Spontaneous Intracranial Internal Carotid Artery Dissection

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Spontaneous Intracranial Internal Carotid Artery Dissection

  1. 1. ORIGINAL CONTRIBUTIONSpontaneous IntracranialInternal Carotid Artery DissectionReport of 10 PatientsClaudia Chaves, MD; Conrado Estol, MD; Maria M. Esnaola, MD; Kenneth Gorson, MD;Margaret O’Donoghue, MD; L. Dana de Witt, MD; Louis R. Caplan, MDBackground: Spontaneous intracranial internal patient had vascular risk factors or a history of neck orcarotid artery (ICA) dissection is an uncommon cause head trauma. Stenosis of the supraclinoid portion of theof cerebral infarction, particularly when compared with ICA occurred in 8 patients, with extension to the middlethe dissection of the ICA’s cervical portion. Most cerebral artery or anterior cerebral artery in 2 patientsreports describe extensive strokes with very high mor- each. Aneurysm formation in the ipsilateral anterior ce-tality rates. rebral artery was seen in 1 patient. Two patients had a total occlusion of the supraclinoid portion of the ICA.Objective: To report the clinical and radiological find- All patients did well, with no (n=3), mild (n=4), or mod-ings of 10 patients with spontaneous intracranial ICA dis- erate (n=3) disability on the Modified Rankin Scale dur-section. ing a 3-month follow-up period.Methods: Ten patients (5 women) were included with Conclusions: Spontaneous intracranial ICA dissectionages ranging from 15 to 59 years (mean age, 28 years). can cause ischemic stroke with or without subarach- noid hemorrhage and should be considered in the dif-Results: Nine patients had a stroke (1 had an associ- ferential diagnosis of intracranial ICA stenosis or occlu-ated subarachnoid hemorrhage), whereas 1 patient had sion, especially in young patients. Some patients surviveonly transient ischemic attacks. Severe retro-orbital or with few or moderate deficits.temporal headache followed by contralateral hemipare-sis was the most common initial clinical symptom. No Arch Neurol. 2002;59:977-981 A RTERIAL DISSECTION is now RESULTS recognized as an impor- tant cause of stroke. Most Our study included 10 patients, 5 women reported dissections in- and 5 men, with ages ranging from 15 to volve the extracranial ca- 59 years (mean age, 28 years). None of the rotid and vertebral arteries. Intracranial ar- patients had known vascular risk factors terial dissections are less common, most or a history of head or neck trauma. How- reported intracranial dissections involve ever, 3 patients had a history of migraine the vertebral and basilar arteries. Verte- with aura, 2 patients were in the postpar- brobasilar dissections are known to cause tum period, and 1 patient had fibromus-From the Department ofNeurology, Lahey Clinic, brain infarction, often fatal or disabling, cular dysplasia of the extracranial ICAs thatBurlington, Mass (Dr Chaves); and subarachnoid hemorrhage. Dissect- appeared on conventional angiographyCentro Neurologico (Dr Estol) ing posterior circulation aneurysms can (Table).and Hospital Frances also cause mass effect on the brainstem and Nine patients experienced a stroke, and(Dr Esnaola), Buenos Aires, cranial nerves.1-5 Intracranial internal ca- 1 patient also had an associated subarach-Argentina; Department of rotid artery (ICA) dissections are consid- noid hemorrhage. One patient had onlyNeurology, Saint Elizabeth ered rare and have been reported infre- transient ischemic attacks: 2 within a 3-dayMedical Center, Boston, Mass quently. 6-10 The risk factors, clinical period (patient 4 in the Table). Severe head-(Drs Gorson and O’Donoghue); findings, imaging abnormalities, and prog- ache, usually retro-orbital, frontal, and/orDepartment of Neurology,Newton-Wellesley Hospital, nosis in patients with intracranial ICA dis- temporal, followed by contralateral hemi-Wellesley, Mass (Dr de Witt); sections previously have not been de- paresis were the most common initial clini-and Department of Neurology, scribed in detail. We report our experience cal symptoms (80% of the patients). Neu-Beth Israel Deaconess Medical with 10 patients who had nontraumatic in- rological signs occurred in most patientsCenter, Boston (Dr Caplan). tracranial ICA dissections. (90%) immediately after the headache on- (REPRINTED) ARCH NEUROL / VOL 59, JUNE 2002 WWW.ARCHNEUROL.COM 977 Downloaded from www.archneurol.com at Duke University, on September 4, 2007 ©2002 American Medical Association. All rights reserved.
  2. 2. PATIENTS AND METHODS ICA dissection was not included in this series because the case had been previously published.8 We reviewed the medical records as well as all of the Personal files and the New England Medical Center (Bos- imaging studies available, including cranial computed ton, Mass) stroke database were searched for patients with tomography and magnetic resonance imaging, magnetic the clinical and/or pathologic diagnosis of intracranial ICA resonance angiography of the head and neck, and conven- dissection. One patient with autopsy-proven intracranial tional cerebral angiography. Clinical, Radiological, and Outcome Data in 10 Patients With Intracranial Dissection* Patient/Sex/ Clinical Outcome Age, y Clinical Symptoms MRI MRA/Cerebral Angiography at 3 mo† 1/F/28 Right retro-orbital pain followed by fluctuating left Right IC infarct Stenosis of the supraclinoid portion of the right ICA 2 hemiparesis (3 mo post partum) and MCA 2/F/38 Right temporal and retro-orbital headache followed Right IC infarct Stenosis of the supraclinoid portion of the right ICA 2 by fluctuating left hemiparesis; history of and MCA migraine 3/F/26 Frontal headache with vomiting followed by Right caudate, right Stenosis of the supraclinoid portion of the right ICA 3 decreased level of consciousness and left MCA, and left and ACA; aneurysm formation in the right A2 hemiparesis (3 mo post partum) ACA infarcts segment; hypoplastic left A1 segment 4/F/35 Left temporal headache followed 3-5 d later by Normal Stenosis of the supraclinoid portion of the left ICA 0 2 spells of right-hand numbness; history of migraine 5/M/20 Right retro-orbital headache followed by transient Right LS infarct Occlusion of the supraclinoid portion of the right 3 left-arm clumsiness and slurred speech; 10 d ICA; fibromuscular dysplasia in the cervical later, acute onset of slurred speech, left portion of both ICAs hemiparesis, and neglect 6/F/35 Left-sided headache followed by transient paresis Left corona radiata Stenosis of the supraclinoid portion of the left ICA 2 of the right arm; 6 d later, transient episode of infarct expressive aphasia; 2 wk later, right hemiplegia and dysarthria 7/M/22 Right temporal headache followed by acute left Right caudate and Stenosis of the supraclinoid portion of the right ICA 3 hemiplegia IC infarcts with thrombosis of the right A1 segment 8/M/59 Left-sided headache followed by expressive aphasia Left frontal infarct Stenosis of the supraclinoid portion of the left ICA 0 9/M/15 Right-sided headache followed 3 d later by Right LS infarct Occlusion of the supraclinoid portion of the right ICA 1 left hemiplegia 10/M/25 Left occipital/temporal headache followed by Left LS infarct Stenosis of the supraclinoid portion of the left ICA 0 transient right-sided weakness; 2 h later, right hemiparesis and aphasia; history of migraine *MRI indicates magnetic resonance imaging; MRA, magnetic resonance angiography; IC, internal capsule; ICA, internal carotid artery; MCA, middle cerebral artery;ACA, anterior cerebral artery; and LS, lenticulostriate. †Modified Rankin Scale score.set and frequently fluctuated (50%) within the first 2 weeks Six patients were immediately treated with antico-after the initial symptoms appeared. agulants, and 3 other patients were given antiplatelet All patients underwent computed tomography and/or agents. No patient had a hemorrhagic complication. Themagnetic resonance imaging of the head within the first remaining patient with intracranial ICA dissection com-24 hours of symptom onset. Deep infarcts occurred in 7 plicated by subarachnoid hemorrhage was initially treatedpatients (Figure 1), whereas cortical infarcts involving with steroids because the presumed diagnosis was vas-the territory of the middle cerebral artery (MCA) and/or culitis. Three months later, the patient was switched toanterior cerebral artery (ACA) occurred in the other 2 antiplatelet treatment. Serial neuroimaging studies per-patients (Figure 2). The patient with transient ische- formed in 9 patients showed partial or complete recana-mic attacks had a normal result on a magnetic reso- lization in 6 of them.nance imaging scan of the head. All patients did well, with no (n=3), mild (n=4), Conventional cerebral angiography was performed or moderate (n=3) disability on the Modified Rankin Scalein all patients. Narrowing of the supraclinoid portion of during a 3-month follow-up period.the ICA was detected in 8 patients, with extension to theMCA (Figure 1) or ACA in 2 patients each. Aneurysm COMMENTformation involving the ACA was also present in 1 of thesepatients (Figure 2). Two patients had a total occlusion Spontaneous dissecting aneurysms of the intracranial ca-of the supraclinoid portion of the ICA. rotid system are uncommon in adults, especially when (REPRINTED) ARCH NEUROL / VOL 59, JUNE 2002 WWW.ARCHNEUROL.COM 978 Downloaded from www.archneurol.com at Duke University, on September 4, 2007 ©2002 American Medical Association. All rights reserved.
  3. 3. A B C DFigure 1. Patient 1: A, T2-weighted magnetic resonance imaging scan shows a right basal ganglia infarct (arrow). B, Magnetic resonance angiography of the headdemonstrates a filling defect (arrowhead) in the distal portion of the right internal carotid artery (ICA) and proximal middle cerebral artery (MCA) stem. Cerebralangiography shows the presence of double lumen in the right MCA stem (C, arrow) and a narrowing of the supraclinoid portion of the right ICA (D, arrow).Figure 2. Patient 3: Left image, Magnetic resonance imaging scan of the head shows both a right middle cerebral artery (left arrow) and left anterior cerebralartery (right arrow) stroke. Cerebral angiography demonstrates an irregular aneurysm in the right A2 segment (middle image, arrow) with proximal narrowingof the right A1 segment and supraclinoid portion of the right ICA (right image, arrow). (REPRINTED) ARCH NEUROL / VOL 59, JUNE 2002 WWW.ARCHNEUROL.COM 979 Downloaded from www.archneurol.com at Duke University, on September 4, 2007 ©2002 American Medical Association. All rights reserved.
  4. 4. compared with those of the vertebrobasilar system. Fewer Subarachnoid hemorrhage and aneurysm forma-than 100 cases have been reported in the English- tion with mass effect are common complications in pa-language literature. Our case material includes 10 pa- tients with intracranial ICA dissections but rare intients, most of them younger than 40 years. patients with dissection of the extracranial vessels.6 The Intracranial ICA dissection has been associated with presence of thinner medial and adventitial layers andfibromuscular dysplasia, cystic medial necrosis, intimal the lack of a well-developed external elastic lamina infibroelastic aberrations, and atherosclerosis.6 However, no the intracranial arteries have been implicated as themicroscopic pathologic changes are usually detected in these main factors causing subarachnoid hemorrhage in thesepatients.7-9 Only 1 of our patients was found to have changes patients.19 Most of the cases reported to date have in-suggestive of fibromuscular dysplasia in both extracranial volved the vertebrobasilar arteries,6,20-23 with a few re-ICAs during conventional angiography. None of them had ports describing subarachnoid hemorrhage in patientsevidence of hypertension, diabetes, hyperlipidemia, car- with dissection of the ACA or MCA.24-27 The reason fordiovascular disease, coagulation abnormalities, or sys- this discrepancy is not known.temic disorders or a history of head or neck trauma. Occasionally, patients with intracranial ICA dissec- The relationship between migraine and intracranial tions have been misdiagnosed as having vasculitis and areICA dissection is unclear. Sinclair11 reported a fatal case treated with steroids. This happened in 1 of our patientsin which a 27-year-old patient with a long-standing his- (patient 3). Among all types of vasculitis with central ner-tory of migraine developed a spontaneous MCA dissec- vous system involvement,28 the only one known to affecttion during a migrainous attack. The author posited that the distal portions of the ICAs is giant cell arteritis.29-31 Inlocal vascular edema during the migraine contributed to most of these patients, only the petrous and cavernous seg-the development of the intracranial artery dissection. Al- ments of the ICA are affected, with no involvement of itsthough 3 of our patients with intracranial ICA dissection supraclinoid portion.32 Giant cell arteritis generally af-had a long-standing history of migraine, all of them stated fects a much older population and is associated with anthat the headaches preceding the onset of their ischemic increased erythrocyte sedimentation rate.28symptoms were more severe than their usual migraines The treatment of patients with intracranial ICA dis-and involved predominantly the retro-orbital, frontal, section is controversial. The development of hemor-and/or temporal regions. These locations are frequent sites rhagic transformation10 and progression of the dissec-of referred pain described during stimulation of the distal tion during treatment with heparin17 have promptedportion of the ICA and MCA.12 speculation that anticoagulation may be harmful in these Similar to the cases described in the literature,13-16 patients. On the other hand, spontaneous progression inthe neurological signs in most of our patients followed patients who were not given anticoagulants has sup-almost immediately after the headache’s onset. This clini- ported the opposite argument.33,34 Six of our patientscal manifestation is different from that usually seen in received immediate anticoagulant therapy with no hem-patients with extracranial ICA dissection, in which ini- orrhagic transformation or progression of the dissec-tial symptoms may precede the stroke by several days.6 tion. We had no complications in the patients treated withAlso, whereas patients with extracranial ICA dissection antiplatelet agents. Even though our sample was too smallmay initially experience headaches, Horner syndrome, to allow any conclusions, the immediate administrationor pulsatile tinnitus without cerebral ischemia, patients of either type of treatment seems to be relatively safe.with intracranial ICA dissection almost invariably have In contrast to most of the literature,8,18,35,36 in whichbrain ischemia and cerebral infarcts. massive stroke has been the rule with a 75% mortality Fluctuation of neurological signs during the first 2 rate, all 10 of our patients with intracranial ICA dissec-weeks after symptom onset was common in our pa- tion did well, with no to moderate disability during atients, occurring in 50% of the cases. Cerebral hypoper- 3-month follow-up period. The discrepancy with priorfusion was probably the mechanism of many of these studies probably represents a bias in the population de-events, in contrast to distal embolism, which is thought scribed; cases diagnosed during autopsy have domi-to be the most important mechanism of cerebral ische- nated the literature. In more recent reports,16,37 the out-mia in patients with extracranial ICA dissection.6 come of these patients has been significantly better than The typical angiographic findings of intracranial ICA previously described, probably because of a higher in-dissection are similar to those observed with dissection dex of suspicion and improved diagnostic methods al-of its extracranial portion. String sign, double lumen, lowing antemortem diagnosis.irregular scalloped stenosis, and vessel occlusion are usu- In conclusion, we believe that intracranial ICA dis-ally seen when the dissection involves the subintimal and section can have a good prognosis and should be con-intramedial layers, and aneurysm formation typically oc- sidered in the differential diagnosis of supraclinoid ICAcurs when the subadventitial layer is affected.13,14,17 The stenosis or occlusion, especially when no other strokemost common intracranial site for aneurysm is the su- risk factors are identified.praclinoid segment of the ICA with occasional exten-sion into the MCA and/or ACA.18 All of our patients had Accepted for publication February 2, 2002.involvement of the supraclinoid portion of the ICA (8 Author contributions: Study concept and design (Drswith stenosis and 2 with occlusion), with extension to Chaves and Caplan); acquisition of data (Drs Chaves, Es-the MCA or ACA in 2 patients each. Aneurysm forma- tol, Esnaola, Gorson, O’Donoghue, de Witt, and Caplan);tion involving the ipsilateral A2 segment was present in analysis and interpretation of data (Drs Chaves and1 patient who developed a subarachnoid hemorrhage. Caplan); drafting of the manuscript (Dr Chaves); critical (REPRINTED) ARCH NEUROL / VOL 59, JUNE 2002 WWW.ARCHNEUROL.COM 980 Downloaded from www.archneurol.com at Duke University, on September 4, 2007 ©2002 American Medical Association. All rights reserved.
  5. 5. revision of the manuscript for important intellectual con- 17. Grosman H, Fomasier VL, Bonder D, et al. Dissecting aneurysm of the cerebral arteries. J Neurosurg. 1980;53:693-697.tent (Drs Chaves, Estol, Esnaola, Gorson, O’Donoghue, 18. Manz HJ, Vester J, Lavenstein B. Dissecting aneurysm of cerebral arteries in child-de Witt, and Caplan); administrative, technical, and hood and adolescence: case report and literature review of 20 cases. Virchowsmaterial support (Drs Estol, Esnaola, and Gorson); study Arch A Pathol Anat Histol. 1979;384:325-335.supervision (Drs Chaves, Estol, O’Donoghue, de Witt, and 19. Yonas H, Agamanolis D, Takaoka Y, White RJ. Dissecting intracranial aneu-Caplan). rysms. Surg Neurol. 1977;8:407-415. 20. Manz HJ, Luessenhop AJ. Dissecting aneurysm of intracranial vertebral artery: Corresponding author and reprints: Claudia Chaves, case report and review of literature. J Neurol. 1983;230:25-35.MD, Department of Neurology, Lahey Clinic, Burlington, 21. 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Surg Neurol. 1996;46:55-61. (REPRINTED) ARCH NEUROL / VOL 59, JUNE 2002 WWW.ARCHNEUROL.COM 981 Downloaded from www.archneurol.com at Duke University, on September 4, 2007 ©2002 American Medical Association. All rights reserved.

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