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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
Arch Neurol. 2002;59:977-981.
ABSTRACT
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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 associated 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 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.
INTRODUCTION
ARTERIAL 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.
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.
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 1).
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Clinical, Radiological, and Outcome Data in 10 Patients With Intracranial
Dissection*
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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 Table
1). 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 onset 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.
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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).
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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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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.
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 compared with those of the vertebrobasilar
system. Fewer than 100 cases have been reported in the English-language 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.
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.
AUTHOR INFORMATION
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
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: claudia.j.chaves{at}lahey.org).
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).
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