Reversible cerebral vasoconstriction syndrome is an unusual entity, characterized by recurrent thunderclap headache and segmental narrowing of the cerebral arteries, typically with remission within three months. It has been described since the 1960s with several names, including Call-Fleming syndrome. More than 500 cases have been described in the literature, yet the pathophysiology remains not well understood. Ischemic or hemorrhagic strokes are the major possible complications of the syndrome, leading to permanent neurological deficits or death in a small percentage of patients. We report a case of a 48-year-old female patient without known risk factors that presented two foci of intracerebral hemorrhages, with hemiparesis ipsilateral to the biggest intracerebral lesion. Magnetic resonance imaging tractography revealed normal pyramidal decussation, and the patient evolved with completely recover of the neurological deficit within a week. The authors believe that in the present case neurological deficit may be related to contralateral narrowing of the cerebral arteries and diffuse impairment of the central nervous system instead of intracerebral hemorrhage itself. RCVS is a rare condition that should be considered in patients with recurrent thunderclap type headache. Further prospective and randomized studies are still necessary to improve the management and treatment of patients with the syndrome.
Published in | Clinical Neurology and Neuroscience (Volume 3, Issue 3) |
DOI | 10.11648/j.cnn.20190303.13 |
Page(s) | 69-73 |
Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
Copyright |
Copyright © The Author(s), 2019. Published by Science Publishing Group |
Reversible Cerebral Vasoconstriction, Intracerebral Hemorrhages, Thunderclap Headache
[1] | Cappelen-Smith C, Calic Z, Cordato D. Reversible Cerebral Vasoconstriction Syndrome: Recognition and Treatment. Curr Treat Options Neurol 2017. 19: 21. |
[2] | Ducross A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012. 11: 906-17. |
[3] | Miller TR, Shivashankar M, Mossa-Basha M, Gandhi D. Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis and Clinical Course. AJNR Am J Neuroradiol 2015. 36: 1392-99. |
[4] | Call GK, Fleming MC, Sealfon S, Levine H, Kistler JF, Fisher CM. Reversible Cerebral Segmental Vasoconstriction. Stroke 1988. 19: 1159-70. |
[5] | Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: Reversible cerebral vasoconstriction syndromes. Ann InternMed 2007. 146: 34-44. |
[6] | Headache classification subcommittee of the International Headache Society. The international classification of headache disorders. Cephalalgia 2004. 24: 1–160. |
[7] | Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009. 9: 256-67. |
[8] | Gupta S, Zivadinov R, Ramasamy D, et al. Reversible cerebral vaso constriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators-case series and review of literature. Clin Rheumatol 2014; 33: 1829–33. |
[9] | Hiraga A, Aotsuka Y, Koide K, Suwabara. Reversible cerebral vasoconstriction syndrome precipitated by airplane descent: Case report. Cephalalgia 2016. 0 (0) 1-4. |
[10] | Costa I, Mendonça MD, Cruz e Silva V, Calado S, Viana-Baptista M. Herbal Supplements Association with Reversible Cerebral Vasoconstriction Syndrome: A Case Report. Journal of Stroke and Cerebrovascular Diseases 2017. Mar; 26 (3): 673-676. |
[11] | Choi HA, Lee MJ, Chung CS. Cerebral endothelial dysfunction in reversible cerebral vasoconstriction syndrome: a case-control study. The Journal of Headache and Pain 2017. 18: 29. |
[12] | Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011. 68: 1005–12. |
[13] | Song JK, Fisher S, Seifert TD, et al. Postpartum cerebral angiopathy: atypical features and treatment with intracranial balloon angioplasty. Neuroradiology 2004; 46: 1022–26. |
[14] | Elstner M, Linn J, Muller-Schunk S, Straube A. Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia 2009; 29: 677–82. |
[15] | Linn J, Fesl G, Ottomeyer C, et al. Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a diagnostic tool in select cases? Cephalalgia 2011; 31: 1074–81. |
[16] | Santos L, Azevedo E. Reversible cerebral vasoconstriction syndrome – A narrative revision of the literature. Porto Biomed. J 2016; 1 (2): 65-71. |
APA Style
François Dantas, Eustáquio Claret dos Santos Júnior, Tiago Silva e Carvalho, Róberti Uili Rodrigues Firmino, Rômulo Tscherbakowski Nunes de Guimarães Mourão, et al. (2019). Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review. Clinical Neurology and Neuroscience, 3(3), 69-73. https://doi.org/10.11648/j.cnn.20190303.13
ACS Style
François Dantas; Eustáquio Claret dos Santos Júnior; Tiago Silva e Carvalho; Róberti Uili Rodrigues Firmino; Rômulo Tscherbakowski Nunes de Guimarães Mourão, et al. Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review. Clin. Neurol. Neurosci. 2019, 3(3), 69-73. doi: 10.11648/j.cnn.20190303.13
AMA Style
François Dantas, Eustáquio Claret dos Santos Júnior, Tiago Silva e Carvalho, Róberti Uili Rodrigues Firmino, Rômulo Tscherbakowski Nunes de Guimarães Mourão, et al. Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review. Clin Neurol Neurosci. 2019;3(3):69-73. doi: 10.11648/j.cnn.20190303.13
@article{10.11648/j.cnn.20190303.13, author = {François Dantas and Eustáquio Claret dos Santos Júnior and Tiago Silva e Carvalho and Róberti Uili Rodrigues Firmino and Rômulo Tscherbakowski Nunes de Guimarães Mourão and Mariana Couy Fonseca and Fernando Luiz Rolemberg Dantas and Ricardo Vieira Botelho and Rogério Zenóbio Darwich}, title = {Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review}, journal = {Clinical Neurology and Neuroscience}, volume = {3}, number = {3}, pages = {69-73}, doi = {10.11648/j.cnn.20190303.13}, url = {https://doi.org/10.11648/j.cnn.20190303.13}, eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.cnn.20190303.13}, abstract = {Reversible cerebral vasoconstriction syndrome is an unusual entity, characterized by recurrent thunderclap headache and segmental narrowing of the cerebral arteries, typically with remission within three months. It has been described since the 1960s with several names, including Call-Fleming syndrome. More than 500 cases have been described in the literature, yet the pathophysiology remains not well understood. Ischemic or hemorrhagic strokes are the major possible complications of the syndrome, leading to permanent neurological deficits or death in a small percentage of patients. We report a case of a 48-year-old female patient without known risk factors that presented two foci of intracerebral hemorrhages, with hemiparesis ipsilateral to the biggest intracerebral lesion. Magnetic resonance imaging tractography revealed normal pyramidal decussation, and the patient evolved with completely recover of the neurological deficit within a week. The authors believe that in the present case neurological deficit may be related to contralateral narrowing of the cerebral arteries and diffuse impairment of the central nervous system instead of intracerebral hemorrhage itself. RCVS is a rare condition that should be considered in patients with recurrent thunderclap type headache. Further prospective and randomized studies are still necessary to improve the management and treatment of patients with the syndrome.}, year = {2019} }
TY - JOUR T1 - Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review AU - François Dantas AU - Eustáquio Claret dos Santos Júnior AU - Tiago Silva e Carvalho AU - Róberti Uili Rodrigues Firmino AU - Rômulo Tscherbakowski Nunes de Guimarães Mourão AU - Mariana Couy Fonseca AU - Fernando Luiz Rolemberg Dantas AU - Ricardo Vieira Botelho AU - Rogério Zenóbio Darwich Y1 - 2019/08/12 PY - 2019 N1 - https://doi.org/10.11648/j.cnn.20190303.13 DO - 10.11648/j.cnn.20190303.13 T2 - Clinical Neurology and Neuroscience JF - Clinical Neurology and Neuroscience JO - Clinical Neurology and Neuroscience SP - 69 EP - 73 PB - Science Publishing Group SN - 2578-8930 UR - https://doi.org/10.11648/j.cnn.20190303.13 AB - Reversible cerebral vasoconstriction syndrome is an unusual entity, characterized by recurrent thunderclap headache and segmental narrowing of the cerebral arteries, typically with remission within three months. It has been described since the 1960s with several names, including Call-Fleming syndrome. More than 500 cases have been described in the literature, yet the pathophysiology remains not well understood. Ischemic or hemorrhagic strokes are the major possible complications of the syndrome, leading to permanent neurological deficits or death in a small percentage of patients. We report a case of a 48-year-old female patient without known risk factors that presented two foci of intracerebral hemorrhages, with hemiparesis ipsilateral to the biggest intracerebral lesion. Magnetic resonance imaging tractography revealed normal pyramidal decussation, and the patient evolved with completely recover of the neurological deficit within a week. The authors believe that in the present case neurological deficit may be related to contralateral narrowing of the cerebral arteries and diffuse impairment of the central nervous system instead of intracerebral hemorrhage itself. RCVS is a rare condition that should be considered in patients with recurrent thunderclap type headache. Further prospective and randomized studies are still necessary to improve the management and treatment of patients with the syndrome. VL - 3 IS - 3 ER -