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ORIGINAL CONTRIBUTION
Spontaneous Intracranial
Internal Carotid Artery Dissection
Report of 10 Patients
Claudia Chaves, MD; Conrado Estol, MD; Maria M. Esnaola, MD; Kenneth Gorson, MD;
Margaret O’Donoghue, MD; L. Dana de Witt, MD; Louis R. Caplan, MD
Background: Spontaneous intracranial internal
carotid artery (ICA) dissection is an uncommon cause
of cerebral infarction, particularly when compared with
the dissection of the ICA’s cervical portion. Most
reports describe extensive strokes with very high mortality rates.
Objective: To report the clinical and radiological findings of 10 patients with spontaneous intracranial ICA dissection.
Methods: Ten patients (5 women) were included with
ages ranging from 15 to 59 years (mean age, 28 years).
Results: Nine patients had a stroke (1 had an associ-
ated subarachnoid hemorrhage), whereas 1 patient had
only transient ischemic attacks. Severe retro-orbital or
temporal headache followed by contralateral hemiparesis was the most common initial clinical symptom. No
A
From the Department of
Neurology, Lahey Clinic,
Burlington, Mass (Dr Chaves);
Centro Neurologico (Dr Estol)
and Hospital Frances
(Dr Esnaola), Buenos Aires,
Argentina; Department of
Neurology, Saint Elizabeth
Medical Center, Boston, Mass
(Drs Gorson and O’Donoghue);
Department of Neurology,
Newton-Wellesley Hospital,
Wellesley, Mass (Dr de Witt);
and Department of Neurology,
Beth Israel Deaconess Medical
Center, Boston (Dr Caplan).
patient had vascular risk factors or a history of neck or
head trauma. Stenosis of the supraclinoid portion of the
ICA occurred in 8 patients, with extension to the middle
cerebral artery or anterior cerebral artery in 2 patients
each. Aneurysm formation in the ipsilateral anterior cerebral artery was seen in 1 patient. Two patients had a
total occlusion of the supraclinoid portion of the ICA.
All patients did well, with no (n=3), mild (n=4), or moderate (n=3) disability on the Modified Rankin Scale during a 3-month follow-up period.
Conclusions: Spontaneous intracranial ICA dissection
can cause ischemic stroke with or without subarachnoid hemorrhage and should be considered in the differential diagnosis of intracranial ICA stenosis or occlusion, especially in young patients. Some patients survive
with few or moderate deficits.
Arch Neurol. 2002;59:977-981
RTERIAL DISSECTION is now
recognized as an important cause of stroke. Most
reported dissections involve the extracranial carotid and vertebral arteries. Intracranial arterial dissections are less common, most
reported intracranial dissections involve
the vertebral and basilar arteries. Vertebrobasilar dissections are known to cause
brain infarction, often fatal or disabling,
and subarachnoid hemorrhage. Dissecting posterior circulation aneurysms can
also cause mass effect on the brainstem and
cranial nerves.1-5 Intracranial internal carotid artery (ICA) dissections are considered rare and have been reported infrequently. 6-10 The risk factors, clinical
findings, imaging abnormalities, and prognosis in patients with intracranial ICA dissections previously have not been described in detail. We report our experience
with 10 patients who had nontraumatic intracranial ICA dissections.
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977
RESULTS
Our study included 10 patients, 5 women
and 5 men, with ages ranging from 15 to
59 years (mean age, 28 years). None of the
patients had known vascular risk factors
or a history of head or neck trauma. However, 3 patients had a history of migraine
with aura, 2 patients were in the postpartum period, and 1 patient had fibromuscular dysplasia of the extracranial ICAs that
appeared on conventional angiography
(Table).
Nine patients experienced a stroke, and
1 patient also had an associated subarachnoid hemorrhage. One patient had only
transient ischemic attacks: 2 within a 3-day
period (patient 4 in the Table). Severe headache, usually retro-orbital, frontal, and/or
temporal, followed by contralateral hemiparesis were the most common initial clinical symptoms (80% of the patients). Neurological signs occurred in most patients
(90%) immediately after the headache on-
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PATIENTS AND METHODS
Personal files and the New England Medical Center (Boston, Mass) stroke database were searched for patients with
the clinical and/or pathologic diagnosis of intracranial ICA
dissection. One patient with autopsy-proven intracranial
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
imaging studies available, including cranial computed
tomography and magnetic resonance imaging, magnetic
resonance angiography of the head and neck, and conventional cerebral angiography.
Clinical, Radiological, and Outcome Data in 10 Patients With Intracranial Dissection*
Patient/Sex/
Age, y
1/F/28
2/F/38
3/F/26
4/F/35
5/M/20
6/F/35
7/M/22
8/M/59
9/M/15
10/M/25
Clinical Symptoms
Right retro-orbital pain followed by fluctuating left
hemiparesis (3 mo post partum)
Right temporal and retro-orbital headache followed
by fluctuating left hemiparesis; history of
migraine
Frontal headache with vomiting followed by
decreased level of consciousness and left
hemiparesis (3 mo post partum)
Left temporal headache followed 3-5 d later by
2 spells of right-hand numbness; history of
migraine
Right retro-orbital headache followed by transient
left-arm clumsiness and slurred speech; 10 d
later, acute onset of slurred speech, left
hemiparesis, and neglect
Left-sided headache followed by transient paresis
of the right arm; 6 d later, transient episode of
expressive aphasia; 2 wk later, right hemiplegia
and dysarthria
Right temporal headache followed by acute left
hemiplegia
Left-sided headache followed by expressive aphasia
Right-sided headache followed 3 d later by
left hemiplegia
Left occipital/temporal headache followed by
transient right-sided weakness; 2 h later, right
hemiparesis and aphasia; history of migraine
MRI
MRA/Cerebral Angiography
Right IC infarct
Stenosis of the supraclinoid portion of the right ICA
and MCA
Stenosis of the supraclinoid portion of the right ICA
and MCA
Right IC infarct
Clinical Outcome
at 3 mo†
2
2
Right caudate, right Stenosis of the supraclinoid portion of the right ICA
MCA, and left
and ACA; aneurysm formation in the right A2
segment; hypoplastic left A1 segment
ACA infarcts
Normal
Stenosis of the supraclinoid portion of the left ICA
3
0
Right LS infarct
Occlusion of the supraclinoid portion of the right
ICA; fibromuscular dysplasia in the cervical
portion of both ICAs
3
Left corona radiata
infarct
Stenosis of the supraclinoid portion of the left ICA
2
Right caudate and
IC infarcts
Left frontal infarct
Right LS infarct
Stenosis of the supraclinoid portion of the right ICA
with thrombosis of the right A1 segment
Stenosis of the supraclinoid portion of the left ICA
Occlusion of the supraclinoid portion of the right ICA
3
0
1
Left LS infarct
Stenosis of the supraclinoid portion of the left ICA
0
*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
after the initial symptoms appeared.
All patients underwent computed tomography and/or
magnetic resonance imaging of the head within the first
24 hours of symptom onset. Deep infarcts occurred in 7
patients (Figure 1), whereas cortical infarcts involving
the territory of the middle cerebral artery (MCA) and/or
anterior cerebral artery (ACA) occurred in the other 2
patients (Figure 2). The patient with transient ischemic attacks had a normal result on a magnetic resonance imaging scan of the head.
Conventional cerebral angiography was performed
in all patients. Narrowing of the supraclinoid portion of
the ICA was detected in 8 patients, with extension to the
MCA (Figure 1) or ACA in 2 patients each. Aneurysm
formation involving the ACA was also present in 1 of these
patients (Figure 2). Two patients had a total occlusion
of the supraclinoid portion of the ICA.
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978
Six patients were immediately treated with anticoagulants, and 3 other patients were given antiplatelet
agents. No patient had a hemorrhagic complication. The
remaining patient with intracranial ICA dissection complicated by subarachnoid hemorrhage was initially treated
with steroids because the presumed diagnosis was vasculitis. Three months later, the patient was switched to
antiplatelet treatment. Serial neuroimaging studies performed in 9 patients showed partial or complete recanalization in 6 of them.
All patients did well, with no (n=3), mild (n=4),
or moderate (n=3) disability on the Modified Rankin Scale
during a 3-month follow-up period.
COMMENT
Spontaneous dissecting aneurysms of the intracranial carotid system are uncommon in adults, especially when
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A
B
C
D
Figure 1. Patient 1: A, T2-weighted magnetic resonance imaging scan shows a right basal ganglia infarct (arrow). B, Magnetic resonance angiography of the head
demonstrates a filling defect (arrowhead) in the distal portion of the right internal carotid artery (ICA) and proximal middle cerebral artery (MCA) stem. Cerebral
angiography 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 cerebral
artery (right arrow) stroke. Cerebral angiography demonstrates an irregular aneurysm in the right A2 segment (middle image, arrow) with proximal narrowing
of the right A1 segment and supraclinoid portion of the right ICA (right image, arrow).
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compared with those of the vertebrobasilar system. Fewer
than 100 cases have been reported in the Englishlanguage literature. Our case material includes 10 patients, most of them younger than 40 years.
Intracranial ICA dissection has been associated with
fibromuscular dysplasia, cystic medial necrosis, intimal
fibroelastic aberrations, and atherosclerosis.6 However, no
microscopic pathologic changes are usually detected in these
patients.7-9 Only 1 of our patients was found to have changes
suggestive of fibromuscular dysplasia in both extracranial
ICAs during conventional angiography. None of them had
evidence of hypertension, diabetes, hyperlipidemia, cardiovascular disease, coagulation abnormalities, or systemic disorders or a history of head or neck trauma.
The relationship between migraine and intracranial
ICA dissection is unclear. Sinclair11 reported a fatal case
in which a 27-year-old patient with a long-standing history of migraine developed a spontaneous MCA dissection during a migrainous attack. The author posited that
local vascular edema during the migraine contributed to
the development of the intracranial artery dissection. Although 3 of our patients with intracranial ICA dissection
had a long-standing history of migraine, all of them stated
that the headaches preceding the onset of their ischemic
symptoms were more severe than their usual migraines
and involved predominantly the retro-orbital, frontal,
and/or temporal regions. These locations are frequent sites
of referred pain described during stimulation of the distal
portion of the ICA and MCA.12
Similar to the cases described in the literature,13-16
the neurological signs in most of our patients followed
almost immediately after the headache’s onset. This clinical manifestation is different from that usually seen in
patients with extracranial ICA dissection, in which initial symptoms may precede the stroke by several days.6
Also, whereas patients with extracranial ICA dissection
may initially experience headaches, Horner syndrome,
or pulsatile tinnitus without cerebral ischemia, patients
with intracranial ICA dissection almost invariably have
brain ischemia and cerebral infarcts.
Fluctuation of neurological signs during the first 2
weeks after symptom onset was common in our patients, occurring in 50% of the cases. Cerebral hypoperfusion was probably the mechanism of many of these
events, in contrast to distal embolism, which is thought
to be the most important mechanism of cerebral ischemia in patients with extracranial ICA dissection.6
The typical angiographic findings of intracranial ICA
dissection are similar to those observed with dissection
of its extracranial portion. String sign, double lumen,
irregular scalloped stenosis, and vessel occlusion are usually seen when the dissection involves the subintimal and
intramedial layers, and aneurysm formation typically occurs when the subadventitial layer is affected.13,14,17 The
most common intracranial site for aneurysm is the supraclinoid segment of the ICA with occasional extension into the MCA and/or ACA.18 All of our patients had
involvement of the supraclinoid portion of the ICA (8
with stenosis and 2 with occlusion), with extension to
the MCA or ACA in 2 patients each. Aneurysm formation involving the ipsilateral A2 segment was present in
1 patient who developed a subarachnoid hemorrhage.
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980
Subarachnoid hemorrhage and aneurysm formation with mass effect are common complications in patients with intracranial ICA dissections but rare in
patients with dissection of the extracranial vessels.6 The
presence of thinner medial and adventitial layers and
the lack of a well-developed external elastic lamina in
the intracranial arteries have been implicated as the
main factors causing subarachnoid hemorrhage in these
patients.19 Most of the cases reported to date have involved the vertebrobasilar arteries,6,20-23 with a few reports describing subarachnoid hemorrhage in patients
with dissection of the ACA or MCA.24-27 The reason for
this discrepancy is not known.
Occasionally, patients with intracranial ICA dissections have been misdiagnosed as having vasculitis and are
treated with steroids. This happened in 1 of our patients
(patient 3). Among all types of vasculitis with central nervous system involvement,28 the only one known to affect
the distal portions of the ICAs is giant cell arteritis.29-31 In
most of these patients, only the petrous and cavernous segments of the ICA are affected, with no involvement of its
supraclinoid portion.32 Giant cell arteritis generally affects a much older population and is associated with an
increased erythrocyte sedimentation rate.28
The treatment of patients with intracranial ICA dissection is controversial. The development of hemorrhagic transformation10 and progression of the dissection during treatment with heparin17 have prompted
speculation that anticoagulation may be harmful in these
patients. On the other hand, spontaneous progression in
patients who were not given anticoagulants has supported the opposite argument.33,34 Six of our patients
received immediate anticoagulant therapy with no hemorrhagic transformation or progression of the dissection. We had no complications in the patients treated with
antiplatelet agents. Even though our sample was too small
to allow any conclusions, the immediate administration
of either type of treatment seems to be relatively safe.
In contrast to most of the literature,8,18,35,36 in which
massive stroke has been the rule with a 75% mortality
rate, all 10 of our patients with intracranial ICA dissection did well, with no to moderate disability during a
3-month follow-up period. The discrepancy with prior
studies probably represents a bias in the population described; cases diagnosed during autopsy have dominated the literature. In more recent reports,16,37 the outcome of these patients has been significantly better than
previously described, probably because of a higher index of suspicion and improved diagnostic methods allowing antemortem diagnosis.
In conclusion, we believe that intracranial ICA dissection can have a good prognosis and should be considered in the differential diagnosis of supraclinoid ICA
stenosis or occlusion, especially when no other stroke
risk factors are identified.
Accepted for publication February 2, 2002.
Author contributions: Study concept and design (Drs
Chaves and Caplan); acquisition of data (Drs Chaves, Estol, Esnaola, Gorson, O’Donoghue, de Witt, and Caplan);
analysis and interpretation of data (Drs Chaves and
Caplan); drafting of the manuscript (Dr Chaves); critical
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revision of the manuscript for important intellectual content (Drs Chaves, Estol, Esnaola, Gorson, O’Donoghue,
de Witt, and Caplan); administrative, technical, and
material support (Drs Estol, Esnaola, and Gorson); study
supervision (Drs Chaves, Estol, O’Donoghue, de Witt, and
Caplan).
Corresponding author and reprints: Claudia Chaves,
MD, Department of Neurology, Lahey Clinic, Burlington,
MA 01805 (e-mail: [email protected]).
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