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Spontaneous bilateral carotid artery dissection in a woman presenting with isolated headache
American Journal of Emergency Medicine (2009) 27, 754.e1–754.e3
www.elsevier.com/locate/ajem
Case Report Spontaneous bilateral carotid artery dissection in a woman presenting with isolated headache
Abstract Carotid artery dissection is a major cause of ischemic stroke in young adults, usually preceded by trivial to significant trauma. When focal neurologic deficit or classic traumatic history is absent, the diagnosis can be elusive. We describe a case of bilateral carotid artery dissection in a 36year-old woman with no antecedent cervical trauma or manipulation. Because the patient presented without neurologic deficit, diagnosis was not made on the patient's first presentation to the emergency department. On her second visit, the patient underwent an exhaustive workup leading to diagnosis. Carotid artery dissection should be considered in the differential diagnosis of any adult with new severe headache, even in the absence of neurologic deficit. A 36-year-old white female presented to the emergency department with a chief complaint of headache and blurred vision. She stated that the headache was gradual in onset, had been intermittently worsening, and was frontal in location. The patient was concerned when on the night prior her emergency department visit, she had blurred vision, which she described as difficulty visualizing peripheral objects. This resolved but then recurred before arrival in the emergency department, which prompted her to seek care. On arrival, the patient stated the visual disturbance had again subsided. On examination, the patient was nontoxic appearing with normal vital signs. Examination of the head and neck and neurologic examination were unremarkable. The patient was treated with intravenous prochlorperazine and ketorolac, with improvement in her headache from a 9/10 to 4/10, before discharge. The patient returned to the emergency department 3 days later with continued waxing and waning bifrontal headache and occasional nausea. She reported no overall change in symptoms. On examination, the patient had anisocoria, with her right pupil slightly larger than the left, but both were reactive. On reassessment 2 hours into her emergency department stay, her anisocoria had resolved. The patient was otherwise neurologically intact. Because the patient had no improvement and because she had no personal history of 0735-6757/$ – see front matter © 2009 Elsevier Inc. All rights reserved.
migraine headache, a noncontrasted computed tomography (CT) of the patient's head was done to look for intracranial hemorrhage or mass. This was negative. A lumbar puncture was also performed, which was negative for any pathologic condition. She did not improve despite therapy with dihydroergotamine, prochlorperazine, and narcotics, so magnetic resonance imaging was performed to look for possible venous sinus thrombosis. On this study, the patient's carotid arteries were only partially visualized but were felt to be abnormal, so the patient went on to CT angiography of her head and neck. This study revealed bilateral internal carotid artery (ICA) dissections with markedly decreased carotid blood flow (Fig. 1). The patient was requestioned regarding the possibility of trivial trauma, and she continued to deny any inciting event. The patient was subsequently admitted to the hospital and received systemic anticoagulation. She underwent testing for factor V Leiden and prothrombin gene mutation, which were unremarkable. She was tested for autoimmune disease as well as disorders involving protein C and protein S, which also were negative. The patient also underwent testing for systemic inflammatory conditions including Lyme and syphilis that were negative, as well. The patient did well in the hospital and was discharged on hospital day 7 with no neurologic deficits and resolution of her headache. Dissection of the carotid artery is a major cause of cerebral infarction in young adults age 30 to 50, occurring in 2.6 per 100 000 each year [1-3]. It is one of the most frequent causes of ischemic stroke unrelated to atherosclerosis in the young and middle-aged, causing 10% of strokes in patients younger than 50 and 20% of strokes in patients younger than 30 [4]. Males and females experience carotid artery dissection at similar rates [1,3]. Extracranial or common carotid artery dissections are rare, usually attributed to major trauma [5], and nearly all of common carotid dissections are a result of aortic dissection [6]. Internal carotid artery dissection can occur spontaneously or after trauma. Cases of ICA dissection have been cited after both severe and trivial trauma to the head or neck [7,8]. Risk factors for traumatic ICA dissection include neck manipulation or strain, blunt or penetrating trauma, or major systemic trauma such as that encountered in a motor vehicle crash. Spontaneous ICA dissection has been linked to hypertension, oral contraceptives, hypercholesterolemia, pregnancy, and smoking. Connective tissue disorders, osteogenesis imperfecta, and α-1 antitrypsin deficiency have also been
754.e2
Fig. 1 CT scan of the head demonstrating severe bilateral narrowing of the carotid arteries.
implicated in increasing the risk of ICA dissection, especially when multiple vessels are involved [9]. Spontaneous bilateral ICA dissection is quite rare, being described in only a few case reports [4,10-12]. Almost all patients with ICA dissection present with headache or neck pain [3,8]. The associated headache is most often described as sudden in onset and unlike previous headaches, throbbing, constant, and severe [13], and is most often localized to the frontal area or around the eye [9]. Head or neck pain is often the initial symptom and the most common, but it is rare for pain to be the only symptom of a dissecting ICA [8,13,14], and most patients will develop localized neurologic symptoms or symptoms of transient ischemic attacks or ischemic stroke [8,14]. Other common presenting symptoms include cranial nerve palsy or Horner's syndrome, or localized neurologic symptoms [3]. Hypoageusia, amaurosis fugax, or pulsatile tinnitus have also been identified as symptoms of ICA dissection [8,12]. Morbidity of ICA dissection can vary from transient neurologic symptoms to more significant cerebral or retinal ischemic injury. The clinical presentation of bilateral dissection usually includes ischemic symptoms [4,10,12]. Because these patients are generally younger and therefore unlikely candidates for a stroke, this initiates a full workup resulting in the diagnosis of bilateral carotid artery dissection. Generally, once ICA dissection is diagnosed, anticoagulation is initiated using heparin and then warfarin to prevent embolism. However, medically treating with anticoagulation therapy has not been proven to be more successful than close observation, as the vessel has been found to recanalize on its
Case Report own [9,15]. Most clinicians still treat with anticoagulation therapy that can be discontinued usually once the vessel is greater than 50% patent. This occurs most often within a few months to a year. Furthermore, surgery or catheter revascularization has not decreased stroke rate in patients with ICA dissection [16]. Various approaches have been used to treat ICA dissection including surgery, medical anticoagulation therapy, or simply close observation with none showing clear benefit. To diagnose ICA dissection, clinicians need to have a high index of suspicion. Ischemic or neurologic symptoms in young patients should cause cervical artery dissection to be included on the differential diagnosis, as up to 20% of transient ischemic attack or ischemic stroke in patients younger than 45 can be attributed to dissecting ICA [17]. However, without obvious symptoms, such as in this patient, the diagnosis may be overlooked, leading to delay to diagnosis and increased morbidity. Currently there is no literature describing spontaneous bilateral carotid artery dissection in which headache was the only symptom. This case is unusual because of the limited, nonspecific symptoms associated with the disease. Therefore, ICA dissection should be considered in patients describing persistent headache unlike previous headaches that is unresponsive to typical migraine treatment protocols. In a patient with no history of migraine, such as this patient, further evaluation is warranted for a new severe headache. Neck ultrasound, CT angiogram, or head and neck CT can be used to help diagnose ICA dissection and are becoming widely available modalities. Noncontrasted head CT is unlikely to be helpful in these patients. Therefore, if head CT is going to be obtained for evaluation of a new headache in a young patient, consideration should be given to imaging of the neck with contrast to identify pathologic condition of the carotid arteries. Alexandra Chomut Albany Medical College Albany, NY Rebecca Jeanmonod MD Donald Jeanmonod MD St. Luke's Hospital and Health Network Bethlehem, PA 18015 Robert Ward Elmira College Elmira, NY doi:10.1016/j.ajem.2008.10.004
References [1] Marshman L, Ball L, Jadun C. Spontaneous bilateral carotid and vertebral artery dissections associated with multiple disparate intracranial aneurysms, subarachnoid hemorrhage and spontaneous resolution: case report and literature review. Clin Neurol Neurosurg 2007;109:816-20.
Case Report [2] Schievink W, Mokri B, Whisnant JP. Internal carotid artery dissection in a community: Rochester, Minnesota, 1987-1992. Stroke 1993;24: 1678-80. [3] Zetterling M, Carlström C, Konrad P. Internal carotid artery dissection. Acta Neurol Scand 2000;101:1-7. [4] Wu H, Chen Y, Chen C, et al. Spontaneous bilateral internal carotid artery dissection with acute stroke in young patients. Eur Neurol 2006; 56:230-4. [5] Dittrich R, Draeger B, Nassenstein I, et al. Dissection of the common and external carotid artery. Cerebrovasc Dis 2006;21: 208-10. [6] Chen Y, Lee T, Chen C, et al. Spontaneous common carotid artery dissection: a case report and review of the literature. Eur Neurol 2003; 50:58-60. [7] Hart R, Easton J. Dissections of cervical and cerebral arteries. Neurol Clin 1983;1:155-82. [8] Schievink W. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 2001;344:897-8. [9] Ropper A, Brown R. Cerebrovascular disease. Adams and Victor's principles of neurology. 8th ed. New York: The McGraw-Hill Companies Inc.; 2005.
754.e3 [10] Lloyd J. Bilateral dissecting aneurysms of the internal carotid arteries. Am J Surg 1971;122:549-51. [11] Sellier N, Chiras J, Benhamou M, et al. Spontaneous dissection of the internal carotid artery. Clinical radiologic and evolutive aspects. Apropos of 46 cases. J Neuroradiol 1983;10:243-59. [12] Townend B, Traves L, Crimmins D. Bilateral spontaneous carotid artery dissection. J Clin Neurosci 2005;12:592-4. [13] Arnold M, Cumurciuc R, Stapf C, et al. Pain as the only symptom of cervical artery dissection. J Neurol Neurosurg Psychiatry 2006;77: 1021-4. [14] Biousse V, D'Anglejan-Chatillon J, Touboul P, et al. Time course of symptoms in extracranial carotid artery dissections: a series of 80 patients. Stroke 1995;26:235-9. [15] Markwalder T, Starrett R, Mumenthaler M. Spontaneous bilateral recanalization in bilateral internal carotid artery occlusion. Stroke 1980;11:95-8. [16] Kremer C, Mosso M, Georgiadis D, et al. Carotid dissection with permanent and transient occlusion or severe stenosis: long-term outcome. Neurology 2003;60:271-5. [17] Leys D, Lucas C, Gobert M, et al. Cervical artery dissections. Eur Neurol 1997;37:3-12.
www.elsevier.com/locate/ajem
Case Report Spontaneous bilateral carotid artery dissection in a woman presenting with isolated headache
Abstract Carotid artery dissection is a major cause of ischemic stroke in young adults, usually preceded by trivial to significant trauma. When focal neurologic deficit or classic traumatic history is absent, the diagnosis can be elusive. We describe a case of bilateral carotid artery dissection in a 36year-old woman with no antecedent cervical trauma or manipulation. Because the patient presented without neurologic deficit, diagnosis was not made on the patient's first presentation to the emergency department. On her second visit, the patient underwent an exhaustive workup leading to diagnosis. Carotid artery dissection should be considered in the differential diagnosis of any adult with new severe headache, even in the absence of neurologic deficit. A 36-year-old white female presented to the emergency department with a chief complaint of headache and blurred vision. She stated that the headache was gradual in onset, had been intermittently worsening, and was frontal in location. The patient was concerned when on the night prior her emergency department visit, she had blurred vision, which she described as difficulty visualizing peripheral objects. This resolved but then recurred before arrival in the emergency department, which prompted her to seek care. On arrival, the patient stated the visual disturbance had again subsided. On examination, the patient was nontoxic appearing with normal vital signs. Examination of the head and neck and neurologic examination were unremarkable. The patient was treated with intravenous prochlorperazine and ketorolac, with improvement in her headache from a 9/10 to 4/10, before discharge. The patient returned to the emergency department 3 days later with continued waxing and waning bifrontal headache and occasional nausea. She reported no overall change in symptoms. On examination, the patient had anisocoria, with her right pupil slightly larger than the left, but both were reactive. On reassessment 2 hours into her emergency department stay, her anisocoria had resolved. The patient was otherwise neurologically intact. Because the patient had no improvement and because she had no personal history of 0735-6757/$ – see front matter © 2009 Elsevier Inc. All rights reserved.
migraine headache, a noncontrasted computed tomography (CT) of the patient's head was done to look for intracranial hemorrhage or mass. This was negative. A lumbar puncture was also performed, which was negative for any pathologic condition. She did not improve despite therapy with dihydroergotamine, prochlorperazine, and narcotics, so magnetic resonance imaging was performed to look for possible venous sinus thrombosis. On this study, the patient's carotid arteries were only partially visualized but were felt to be abnormal, so the patient went on to CT angiography of her head and neck. This study revealed bilateral internal carotid artery (ICA) dissections with markedly decreased carotid blood flow (Fig. 1). The patient was requestioned regarding the possibility of trivial trauma, and she continued to deny any inciting event. The patient was subsequently admitted to the hospital and received systemic anticoagulation. She underwent testing for factor V Leiden and prothrombin gene mutation, which were unremarkable. She was tested for autoimmune disease as well as disorders involving protein C and protein S, which also were negative. The patient also underwent testing for systemic inflammatory conditions including Lyme and syphilis that were negative, as well. The patient did well in the hospital and was discharged on hospital day 7 with no neurologic deficits and resolution of her headache. Dissection of the carotid artery is a major cause of cerebral infarction in young adults age 30 to 50, occurring in 2.6 per 100 000 each year [1-3]. It is one of the most frequent causes of ischemic stroke unrelated to atherosclerosis in the young and middle-aged, causing 10% of strokes in patients younger than 50 and 20% of strokes in patients younger than 30 [4]. Males and females experience carotid artery dissection at similar rates [1,3]. Extracranial or common carotid artery dissections are rare, usually attributed to major trauma [5], and nearly all of common carotid dissections are a result of aortic dissection [6]. Internal carotid artery dissection can occur spontaneously or after trauma. Cases of ICA dissection have been cited after both severe and trivial trauma to the head or neck [7,8]. Risk factors for traumatic ICA dissection include neck manipulation or strain, blunt or penetrating trauma, or major systemic trauma such as that encountered in a motor vehicle crash. Spontaneous ICA dissection has been linked to hypertension, oral contraceptives, hypercholesterolemia, pregnancy, and smoking. Connective tissue disorders, osteogenesis imperfecta, and α-1 antitrypsin deficiency have also been
754.e2
Fig. 1 CT scan of the head demonstrating severe bilateral narrowing of the carotid arteries.
implicated in increasing the risk of ICA dissection, especially when multiple vessels are involved [9]. Spontaneous bilateral ICA dissection is quite rare, being described in only a few case reports [4,10-12]. Almost all patients with ICA dissection present with headache or neck pain [3,8]. The associated headache is most often described as sudden in onset and unlike previous headaches, throbbing, constant, and severe [13], and is most often localized to the frontal area or around the eye [9]. Head or neck pain is often the initial symptom and the most common, but it is rare for pain to be the only symptom of a dissecting ICA [8,13,14], and most patients will develop localized neurologic symptoms or symptoms of transient ischemic attacks or ischemic stroke [8,14]. Other common presenting symptoms include cranial nerve palsy or Horner's syndrome, or localized neurologic symptoms [3]. Hypoageusia, amaurosis fugax, or pulsatile tinnitus have also been identified as symptoms of ICA dissection [8,12]. Morbidity of ICA dissection can vary from transient neurologic symptoms to more significant cerebral or retinal ischemic injury. The clinical presentation of bilateral dissection usually includes ischemic symptoms [4,10,12]. Because these patients are generally younger and therefore unlikely candidates for a stroke, this initiates a full workup resulting in the diagnosis of bilateral carotid artery dissection. Generally, once ICA dissection is diagnosed, anticoagulation is initiated using heparin and then warfarin to prevent embolism. However, medically treating with anticoagulation therapy has not been proven to be more successful than close observation, as the vessel has been found to recanalize on its
Case Report own [9,15]. Most clinicians still treat with anticoagulation therapy that can be discontinued usually once the vessel is greater than 50% patent. This occurs most often within a few months to a year. Furthermore, surgery or catheter revascularization has not decreased stroke rate in patients with ICA dissection [16]. Various approaches have been used to treat ICA dissection including surgery, medical anticoagulation therapy, or simply close observation with none showing clear benefit. To diagnose ICA dissection, clinicians need to have a high index of suspicion. Ischemic or neurologic symptoms in young patients should cause cervical artery dissection to be included on the differential diagnosis, as up to 20% of transient ischemic attack or ischemic stroke in patients younger than 45 can be attributed to dissecting ICA [17]. However, without obvious symptoms, such as in this patient, the diagnosis may be overlooked, leading to delay to diagnosis and increased morbidity. Currently there is no literature describing spontaneous bilateral carotid artery dissection in which headache was the only symptom. This case is unusual because of the limited, nonspecific symptoms associated with the disease. Therefore, ICA dissection should be considered in patients describing persistent headache unlike previous headaches that is unresponsive to typical migraine treatment protocols. In a patient with no history of migraine, such as this patient, further evaluation is warranted for a new severe headache. Neck ultrasound, CT angiogram, or head and neck CT can be used to help diagnose ICA dissection and are becoming widely available modalities. Noncontrasted head CT is unlikely to be helpful in these patients. Therefore, if head CT is going to be obtained for evaluation of a new headache in a young patient, consideration should be given to imaging of the neck with contrast to identify pathologic condition of the carotid arteries. Alexandra Chomut Albany Medical College Albany, NY Rebecca Jeanmonod MD Donald Jeanmonod MD St. Luke's Hospital and Health Network Bethlehem, PA 18015 Robert Ward Elmira College Elmira, NY doi:10.1016/j.ajem.2008.10.004
References [1] Marshman L, Ball L, Jadun C. Spontaneous bilateral carotid and vertebral artery dissections associated with multiple disparate intracranial aneurysms, subarachnoid hemorrhage and spontaneous resolution: case report and literature review. Clin Neurol Neurosurg 2007;109:816-20.
Case Report [2] Schievink W, Mokri B, Whisnant JP. Internal carotid artery dissection in a community: Rochester, Minnesota, 1987-1992. Stroke 1993;24: 1678-80. [3] Zetterling M, Carlström C, Konrad P. Internal carotid artery dissection. Acta Neurol Scand 2000;101:1-7. [4] Wu H, Chen Y, Chen C, et al. Spontaneous bilateral internal carotid artery dissection with acute stroke in young patients. Eur Neurol 2006; 56:230-4. [5] Dittrich R, Draeger B, Nassenstein I, et al. Dissection of the common and external carotid artery. Cerebrovasc Dis 2006;21: 208-10. [6] Chen Y, Lee T, Chen C, et al. Spontaneous common carotid artery dissection: a case report and review of the literature. Eur Neurol 2003; 50:58-60. [7] Hart R, Easton J. Dissections of cervical and cerebral arteries. Neurol Clin 1983;1:155-82. [8] Schievink W. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 2001;344:897-8. [9] Ropper A, Brown R. Cerebrovascular disease. Adams and Victor's principles of neurology. 8th ed. New York: The McGraw-Hill Companies Inc.; 2005.
754.e3 [10] Lloyd J. Bilateral dissecting aneurysms of the internal carotid arteries. Am J Surg 1971;122:549-51. [11] Sellier N, Chiras J, Benhamou M, et al. Spontaneous dissection of the internal carotid artery. Clinical radiologic and evolutive aspects. Apropos of 46 cases. J Neuroradiol 1983;10:243-59. [12] Townend B, Traves L, Crimmins D. Bilateral spontaneous carotid artery dissection. J Clin Neurosci 2005;12:592-4. [13] Arnold M, Cumurciuc R, Stapf C, et al. Pain as the only symptom of cervical artery dissection. J Neurol Neurosurg Psychiatry 2006;77: 1021-4. [14] Biousse V, D'Anglejan-Chatillon J, Touboul P, et al. Time course of symptoms in extracranial carotid artery dissections: a series of 80 patients. Stroke 1995;26:235-9. [15] Markwalder T, Starrett R, Mumenthaler M. Spontaneous bilateral recanalization in bilateral internal carotid artery occlusion. Stroke 1980;11:95-8. [16] Kremer C, Mosso M, Georgiadis D, et al. Carotid dissection with permanent and transient occlusion or severe stenosis: long-term outcome. Neurology 2003;60:271-5. [17] Leys D, Lucas C, Gobert M, et al. Cervical artery dissections. Eur Neurol 1997;37:3-12.