factors in Colombia , Biomedica: Vol. 32 No. 4 (2012)
- Sergio Velásquez, Juan D. Matute, Laura Y. Gámez, Luis E. Enríquez, Iván D. Gómez, Fabiola Toro, Martha L. Valencia, Gisela De La Rosa, Pablo J. Patiño, Fabián A. Jaimes, Characterization of nCD64 expression in neutrophils and levels of s-TREM-1 and HMGB-1 in patients with suspected infection admitted in an emergency department , Biomedica: Vol. 33 No. 4 (2013)
- Luis Eduardo Echeverría, Clara Saldarriaga, Sebastián Campbell-Quintero, Lisbeth Natalia Morales-Rodríguez, Juan David López-Ponce de León, Andrés Felipe Buitrago, Erika Martínez-Carreño, Jorge Alberto Sandoval-Luna, Alexis Llamas, Gustavo Adolfo Moreno-Silgado, Julián Vanegas-Eljach, Nelson Eduardo Murillo-Benítez, Ricardo Gómez-Paláu, Alex Arnulfo Rivera-Toquica, Juan Esteban Gómez-Mesa, RECOLFACA research group, Diabetes mellitus in patients with heart failure and effect modification of risk factors for short-term mortality: An observational study from the Registro Colombiano de Falla Cardíaca (RECOLFACA) , Biomedica: Vol. 44 No. Sp. 1 (2024): Enfermedades crónicas no transmisibles
- Doris Cardona, Ángela María Segura, Adriana Espinosa, Alejandra Segura, Homicide and suicide in 15 to 24 year-old people in Colombia, 1998-2008 , Biomedica: Vol. 33 No. 4 (2013)
| Article metrics | |
|---|---|
| Abstract views | |
| Galley vies | |
| PDF Views | |
| HTML views | |
| Other views | |
