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CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 1  |  Page : 192-198

Reversible Cerebral Vasoconstriction Syndrome: A Severe Neurological Complication in Postpartum Period


1 Department of Di Diagnostica Per Immagini, Radioterapia Oncologica Ed Ematologia, UOC Radiologia e Neuroradiologia, Roma, Italia
2 Department of Di Scienze Dell'Invecchiamento, Neurologiche, Ortopediche e Della Testa-Collo, UOC Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italia

Date of Web Publication28-Feb-2020

Correspondence Address:
Dr. Fabio Pilato
Polo Scienze Dell'Invecchiamento, Neurologiche, Ortopediche E Della Testa-Collo, Area Neuroscienze, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, Largo Francesco Vito 1 – 00168, Roma
Italia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.279674

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 » Abstract 


A 38-year-old woman 12 days after delivery of her second pregnancy was admitted to emergency room for a severe occipital headache started 3 days before, associated with confusion, nausea, vomiting and walking impairment. Neurological examination showed left hemiparesis, hypoesthesia in left arm and leg. Brain computer tomography images showed a large intraparenchymal hematoma in the right frontoparietal lobes with mass effect on adjacent subarachnoid spaces and on lateral ventricle. The third day during hospitalization, the patient experienced a sudden worsening of the neurological symptoms and a severe headache peaking within 1 minute (min); a new brain computed tomography and brain magnetic revealed another small intraparenchymal hematoma in the left parietal lobe with increase of the amount of subarachnoid hemorrhage. Digital subtraction angiography discloses multifocal narrowing of the middle and small arteries in both anterior and posterior circulation with a relative spare of large vessels. Postpartum reversible cerebral vasoconstriction syndrome with intracranial hemorrhage is a rare clinical condition that can be misdiagnosed with other dramatic neurological diseases such as eclamptic encephalopathy, cortical venous thrombosis, primary angiitis of the central nervous system or posterior reversible encephalopathy syndrome with whom may share predisposing factors and neurological presentation but clinical course, treatment and prognosis is quite different and emergency physicians and neurologists should consider this diagnosis in postpartum patients with hemorrhage.


Keywords: Digital subtraction angiography, headache, intracranial hemorrhage, magnetic resonance imaging, reversible cerebral vasoconstriction syndrome
Key Messages: RCVS is a rare clinical condition that can be confused with other dramatic neurological diseases but it should be taken into account in postpartum patients because prompt recognition may avoid time loss for appropriate treatment.


How to cite this article:
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

How to cite this URL:
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 [serial online] 2020 [cited 2020 Mar 31];68:192-8. Available from: http://www.neurologyindia.com/text.asp?2020/68/1/192/279674




A 38-year-old woman 12 days after delivery of her second pregnancy was admitted to emergency room for a severe occipital headache started 3 days earlier and was associated with confusion, nausea, vomiting and walking impairment. She had been treated with Low-Molecular-Weight-Heparin (LMWH) at the dosage of 4000 UI daily during pregnancy because of her history of heterozygosis for factor V Leiden. She denied foreign travel, consumption of alcohol, tobacco, illicit drugs, nutritional supplements, herbal remedies and risky sexual behaviors. She had a 2-year-old child after a cesarean section following an uncomplicated pregnancy. On general examination, she had high systolic blood pressure (160/80 mmHg), she was afebrile and postpartum abdominal examination was unremarkable.

Neurological examination showed left hemiparesis, hypoesthesia in left arm and leg and she was unable to walk. Blood tests were unremarkable. Brain computer tomography (CT) images [Figure 1]a and [Figure 1]b showed a large intraparenchymal hematoma in the right frontoparietal lobes with mass effect on adjacent subarachnoid spaces and on lateral ventricle. A small amount of subarachnoid hemorrhage (SAH) was also demonstrated in the both frontoparietal cortical sulci on both sides, whereas CT-angiography ruled out large vessel occlusion, cerebral aneurysms, brain arteriovenous malformations and dural sinus thrombosis [Figure 1]c. The third day during hospitalization, the patient experienced a sudden worsening of the neurological symptoms and she complained severe headache peaking within 1 minute (min); a new brain CT and brain magnetic resonance (MR) disclosed another small intraparenchymal hematoma in the left parietal lobe with increase of the amount of SAH [Figure 1]d-f].
Figure 1: Brain axial Non-Enhanced Computer Tomography (NECT) images (a and b) demonstrated a large cortical--subcortical hematoma (asterisk) in the right mesial frontoparietal lobe with a small amount of SAH in the frontoparietal sulci of both hemispheres (arrows). Axial Maximum Intensity Projection (MIP) CT-angiography (c) images ruled out large vessel occlusion, vascular malformations and dural sinus thrombosis. Axial NECT image (d) performed 2 days after symptoms onset detected a new small hematoma in the left parietal lobe (arrowhead) and the increase of the SAH in the adjacent sulci. Axial MR Fluid Attenuated Inversion Recovery (FLAIR) (e) and SWI (f) MR images better disclosed the two hematomas, surrounded by vasogenic edema, and the SAH in the frontoparietal cortical sulci of both hemispheres (arrows)

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Digital subtraction angiography (DSA) revealed a multifocal narrowing of the middle and small arteries in both anterior and posterior circulation with a relative spare of large vessels [Figure 2]. The vessels involved showed stenosis with dilation of the poststenotic segment giving beaded appearance/sausage-shaped arteries [Figure 2]. No vessel abnormality was detected in extracranial and splanchnic vessels (renal arteries).
Figure 2: DSA performed 6 days after the clinical onset. Frontal angiogram (a) and oblique magnification (b) of right anterior circulation disclose multiple focal narrowing distal branches of Middle Cerebral Artery (MCA) and Anterior Cerebral Artery (ACA) (arrows). Few occluded branches with ipovascular area in the region of the right frontoparietal hematoma is demonstrated (asterisk). Oblique magnification angiogram (c) of left anterior circulation highlights the typical “string of pearls” appearance of a distal branch of ACA (white arrowheads). Similar findings are founded in PCA territories (d and e) and in the right PICA (f) (arrows). A small pseudoaneurysm of V3 segment of right vertebral artery is detected (black arrowhead)

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 » Follow up Top


Neurological symptoms slightly improved during hospitalization and she was discharged to rehabilitation ward to continue physiotherapy.

DSA performed three months later [Figure 3] disclosed no intracranial arteries abnormalities and the narrowing of the middle and small arteries in both anterior and posterior circulation vanished; the patient showed motor functions improvements and she returned to her normal life.
Figure 3:Upper sequence: Follow-up DSA was performed 3 months after the clinical onset. Complete resolution of the multiple stenosis were demonstrated in both anterior (a and b) and posterior circulation (c and d). Lower sequence: (e-h) Angiographic findings at the beginning for comparison

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 » Clinical Considerations Top


Our patient showed acute onset of severe headache and neurologic signs and symptoms developed in puerperium period and neuroimaging showed brain hemorrhage and SAH; DSA ruled out aneurysms or vascular malformations but it showed sausage-shaped arteries and vasoconstriction in small and medium arteries. During hospitalization patient complained again of her headache peaking within 1 min suggestive of thunderclap headache (TCH). At 3 month follow-up visit, DSA showed the complete vanishing of the vasoconstriction, clinical symptoms disappeared and neurological deficits improved. These features are suggestive of Reversible Cerebral Vasoconstriction Syndrome (RCVS).

RCVS must be differentiated from primary angiitis of the central nervous system (PACNS), a condition with similar angiographic abnormalities but a substantially different diagnostic and therapeutic approach.[1]

RCVS is characterized by severe headaches with or without other acute neurological symptoms and diffuse segmental constriction of cerebral arteries that resolves spontaneously within 3 months. Manifestations are attributed to a transient disturbance of the regulation of cerebral arterial tone. Thunderclap headache—severe pain peaking in seconds—is usually the first symptom and typically recurs for 1–2 weeks. Some authors proposed the name RCVS and a set of diagnostic criteria to regroup all similar cases that had been reported[1] to differentiate these patients from others with similar symptoms but expression of different diseases.

RCVS is a group of syndromes characterized by reversible segmental constriction of cerebral arteries, typically associated with severe headaches and often complicated by ischemic or hemorrhagic stroke.[2] Only one-third of patients initially present with hemorrhagic stroke, typically isolated cortical subarachnoid hemorrhage with or without superficial intracerebral hemorrhage (ICH). Some patients (less than 3%) may show isolated deep ICH, making differential diagnosis challenging.[3]

RCVS may share some characteristics with posterior reversible encephalopathy syndrome (PRES) and occasionally they might be mutually misinterpreted[4],[5] or may be concurrent.[6] The pathophysiology of RCVS and PRES remain poorly understood, and it has been hypothesized that PRES and RCVS represent a spectrum of pathophysiology related to cerebral vascular dysregulation and dysfunction. Moreover, RCVS may share some clinical and neuroradiological features with PACNS and they may be misdiagnosed occasionally even if prognosis is opposed: a self-limited benign syndrome the former and devastating neurological condition with potentially fatal outcome the latter[7] but rare cases of RCVS-PACNS overlap syndrome were described.[8] The key diagnostic criteria for RCVS were first proposed by Calabrese et al.[1] in 2007 and then modified in 2012.[2] The criteria include: (1) acute and severe headache (often thunderclap) with or without focal deficits or seizures; (2) monophasic course without new symptoms more than 1 month after clinical onset; (3) segmental vasoconstriction of cerebral arteries shown by indirect (e.g. magnetic resonance or CT) or direct catheter angiography; (4) no evidence of aneurysmal subarachnoid hemorrhage; normal or near-normal Cerebrospinal Fluid (CSF) (protein concentrations <100 mg/dL, <15 white blood cells per μL); complete or substantial normalization of arteries shown by follow-up indirect or direct angiography within 12 weeks of clinical onset. Although these criteria have not been validated in prospective studies yet, they are considered very useful to diagnose RCVS and to increase physician awareness of the disease. Few reports described RCVS in postpartum period associated with hemorrhagic complications [Table 1] and recurrent lesions are even more rare [Table 2].[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32]
Table 1: Postpartum RCVS with intracranial hemorrhage: Clinical features

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Table 2: Postpartum RCVS with intracranial hemorrhage: Prognosis and follow-up

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 » Discussion Top


Postpartum RCVS also called Call–Fleming syndrome[9] or post-partum angiopathy is a group of syndromes characterized by reversible segmental constriction of cerebral arteries, typically associated with severe headaches and often complicated by ischemic or hemorrhagic stroke.

The presentation of RCVS is usually dramatic, with sudden, excruciating headaches that reach peak intensity within seconds, meeting the definition for “thunderclap headache”.[10] The thunderclap headaches tend to recur over a span of days to weeks. Approximately one-third of patients develop ischemic or hemorrhagic strokes or reversible brain edema.[2] Less than 10% of patients with RCVS present with subacute or less severe headaches and the absence of headache at onset is exceptional.[7] Clinical symptoms at the onset may vary and diagnosis may be challenging. Although RCVS is usually a benign and nonrelapsing disease, fatalities have been reported and sometimes it may recur.[11]

Several factors have been associated with hemorrhage in RCVS patients such as migraine history and female gender[3] but despite the dramatic onset over 90% of patients have excellent clinical outcome; however, some factors such as glucocorticoid therapy, intraarterial vasodilator therapy, and infarction on baseline imaging may be associated with poor outcome.[12]

Diagnostic criteria for RCVS are commonly accepted[1] and recent studies tried to identify differences between RCVS and PACNS highlighting the importance of clinical features (TCH, pregnancy or puerperium), neuroimaging (nonconvexal SAH) and angiographic results (sausaging) that should be diagnostic for RCVS.[7]


 » Conclusion Top


In conclusion, postpartum RCVS with intracranial hemorrhage is a rare clinical condition that can be confused with other dramatic neurological diseases, such as eclamptic encephalopathy, cortical venous thrombosis, PACNS, or PRES but emergency physicians and neurologists should take into account this condition in differential diagnosis in postpartum patients because these conditions may share predisposing factors and neurological presentation but clinical course, treatment, and prognosis is quite different and the prompt recognition may avoid time loss for treatment in PACNS or overtreatment in RCVS.

Ethical standards

Patient gave her informed consent prior to her inclusion in the study.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

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Ducros A, Fiedler U, Porcher R, Boukobza M, Stapf C, Bousser MG. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: Frequency, features, and risk factors. Stroke 2010;41:2505-11.  Back to cited text no. 3
    
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Nabavizadeh A, Vossough A. Reversible cerebral vasoconstriction syndrome vs posterior reversible encephalopathy syndrome. JAMA Neurol 2016;73:606.  Back to cited text no. 4
    
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Chan T, Mayich M, Budhram A, Sallam Y, Becker W. Teaching images in headache: Concurrent hypercalcemia-induced reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome. Headache J Head Face Pain 2019;59:933-5.  Back to cited text no. 6
    
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Calado S, Vale-Santos J, Lima C, Viana-Baptista M. Postpartum cerebral angiopathy: Vasospasm, vasculitis or both? Cerebrovasc Dis 2004;18:340-1.  Back to cited text no. 8
    
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Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke 1988;19:1159-70.  Back to cited text no. 9
    
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Singhal A, Topcuoglu M. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology 2017;88:228-36.  Back to cited text no. 12
    
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32.
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

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