Mædica - a Journal of Clinical Medicine C ASE
MAEDICA – a Journal of Clinical Medicine 2014; 9(2): 194-197
REPORTS
Evolution of Internal Carotid Artery Occlusion in Non-Traumatic Carotid Dissection Octavia RUSUa; Mihai VASILEa,b; Ovidiu BAJENARUa,b; Florina ANTOCHIa a
Emergency University Hospital, Bucharest, Romania Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania b
ABSTRACT Cervical artery dissection is becoming a more frequently identified cause of ischemic stroke among the young and middleaged patients. The pathogenesis of non-traumatic dissection has not been yet entirely elucidated, but certain risk factors have been reported. We present the case of a young patient with ischemic stroke in the middle cerebral artery territory secondary to internal carotid artery dissection and occlusion, in whom we identified two rarely incriminated risk factors: migraine and recent infection (pneumonia). Keywords: spontaneous cervical artery dissection, stroke, young, migraine, recent infection
INTRODUCTION
C
ervical artery dissection (CAD) is becoming a more frequently identified cause of ischemic stroke among the young and middleaged patients. The pathogenesis of non-traumatic dissection has not been yet entirely elucidated, but certain risk factors have been reported: 1-antitrypsin deficiency, hyperhomocysteinemia, connective tissue disorders, vessel abnormalities, fibromuscular dysplasia, hypertension, diabetes mellitus, hypercholesterolaemia, smoking, oral contraceptives use, migraine, recent infection and genetic factors (1). The prognosis of CAD patients depends mainly on the severity of the initial stroke, the
recurrence risk and also on the arterial repermeabilisation. Recanalisation of occluded vessels occurred in up to 90% cases, in recent studies (2). CASE REPORT
A
39-year-old female was admitted in the Neurology Clinic of the Emergency University Hospital Bucharest for abrupt onset of slurred speech accompanied by left laterocervical pain which developed a week before presentation. The patient had a history of migraines without aura, in the last several years and a upper respiratory tract infection with fever and cough three weeks ago, self-medicated with antibiotics. She denied smoking, the use of oral contraceptives and did not report any neck injury.
Address for correspondence: Florina Antochi, Emergency University Hospital, 169 Splaiul Independentei, 5th District, Bucharest, Romania. E-mail: [email protected] Article received on the 10th of February 2014. Article accepted on the 4th of May 2014.
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EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION On admission, the neurological examination showed right central facial palsy, mild right hemiparesis, Babinski reflex on the right side and non-fluent aphasia. Laboratory findings revealed leukocytosis and an inflammatory syndrome. She underwent cerebral CT–scan which demonstrated early signs of cerebral ischemia in the left middle cerebral artery territory. Doppler ultrasonography examination of the cervico-cerebral arteries exhibited the presence of a thin flapping fold of the vessel wall, distal of which a thrombus occluded the origin of the left internal carotid artery. Cervical region contrast-enhanced CT angiography illustrated gradually narrowing of the left internal carotid artery, with the absence of blood flow at the level of the second cervical vertebrae and a circumferential parietal hematoma. Cerebral angiography confirmed the occlusion of the left internal carotid artery at the origin and the presence of collateral flow through the circle of Willis. Completing the course of investigations, we stated our final diagnosis: cerebral ischemic stroke due to occlusion of the left internal carotid artery secondary to artery dissection. In evaluating the risk factors for CAD, we tested for connective tissue disorders – serum complement, anti ds-DNA, anti-U1RNP antibodies -, for hypercoagulant status – 1-antitrypsin serum level, homocysteinemia, antiphospholipid antibodies – all these had normal titers. The thoracic CT scan showed diffuse alveolar infiltrates and “ground-glass” appearance with partial confluence in the superior lobe of the left lung, suggesting pneumonia. Our patient underwent a favorable course on receiving dual antiplatelet therapy with aspirin and clopidogrel, beta-blocker, statin, antibiotic therapy for the lung infection, with obvious improvement of neurological status. At the three months follow-up visit, the ultrasonography of the cervico-cerebral arteries revealed almost complete spontaneous recanalisation of the left internal carotid artery, also confirmed by cerebral angiography, which demonstrated minimal residual stenosis.
FIGURE 1. The cerebral CT–scan, which showed a recent hypodensity in the superficial territory of the left middle cerebral artery.
DISCUSSION
C
arotid artery dissection should always be included in the differential diagnosis of
FIGURE 2. The cervical region contrast-enhanced CT angiography.
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A Journal of Clinical Medicine, Volume 9 No.2 2014
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EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION
FIGURE 3. The cerebral angiography (lateral view).
ischemic stroke, in a young patient, even with the absence of vascular risk factors or a negative history of trauma, especially when pain in the neck area is one of the symptoms (3). In addition to trivial trauma, several other potential risk factors for CAD occurring in younger people should be investigated, as stat-
ed earlier – history of smoking, use of oral contraceptives, hypertension, diabetes mellitus, connective tissue disorders, hematologic dyscrasias and genetic predisposition. Some of these factors superimpose those for atherosclerosis and ischemic stroke, but CAD is not in all cases an atherosclerotic disease (4). In the case presented, our patient had neither obvious risk factors for atherosclerosis, hypercoagulability, nor connective tissue disorders, conversely she had a history of migraines and a recent lung infection, both incriminated as potential risk factors for CAD. According to a number of studies, the underlying pathophysiological mechanism of the ischemic stroke in CAD is embolic in nature (5). This phenomenon occurs through the detachment of microemboli from the distal part of the occluding thrombus by the retrograde flow coming from collateral pathways. This is documented as carotid stump syndrome (6). We speculate that this is also the case of our patient, in whom the dissection and thrombus occurred three weeks earlier than the embolic stroke, time during which the collateral flow developed. In the acute phase of CAD, both anticoagulant and antiplatelet drugs are accepted, aiming the prevention of a primary or recurrent ischemic event. There are no evidence-based
FIGURE 4, 5. The follow-up cerebral angiography, which showed recanalisation, with irregularity of the artery wall and a good intracerebral perfusion.
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A Journal of Clinical Medicine, Volume 9 No.2 2014
EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION guidelines for the therapy, thus there is lack of a uniform approach among clinicians (7,8). In our case, we opted for dual antiplatelet therapy as secondary prevention, considering that the acute dissection had taken place earlier than the stroke, given the collateral flow that had developed through the circle of Willis, as revealed by the cerebral angiography. CAD is a potentially serious condition and may generate a poor prognosis with permanent deficits or even fatal outcomes, depending on the severity of the initial stroke and the risk of recurrence, estimated at 0.3 to 3.4% (1). The rate of complete recanalisation of occluded vessels occurs in 50% of cases at three months, and 60% at six months on evaluation by ultrasound imaging (5). In our case, at three months follow-up by cerebral angiography, the patient presented almost complete recanalization of the left internal carotid artery, with good functional outcome.
CONCLUSIONS
O
ur case report illustrates the requirement of taking into consideration the spontaneous dissection of cervical arteries as a potential cause of stroke in young people, who most often lack the risk factors of ischemic stroke. After establishing an accurate diagnosis, the potential cause of the vascular pathology should be investigated, starting with detailed history-taking and pursuing with more elaborate tests to asses blood dyscrasia or connective tissue disorders. CAD warrants further research for a better understanding of its pathophysiology and associated risk factors, consequently improving the treatment and prevention strategies. Conflict of interests: none declared. Financial support: none declared.
REFERENCES 1.
2.
3.
Rubinstein SM, Peerdeman SM, van Tulder MW, et al. – A systematic review of the risk factors for cervical artery dissection. Stroke 2005; 36:1575-80 Touzé E, Gauvrit JY, Meder JF, et al. – Prognosis of cervical artery dissection. Front NeurolNeurosci. 2005; 20:129-39 Nesteruk T, Nesteruk M, Bulik-Pasinska M, et al. – Brainstem ischemic stroke without permanent sequelae during the course of spontaneous
4.
5.
6.
internal carotid artery dissection – case report. Pol J Radiol. 2012; 77:58-62 Brandt T, Grond Ginsbach C – Spontaneous cervical artery dissection: from risk factors toward pathogenesis. Stroke 2002; 33:657-658 Nedeltchev K, Bickel S, Arnold M, et al. – Recanalization of spontaneous carotid artery dissection. Stroke 2009; 40:499-504 Waters D, Stanley WE – Cerebral emboli from the stump of a totally
Maedica
7.
8.
occluded carotid artery: surgical management. J Am Osteopath Assoc. 1992; 92:1052-5 Divjak I, Slankamenac P, Jovicevic M, et al. – Diagnosis and outcome of cervical artery dissection. Source Med Pregl. 2011; 64:392-6 Zetterling M, Carlström C, Konrad P – Internal carotid artery dissection. Acta Neurol Scand. 2000; 101:1-7.
A Journal of Clinical Medicine, Volume 9 No.2 2014
197
MAEDICA – a Journal of Clinical Medicine 2014; 9(2): 194-197
REPORTS
Evolution of Internal Carotid Artery Occlusion in Non-Traumatic Carotid Dissection Octavia RUSUa; Mihai VASILEa,b; Ovidiu BAJENARUa,b; Florina ANTOCHIa a
Emergency University Hospital, Bucharest, Romania Department of Neurology, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania b
ABSTRACT Cervical artery dissection is becoming a more frequently identified cause of ischemic stroke among the young and middleaged patients. The pathogenesis of non-traumatic dissection has not been yet entirely elucidated, but certain risk factors have been reported. We present the case of a young patient with ischemic stroke in the middle cerebral artery territory secondary to internal carotid artery dissection and occlusion, in whom we identified two rarely incriminated risk factors: migraine and recent infection (pneumonia). Keywords: spontaneous cervical artery dissection, stroke, young, migraine, recent infection
INTRODUCTION
C
ervical artery dissection (CAD) is becoming a more frequently identified cause of ischemic stroke among the young and middleaged patients. The pathogenesis of non-traumatic dissection has not been yet entirely elucidated, but certain risk factors have been reported: 1-antitrypsin deficiency, hyperhomocysteinemia, connective tissue disorders, vessel abnormalities, fibromuscular dysplasia, hypertension, diabetes mellitus, hypercholesterolaemia, smoking, oral contraceptives use, migraine, recent infection and genetic factors (1). The prognosis of CAD patients depends mainly on the severity of the initial stroke, the
recurrence risk and also on the arterial repermeabilisation. Recanalisation of occluded vessels occurred in up to 90% cases, in recent studies (2). CASE REPORT
A
39-year-old female was admitted in the Neurology Clinic of the Emergency University Hospital Bucharest for abrupt onset of slurred speech accompanied by left laterocervical pain which developed a week before presentation. The patient had a history of migraines without aura, in the last several years and a upper respiratory tract infection with fever and cough three weeks ago, self-medicated with antibiotics. She denied smoking, the use of oral contraceptives and did not report any neck injury.
Address for correspondence: Florina Antochi, Emergency University Hospital, 169 Splaiul Independentei, 5th District, Bucharest, Romania. E-mail: [email protected] Article received on the 10th of February 2014. Article accepted on the 4th of May 2014.
194
Maedica
A Journal of Clinical Medicine, Volume 9 No.2 2014
EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION On admission, the neurological examination showed right central facial palsy, mild right hemiparesis, Babinski reflex on the right side and non-fluent aphasia. Laboratory findings revealed leukocytosis and an inflammatory syndrome. She underwent cerebral CT–scan which demonstrated early signs of cerebral ischemia in the left middle cerebral artery territory. Doppler ultrasonography examination of the cervico-cerebral arteries exhibited the presence of a thin flapping fold of the vessel wall, distal of which a thrombus occluded the origin of the left internal carotid artery. Cervical region contrast-enhanced CT angiography illustrated gradually narrowing of the left internal carotid artery, with the absence of blood flow at the level of the second cervical vertebrae and a circumferential parietal hematoma. Cerebral angiography confirmed the occlusion of the left internal carotid artery at the origin and the presence of collateral flow through the circle of Willis. Completing the course of investigations, we stated our final diagnosis: cerebral ischemic stroke due to occlusion of the left internal carotid artery secondary to artery dissection. In evaluating the risk factors for CAD, we tested for connective tissue disorders – serum complement, anti ds-DNA, anti-U1RNP antibodies -, for hypercoagulant status – 1-antitrypsin serum level, homocysteinemia, antiphospholipid antibodies – all these had normal titers. The thoracic CT scan showed diffuse alveolar infiltrates and “ground-glass” appearance with partial confluence in the superior lobe of the left lung, suggesting pneumonia. Our patient underwent a favorable course on receiving dual antiplatelet therapy with aspirin and clopidogrel, beta-blocker, statin, antibiotic therapy for the lung infection, with obvious improvement of neurological status. At the three months follow-up visit, the ultrasonography of the cervico-cerebral arteries revealed almost complete spontaneous recanalisation of the left internal carotid artery, also confirmed by cerebral angiography, which demonstrated minimal residual stenosis.
FIGURE 1. The cerebral CT–scan, which showed a recent hypodensity in the superficial territory of the left middle cerebral artery.
DISCUSSION
C
arotid artery dissection should always be included in the differential diagnosis of
FIGURE 2. The cervical region contrast-enhanced CT angiography.
Maedica
A Journal of Clinical Medicine, Volume 9 No.2 2014
195
EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION
FIGURE 3. The cerebral angiography (lateral view).
ischemic stroke, in a young patient, even with the absence of vascular risk factors or a negative history of trauma, especially when pain in the neck area is one of the symptoms (3). In addition to trivial trauma, several other potential risk factors for CAD occurring in younger people should be investigated, as stat-
ed earlier – history of smoking, use of oral contraceptives, hypertension, diabetes mellitus, connective tissue disorders, hematologic dyscrasias and genetic predisposition. Some of these factors superimpose those for atherosclerosis and ischemic stroke, but CAD is not in all cases an atherosclerotic disease (4). In the case presented, our patient had neither obvious risk factors for atherosclerosis, hypercoagulability, nor connective tissue disorders, conversely she had a history of migraines and a recent lung infection, both incriminated as potential risk factors for CAD. According to a number of studies, the underlying pathophysiological mechanism of the ischemic stroke in CAD is embolic in nature (5). This phenomenon occurs through the detachment of microemboli from the distal part of the occluding thrombus by the retrograde flow coming from collateral pathways. This is documented as carotid stump syndrome (6). We speculate that this is also the case of our patient, in whom the dissection and thrombus occurred three weeks earlier than the embolic stroke, time during which the collateral flow developed. In the acute phase of CAD, both anticoagulant and antiplatelet drugs are accepted, aiming the prevention of a primary or recurrent ischemic event. There are no evidence-based
FIGURE 4, 5. The follow-up cerebral angiography, which showed recanalisation, with irregularity of the artery wall and a good intracerebral perfusion.
196
Maedica
A Journal of Clinical Medicine, Volume 9 No.2 2014
EVOLUTION OF INTERNAL CAROTID ARTERY OCCLUSION IN NON-TRAUMATIC CAROTID DISSECTION guidelines for the therapy, thus there is lack of a uniform approach among clinicians (7,8). In our case, we opted for dual antiplatelet therapy as secondary prevention, considering that the acute dissection had taken place earlier than the stroke, given the collateral flow that had developed through the circle of Willis, as revealed by the cerebral angiography. CAD is a potentially serious condition and may generate a poor prognosis with permanent deficits or even fatal outcomes, depending on the severity of the initial stroke and the risk of recurrence, estimated at 0.3 to 3.4% (1). The rate of complete recanalisation of occluded vessels occurs in 50% of cases at three months, and 60% at six months on evaluation by ultrasound imaging (5). In our case, at three months follow-up by cerebral angiography, the patient presented almost complete recanalization of the left internal carotid artery, with good functional outcome.
CONCLUSIONS
O
ur case report illustrates the requirement of taking into consideration the spontaneous dissection of cervical arteries as a potential cause of stroke in young people, who most often lack the risk factors of ischemic stroke. After establishing an accurate diagnosis, the potential cause of the vascular pathology should be investigated, starting with detailed history-taking and pursuing with more elaborate tests to asses blood dyscrasia or connective tissue disorders. CAD warrants further research for a better understanding of its pathophysiology and associated risk factors, consequently improving the treatment and prevention strategies. Conflict of interests: none declared. Financial support: none declared.
REFERENCES 1.
2.
3.
Rubinstein SM, Peerdeman SM, van Tulder MW, et al. – A systematic review of the risk factors for cervical artery dissection. Stroke 2005; 36:1575-80 Touzé E, Gauvrit JY, Meder JF, et al. – Prognosis of cervical artery dissection. Front NeurolNeurosci. 2005; 20:129-39 Nesteruk T, Nesteruk M, Bulik-Pasinska M, et al. – Brainstem ischemic stroke without permanent sequelae during the course of spontaneous
4.
5.
6.
internal carotid artery dissection – case report. Pol J Radiol. 2012; 77:58-62 Brandt T, Grond Ginsbach C – Spontaneous cervical artery dissection: from risk factors toward pathogenesis. Stroke 2002; 33:657-658 Nedeltchev K, Bickel S, Arnold M, et al. – Recanalization of spontaneous carotid artery dissection. Stroke 2009; 40:499-504 Waters D, Stanley WE – Cerebral emboli from the stump of a totally
Maedica
7.
8.
occluded carotid artery: surgical management. J Am Osteopath Assoc. 1992; 92:1052-5 Divjak I, Slankamenac P, Jovicevic M, et al. – Diagnosis and outcome of cervical artery dissection. Source Med Pregl. 2011; 64:392-6 Zetterling M, Carlström C, Konrad P – Internal carotid artery dissection. Acta Neurol Scand. 2000; 101:1-7.
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