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Year : 2013  |  Volume : 61  |  Issue : 5  |  Page : 535--537

An unusual case of reversible cerebral vasoconstriction syndrome presenting with a large intraparenchymal haematoma

Munawwar Ahmed1, Shyamkumar N Keshava1, Suraj Mammen1, Ari G Chacko2, Krishna Prabhu2,  
1 Department of Radiology, Section of Neurosurgery, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Neurological Sciences, Section of Neurosurgery, Christian Medical College, Vellore, Tamil Nadu, India

Correspondence Address:
Munawwar Ahmed
Department of Radiology, Section of Neurosurgery, Christian Medical College, Vellore, Tamil Nadu

How to cite this article:
Ahmed M, Keshava SN, Mammen S, Chacko AG, Prabhu K. An unusual case of reversible cerebral vasoconstriction syndrome presenting with a large intraparenchymal haematoma.Neurol India 2013;61:535-537

How to cite this URL:
Ahmed M, Keshava SN, Mammen S, Chacko AG, Prabhu K. An unusual case of reversible cerebral vasoconstriction syndrome presenting with a large intraparenchymal haematoma. Neurol India [serial online] 2013 [cited 2022 Jun 29 ];61:535-537
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Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by transient reversible multifocal arterial constrictions. [1],[2] Severe headache is the characteristic presenting feature and the imaging findings include: infarcts, convexity subarachnoid hemorrhage (SAH), vasogenic edema, and sometimes intracerebral and subdural hemorrhage. [1],[2],[3],[4],[5] Incidence of intracranial hemorrhage was 34% with SAH being the most common in a large study. [3] Large parenchymal hemorrhage is uncommon and usually requires cerebral angiography to rule out a vascular malformation. Furthermore, diffuse multisegmental vasoconstriction on angiogram is often mistaken for vasculitis. [6],[7] We report a case of RCVS without any precipitating factor in a 43-year-old lady who presented acutely with a large intracerebral hematoma and in whom the digital substraction angiography (DSA) showed a reversible, multisegmental, diffuse vasoconstriction of the cerebral vessels.

A 43-year-old lady presented with severe headache and sudden onset of right side weakness. She had mild holocranial headache for 3 days prior to this event. There was no history of use of any vasoactive substance or any other medication. Vitals were normal. Neurologic examination revealed Glasgow coma scale score of 15/15 and flaccid right hemiplegia. Magnetic resonance imaging [Figure 1]a and b showed a large subacute left frontoparietal hematoma with minimal mass effect, perilesional edema, and adjacent minimal SAH. Magnetic resonance-venogram did not show any cerebral venous thrombosis. Cerebral DSA [Figure 2]a and b showed bilateral, predominantly right side multiple segments of smooth narrowing in small and medium-sized cerebral arteries in both anterior and posterior circulation beyond the circle of Willis. No other abnormalities were noted. Based on these findings, she was started on good hydration, antiepileptic, and antiedema measures with which her headache improved but there was no improvement in the hemiplegia. During the hospitalization blood pressure ranged between 160/90 and 170/100 and was controlled with oral nifedipine. Headache persisted for the initial 3 days. Repeat cerebral DSA [Figure 3]a and b 10 weeks later showed complete resolution of the previously noted multisegmental smooth narrowing in small and medium sized cerebral arteries. Right hemiparesis improved to grade 4. Based on the DSA findings and clinical presentation, the diagnosis was RCVS.{Figure 1}{Figure 2}{Figure 3}

RCVS also known as Call-Fleming syndrome, is an uncommon cerebrovascular disorder described by Call et al., in 1988. [1] It is usually seen in young and middle aged females, between 25 and 50 years of age. [1],[2],[3],[4],[5] Various precipitating factors include: Pregnancy, puerperium, physical exertion such as sexual activity, bathing and showering, high altitude, migraine, vasoactive drugs, eclampsia, and pheochromocytoma. [2],[3],[4],[5] It has also been described in association with other syndromes like posterior reversible encephalopathy and hypertensive encephalopathy. [2],[5] In many cases, no precipitating factor can be identified. [2],[3] The exact pathophysiology of this condition is not clear and thought to be related to transient-altered vascular tone. [2],[3],[4],[5] Patients often present with history of intermittent headaches for 1-2 weeks, followed by a sudden severe headache with or without neurological deficits and seizures. [2],[3],[4],[5] Associated imaging findings include convexity SAH, ischemic changes and infarcts, vasogenic edema, and occasionally intracerebral and subdural hemorrhages. [2],[3] Multisegmental vasoconstrictions with dilatation ["string and beads" appearance] involving the circle of Willis or immediate branches is usually seen on angiogram. [2],[5],[7] Centripetal pattern of involvement of arteries have been described, where involvement of peripheral small and medium sized arteries is seen initially which extends centrally to involve the large arteries of the circle of Willis. [5] Complete or substantial reversal of angiographic findings within 1-3 months is the prerequisite for the diagnosis of RCVS. [2],[3],[4],[5],[6],[7] Cerebrospinal fluid analysis and other investigations are often normal. Neurological deficits may or may not improve depending on the complications. The present case is unusual, since large parenchymal hematomas are uncommon and the angiographic vasoconstriction was smooth without ectasia unlike the usually described "stenosis and ecstatic" [string and beads] appearance.

Vasospasm is a common complication in aneurysmal SAH and usually develops after 3-4 days of ictus. It often involves long segment of terminal internal carotid artery, circle of Willis and immediate branches and seen in the region of ruptured aneurysm and SAH, whereas vasospasm in RCVS is multisegmental, often involves bilateral, small, peripheral arteries remote from the site of hemorrhage. [7],[8] Reversible cerebral vasospasm has also been reported in a case of parenchymal hematoma, where it was limited to the site of hemorrhage. [9]

RCVS is often misdiagnosed as primary angiitis of the central nervous system (PACNS). In contrast to RCVS, primary cerebral angiitis often presents with chronic history of headache, abnormal blood picture, and erythrocyte sedimentation rate and progressive or nonreversible cerebral vasoconstriction on follow-up angiogram. [6],[7] PACNS often involve small leptomeningeal arteries but rarely the arteries of the circle of Willis. [6],[7] Biopsy of the leptomeninges or cortex is required to confirm the diagnosis of PACNS. Basically, vasoconstriction in RCVS represents vasospasm unlike inflammatory changes of vessels wall in PACNS. [6],[7] Differentiation of RCVS from PACNS has clinical implication as the management and prognosis of these two conditions are very different. There is no specific treatment for RCVS though there are reports of successful management with various drugs like nimodipine, verapamil, nifedipine, magnesium sulfate, and milrinone. [2],[10] It is often managed with removal of precipitating vasoactive drug or substance if any, and symptomatic and supportive treatment. RCVS often has a very good prognosis.


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