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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 635-637

Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus

1 Department of Neurology, Malabar Institute of Medical Sciences, Calicut, Kerala, India
2 Department of Rheumatology, Malabar Institute of Medical Sciences, Calicut, Kerala, India
3 Department of Radiology, Malabar Institute of Medical Sciences, Calicut, Kerala, India

Date of Submission12-Oct-2012
Date of Decision14-Oct-2012
Date of Acceptance28-Oct-2012
Date of Web Publication29-Dec-2012

Correspondence Address:
V V Ashraf
Department of Neurology, Malabar Institute of Medical Sciences, Govindapuram PO,Calicut - 673 016, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.105200

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 » Abstract 

A 42-year-old woman, a diagnosed case of systemic lupus erythematosus (SLE), developed severe headache followed by left hemiparesis and cortical blindness. Magnetic resonance imaging (MRI) of brain demonstrated right parieto-occipital infarct and the patient was initiated on high-dose steroids and antiplatelet agents with which the patient had clinical and radiological deterioration. Magnetic Resonance angiography showed severe narrowing of bilateral anterior, middle, and posterior cerebral arteries (PCA) suggestive of reversible cerebral vasoconstriction syndrome (RCVS). Patient was treated with IV nimodipine and she recovered over a period of 2 weeks. Repeat MR-angiography done on day-30 was normal. The diagnosis of RCVS in patients with SLE is of practical importance, because treatment and prognosis for SLE-associated cerebral vasculitis differ strongly from the treatment of RCVS. Empirical high-dose glucocorticoid therapy should be avoided in patients with typical features of RCVS.

Keywords: Cerebral vasculitis, reversible cerebral vasoconstriction syndrome, systemic lupus erythematosus

How to cite this article:
Ashraf V V, Bhasi R, Ramakrishnan K G, Praveenkumar R, Girija A S. Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus. Neurol India 2012;60:635-7

How to cite this URL:
Ashraf V V, Bhasi R, Ramakrishnan K G, Praveenkumar R, Girija A S. Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus. Neurol India [serial online] 2012 [cited 2021 Mar 9];60:635-7. Available from:

 » Introduction Top

Headaches are common in patients with systemic lupus erythematosus (SLE), especially those suffering from central nervous system (CNS) involvement with or without cerebral vasculitis. [1] Incidence of strokes in patients with SLE is about 3-20%. [2] RCVS is a clinical-angiographic syndrome characterized by an abrupt onset of severe headaches, seizures, focal neurological deficits, and multiple segmental narrowing of large and medium-sized arteries, which resolves spontaneously in 1-3 months, and typically occurs in women aged between 20 and 50 years. [3] RCVS and cerebral vasculitis have several overlapping features. We report a case of RCVS in an SLE patient mimicking lupus-associated cerebral vasculitis.

 » Case Report Top

A 42-year-old lady was evaluated and diagnosed to have SLE at our rheumatology clinic 3 years ago and she was put on low dose steroids and hydroxychloroquine sulfate. She had occasional flare-up of articular symptoms, but had no other organ systems involvement. On April 2011, she was admitted into another facility with severe "thunderclap headache" associated with nausea and vomiting. She also experienced blurring of vision at the peak of headache. Physical examination was normal. Computed tomography (CT) scan and magnetic resonance imaging (MRI) brain were normal. Cerebrospinal fluid analysis was normal and MR-angiography of intracranial vessels was reported to be normal. She was managed with analgesics and antidepressants and steroid dose was hiked. On day-7 she developed weakness of left half of body and blurred vision in the left hemifield and was transferred to our center. Neurologic examination revealed left hemiparesis and left homonymous hemianopia. MRI brain revealed right parieto-occipital T2-weighted and Fluid attenuated inversion recovery (FLAIR) hyperintensity and restricted diffusion suggestive of infarct. Blood investigations revealed positive anti-nuclear antibody (ANA), double-stranded DNA (dsDNA). Complement 3 and complement 4 were in normal range. Other blood investigations were normal. Considering the possibility of stroke secondary to lupus associated cerebral vasculitis, she was initiated on high-dose intravenous steroids, anti-platelets, and heparin. On the next day, her neurological deficits worsened and she developed cortical blindness and became bed-bound. Repeat MRI brain showed infarcts in both parieto-occipital regions [Figure 1]a and b. Repeat cerebral MR-angiography showed marked short segment constriction of A1 segment of bilateral internal carotid artery (ICA), M1 segment of bilateral middle cerebral artery (MCA), basilar artery and bilateral posterior cerebral arteries (PCA) [Figure 1]c. A diagnostic possibility of RCVS was considered. She was treated with nimodipine, dopamine infusion as a pressor agent and oral steroids were continued. She made a remarkable recovery in the next 2 weeks except for mild blurred vision. Control cerebral MR-angiography done 30 days later demonstrated complete regression of the vasoconstriction [Figure 1]d confirming the diagnosis of RCVS.
Figure 1: (a) Brain magnetic resonance image (MRI), Fluid attenuated inversion recovery (FLAIR) sequence shows hyperintensities over both parieto‑occipital regions (b) MRI brain diffusion sequences shows bright signals over both parieto‑occipital regions suggestive of infarcts (c) MR angiogram of brain shows marked short segment constriction of A1 segment of bilateral internal carotid artery (ICA), M1 segment of bilateral middle cerebral artery (MCA), basilar artery and bilateral posterior cerebral arteries (PCA) (d) Control cerebral MR angiogram (MRA) after 30 days shows complete regression of cerebral vasoconstriction

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 » Discussion Top

Reversible cerebral vasoconstriction syndrome is a unifying term used to describe a group of disorders sharing cardinal angiographic and clinical features, namely, a reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurological deficits or seizures. [3] The most common clinical feature of RCVS is severe headache, often qualifying as thunderclap headache. Major complication of RCVS is stroke, ischemic or hemorrhagic, eventually leading to permanent sequelae or even death. Non aneurysmal subarachnoid hemorrhage and reversible posterior leukoencephalopathy are the other reported complications. [3] Diagnosis can be hampered by the dynamic nature of clinic-radiological features. Stroke can occur a few days after initial normal imaging and cerebral vasoconstriction is at a maximum on angiograms 2-3 weeks after clinical onset. [4] Our patient had normal brain imaging and cerebral angiogram during the first week of illness. Although pathophysiology of RCVS remains unknown, the prevailing hypothesis involves a transient disturbance in the control of cerebral vascular tone. It may occur spontaneously or be provoked by various precipitating factors, the most common being postpartum and exposure to various vasoactive substances. There are reports of cyclophosphamide precipitating RCVS. [3]

True cerebral vasculitis is considered to be a rare cause of stroke in SLE. The important contributory factors to the development of stroke in patients with SLE include: Hypertension mediated by immunologic abnormalities, coagulation abnormalities due to anti-phospholipid antibodies, and cardiogenic embolism due to endocarditis. [5] A true cerebral vasculitis in association with SLE, as evidenced by the presence of inflammatory cells within the vessel wall is rare in postmortem studies with an incidence of 0-7%. [6] Although SLE is frequently cited as an etiology for angiographically apparent cerebral vasculitis, only very few cases of SLE cerebral vasculitis have been reported. [7] Most of the reported cases had isolated findings more appropriately termed as vasculopathy and not completely conclusive vasculitis. [7] Mitsias et al. in a series of thirty patients with SLE and large-artery strokes, reported typical angiographic features of cerebral vasculitis in only two patients. [5] The typical angiographical features attributed to SLE cerebral vasculitis include arterial occlusions, focal ectasia, especially at bifurcations, and aneurysms. [8] Cerebral vasculitis in SLE patients usually occurs in patients with active lupus, [9] whereas our patient had an inactive lupus when this event occurred.

Despite significant advances in characterizing RCVS and cerebral vasculitis, physicians remain fearful of delaying immunosuppressive therapy in patients who may ultimately prove to have cerebral vasculitis. Hence, patients who have RCVS are frequently administered immunosuppressive agents. Our patient had clinical and radiological deterioration after administering high-dose steroids. Similar observation of neurological deterioration with high dose steroids in RCVS was reported in two patients. [10] Shingal et al.[11] studied the effects of empirical steroids in a series of 139 patients with RCVS. The authors noted a trend for poor outcome in patients treated with steroids. Possible explanation given by the authors was that, steroids were initiated in sicker patients with severe vasoconstriction or established brain lesions. Until further studies are performed, it is prudent to focus on distinguishing RCVS from cerebral vasculitis and to withhold empirical steroid therapy in patients exhibiting the typical clinical and imaging features of RCVS.

 » References Top

1.Joseph FG, Lammie GA, Scolding NJ. CNS lupus: A study of 41 patients. Neurology 2007;69:644-54.  Back to cited text no. 1
2.Kitagawa Y, Gotoh F, Koto A, Okayasu H. Stroke in systemic lupus erythematosus. Stroke 1990;21:1533-9.  Back to cited text no. 2
3.Durcose A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11:906-17.  Back to cited text no. 3
4.Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091-101.  Back to cited text no. 4
5.Mitsias P, Levine RS. Large cerebral vessel occlusive disease in systemic lupus erythematosus. Neurology 1994;44:385-93.  Back to cited text no. 5
6.Weiner DK, Allen NB. Large vessel vasculitis of the central nervous system in systemic lupus erythematosus: Report and review of the literature. J Rheumatol 1991;18:748-51.  Back to cited text no. 6
7.Liem MD, Gzesh DJ, Flanders AE. MRI and angiographic diagnosis of lupus cerebral vasculitis. Neuroradiology 1996;38:134-6.  Back to cited text no. 7
8.Ferris EJ, Levine HL. Cerebral arteritis: Classification. Radiology 1973;109:327-41.  Back to cited text no. 8
9.Sayegh J, Marc G, Augusto JF, Caroff J, Subra JF, Besson V. Reversible cerebral vasoconstriction syndrome in a female patient with systemic lupus erythematosus. Rheumatology (Oxford) 2010;49:1993-4.  Back to cited text no. 9
10.French KF, Hoesch RE, Allred J, Wilder M, Smith AG, Digre KB, et al. Repetitive use of intra-arterial verapamil in the treatment of reversible cerebral vasoconstriction syndrome. J Clin Neurosci 2012;19:174-6.  Back to cited text no. 10
11.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.  Back to cited text no. 11


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