Cerebral and Cervical Artery Dissection - Clinical Features and Diagnosis - UpToDate

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Cerebral and cervical artery dissection: Clinical features

and diagnosis
AUTHOR: David S Liebeskind, MD
SECTION EDITOR: Scott E Kasner, MD
DEPUTY EDITOR: John F Dashe, MD, PhD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Dec 2023.

This topic last updated: Sep 15, 2023.
INTRODUCTION
Arterial dissections are a common cause of stroke in the young but may occur at any age.
Dissection occurs when structural integrity of the arterial wall is compromised, allowing blood
to collect between layers as an intramural hematoma. Dissections that occur without overt
trauma are often labeled as "spontaneous" even though there is often a triggering event or
underlying predisposition contributing to the pathogenesis.
This topic will review the pathophysiology, etiology, clinical features, and diagnosis of cerebral
and cervical artery dissection. The treatment and prognosis of cervicocephalic dissection is
reviewed in detail separately. (See "Cerebral and cervical artery dissection: Treatment and
prognosis".)
Other mechanisms of ischemic stroke and subarachnoid hemorrhage are discussed elsewhere.
(See "Ischemic stroke in children and young adults: Epidemiology, etiology, and risk factors" and
"Stroke: Etiology, classification, and epidemiology" and "Aneurysmal subarachnoid hemorrhage:
Clinical manifestations and diagnosis" and "Nonaneurysmal subarachnoid hemorrhage".)
ANATOMY AND PATHOLOGY
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Separation of the arterial wall layers results in dissection. A false lumen arises in the space
where blood seeps into the vessel wall ( figure 1). Hemorrhage may be due to an intimal tear
or result from rupture or other pathology in the vasa vasorum [1,2]. Subintimal dissections
cause luminal stenosis or occlusion, whereas subadventitial dissections largely result in
dissecting aneurysm formation ( figure 2). False lumen extension back into the true lumen
can form a double channel for blood flow in the artery.
[3,4]
Extracranial carotid dissections typically occur 2 cm or more beyond the carotid bifurcation,
near or adjacent to the level of the skull base [3]. Intracranial carotid dissections are most
frequent in the supraclinoid segment [4]. Vertebral artery dissection most often occurs in the
cervical transverse processes of C6 to C2 (V2 segment) or the extracranial segment between the
transverse process of C2 and the foramen magnum at the base of the skull (V3 segment)
( figure 3) [5].
Multiple simultaneous cervicocephalic dissections are found in 13 to 22 percent of cases [6-9]

and may occur more often in females than males [7,8,10]. Three or more dissections occur in
approximately 2 percent of cases [10]. Although evidence is limited to observations in small
numbers of patients, multiple simultaneous dissections are not typically associated with a
known underlying predisposition to dissection [10]. Rather, most cases may be due to a
transient vasculopathy following minor trauma or infection. Ischemia may manifest in only one
of the downstream arterial territories, if at all.
Histopathologic specimens of dissected arteries are rarely obtained; when done, examination
may reveal intramural hemorrhage, disruption of the subintimal plane, and less frequently
separation of the media and adventitia ( picture 1) [11]. In a case-control study, superficial
temporal artery specimens obtained by biopsy or autopsy from 14 patients with cervical artery
dissection showed pathologic changes involving mainly the adventitial and medial layers, with
vacuolar degeneration and fissuring, along with capillary neoangiogenesis and microscopic
erythrocyte extravasation into connective tissue [2]. In contrast, only one of nine control arteries
obtained from accident victims showed pathologic changes in the outer arterial walls.
There is a high prevalence of ultrastructural connective tissue abnormalities in patients with

apparently sporadic cervicocephalic artery dissection [12-14]. These abnormalities consist
primarily of composite fibrils within collagen bundles and fragmented elastic fibers.
PATHOPHYSIOLOGY

The development of intramural hematoma with subintimal dissections causes luminal stenosis
or occlusion. This may result in cerebral ischemia due to thromboembolism, hypoperfusion, or a
combination of both. Thromboembolism rather than hypoperfusion is considered the major
cause of ischemic symptoms [15,16]. (See 'Ischemic stroke or TIA' below.)
Subadventitial dissections with aneurysm or hematoma formation and vessel dilatation

( figure 2) may cause local symptoms from compression of adjacent nerves and their feeding
vessels, resulting in pain, partial oculosympathetic paresis (Horner syndrome), lower cranial
neuropathies, or cervical nerve root involvement. (See 'Local symptoms' below.)
In a minority of cases, dissection of intracranial arteries, which lack an external elastic lamina
and have only a thin adventitial layer, can lead to vessel rupture with subarachnoid
hemorrhage. (See 'Subarachnoid hemorrhage' below.)
Head, neck, or facial pain is thought to be caused by activation of nociceptors from distension of
the vessel wall due to the intimal tear and/or intramural hematoma formation [17,18].
ETIOLOGY
Arterial dissections are generally caused by various degrees of trauma or triggering events, with
or without an underlying predisposition. While major head and neck trauma can cause
dissection, most dissections occur after a mechanical trigger, which may not always be
remembered [19]. Dissections that occur without overt trauma are often labeled as
"spontaneous" even though there is often a triggering event or underlying predisposition
contributing to the pathogenesis.
Minor trauma and other triggers — Observational data suggest that trauma, typically mild or
trivial in nature, or other mechanical events are triggers for cervical artery dissection in up to 40
percent of cases [20]. The list of physical activities associated with dissection is long and
includes the following:
● Basketball [21]
● Childbirth [22]
● Cervical manipulation therapy [23-28]
● Coughing or sneezing [29]
● Dancing [30,31]
● Minor sports injuries [32,33]
● Roller coaster or amusement park rides [34-39]
● Scuba diving [40,41]
● Sexual intercourse [42]

● Skating [43]
● Swimming [44]
● Tennis [45,46]
● Trampoline use [47]
● Vigorous exercise [29]
● Volleyball [48]
● Weightlifting [49]
● Yoga [29]
While cervical manipulation therapy may trigger dissection, causality is difficult to establish, and
the absolute incidence of dissection caused by spinal manipulation is unknown [28,50-52].
Associated conditions and risk factors — Connective tissue disorders, vascular abnormalities,
and other conditions have been associated with dissection.
● Connective tissue or vascular disorders – The proportion of patients with cervical artery
dissection who are affected by a known connective tissue or vascular disorder is low [53-
56]. Nevertheless, various connective tissue and vascular disorders have been associated
with dissection, including the following [13,57,58]:
• Fibromuscular dysplasia [59-63]

• Ehlers-Danlos syndrome type IV (vascular Ehlers-Danlos)
• Marfan syndrome
• Osteogenesis imperfecta [64]
• Cystic medial necrosis [65]
• Reticular fiber deficiency [66]
• Homocystinuria [67]
• Autosomal dominant polycystic kidney disease [68]
• Alpha-1 antitrypsin deficiency [69]
• Segmental mediolytic arteriopathy [70]
• Reversible cerebral vasoconstriction syndromes [71]
• Aortic root diameter >34 mm [72]
• Carotid artery redundancy [73]
• Cervical artery tortuosity [74]
• Cerebral aneurysms [75]
The most common association is with fibromuscular dysplasia, a nonspecific arteriopathy,

which accounts for 15 to 40 percent of cases of cervicocephalic dissection in some reports

[61,63,76]. Ehlers-Danlos syndrome type IV is found in <2 percent of all cases of cervical or
cerebral artery dissection [53,54]. The prevalence of dissection among all patients with
Ehlers-Danlos is similarly infrequent. In one cohort of over 400 patients with Ehlers Danlos
type IV, carotid artery dissection was observed in 2 percent [77].
For the remaining connective tissue and vascular disorders listed above, all of which are
rare diseases, it remains uncertain whether the association with dissection is greater than
would be expected by chance alone [58]. As an example, a series of 1934 patients with
cervical artery dissection identified only 6 (<1 percent) with an inherited connective tissue
disease [55]. There were two patients with genetically confirmed vascular Ehlers-Danlos
and one patient each (diagnosed by clinical criteria) with Marfan syndrome, classic Ehlers-
Danlos, hypermobile Ehlers-Danlos, and osteogenesis imperfecta. Although Marfan
syndrome is a known cause of aortic dissection, only a few cases of isolated
cervicocephalic dissection have been reported in patients with Marfan syndrome [78,79],
and several large series of patients with Marfan syndrome have reported no occurrences
of cervicocephalic dissection [80,81].
In contrast to the rare association of connective tissue disease with cervicocephalic artery
dissection, one case-control study involving 84 patients with cervical artery dissection
compared with 84 matched controls who had ischemic stroke without dissection found
that the individuals with cervical artery dissection were significantly more likely to have
clinical signs suggestive of connective tissue abnormalities [56]. These signs involved
mainly skeletal (eg, scoliosis, pectus excavatum), skin (eg, mild skin hyperextension), and
ocular abnormalities as well as craniofacial dysmorphisms. However, none of the patients
with cervical artery dissection had an established hereditary connective tissue disease.
● Other conditions – A number of other conditions have been associated with

cervicocephalic dissection, including:
• Recent infection [58,72,82-84]

• Hypertension [85,86]
• Migraine [72,87-92]
• Elevated homocysteine levels [58,72,93]
• Oral contraceptive use [63,94]
• Smoking [87]
• Higher body height and lower body weight [95]
• Elongated styloid process compressing the cervical internal carotid artery (sometimes
referred to as the stylocarotid artery syndrome or the vascular variant of the Eagle
syndrome) [96,97]
• Pregnancy, mainly in the postpartum period [98]
Genetics — There are no established genetic markers for cervicocephalic dissection. Studies
using whole exome sequencing of candidate genes associated with arterial dissection or
aneurysm have identified probable pathogenic variants in genes associated with connective
tissue disorders, including Marfan syndrome, vascular Ehlers-Danlos syndrome, classic Ehlers
Danlos syndrome, digenic Alport syndrome, and hereditary angiopathy with nephropathy,
aneurysms, and muscle cramps (HANAC) syndrome [99,100]. In addition, a genome-wide
association study suggested that the rs9349379[G] allele of the PHACTR1 gene was associated
with a lower risk of cervical artery dissection [101]. However, further confirmation in larger
studies is needed to confirm these findings.
In addition to the monogenic connective tissue disorders, polygenetic factors may be involved
in the etiology and pathophysiology of dissection as part a multifactorial predisposition [53]. In
theory, such factors could cause an inherited weakness of the vessel wall, thereby increasing
susceptibility to dissection from minor trauma, inflammation, thrombosis, or other
environmental triggers.
Despite the paucity of evidence, there are valid reasons to suspect that genetic factors are
related to the pathophysiology of cervicocephalic dissection. As an example, patients with a
family history of arterial dissections involving cervicocephalic arteries, renal arteries or the aorta
appear to be at increased risk for recurrent arterial dissection [102,103]. Furthermore, patients
with apparently sporadic cervicocephalic artery dissection have a high prevalence of
ultrastructural connective tissue abnormalities (see 'Anatomy and pathology' above) that are
not associated with any defined collagen vascular disease. In some families, these connective
tissue alterations appear to be transmitted in an autosomal dominant pattern [104,105].
EPIDEMIOLOGY
Dissection is a common cause of stroke, particularly in young adults (see "Ischemic stroke in
children and young adults: Epidemiology, etiology, and risk factors", section on 'Extracranial
dissection'). In studies from North America and Europe, the mean age of individuals affected by
dissection was 44 to 53 years [6-8,106]. While the prevalence of dissection as a cause of
ischemic stroke is higher in younger adults, evidence from national registry data in the United
States indicates that the prevalence of hospitalization for dissection-related stroke is evident
across the lifespan [106].

A population-based study from Minnesota reported that the average annual incidence rate for
internal carotid artery dissection was 1.72 per 100,000 individuals, while that for vertebral artery
dissection was 0.97 per 100,000; the combined annual incidence of dissection was 2.6 per
100,000 [6]. The true incidence is probably even higher, since many cases of dissection may
escape detection because they have minor or no clinical signs [58]. There is no clear sex or
ethnic predilection [6-8,58,107,108]. Dissection may be more common in winter, although the
cause of seasonal variation remains unclear [109].
Extracranial dissection is far more frequent than intracranial dissection in reports from North
America and Europe. However, evidence from several case series suggests that intracranial
dissection is more common in Asian populations and in children [110,111].
CLINICAL MANIFESTATIONS
Evidence from population- and hospital-based reports suggests that dissection most often
results in ischemic stroke or transient ischemic attack, usually associated with local symptoms
such as neck pain or headache [6-8,112]. However, these studies may underestimate the
proportion of cases that are asymptomatic or associated with local symptoms only [58]. For
patients without ischemia at the time of diagnosis, retrospective data suggest that the risk of
ischemic stroke is highest in the first two weeks after the diagnosis of cervical artery dissection
[113].
Local symptoms — Local symptoms caused by cervical or cerebral artery dissection can include
pain, Horner syndrome, cranial and cervical neuropathies, and pulsatile tinnitus.
Head and neck pain — The most frequent initial symptom of cervicocephalic dissection is
head and/or neck pain, found in 57 to 90 percent of cases [6,112,114-117]. The pain is often
severe, continuous, and of recent onset; in a study of 49 patients with cervical artery dissection,
patients with head or neck pain presented within one to five days after the onset of pain [117].
Neck pain may be more frequent with vertebral artery dissection compared with carotid artery
dissection [112].
The headache is typically ipsilateral to the dissection and often localizes to the temple, eye,
cheek, or teeth with carotid artery dissection, and to the occipital region with vertebral artery
dissection [118]. Headaches from dissection may also mimic migraine or cluster headache [119],
or have a sudden and severe ("thunderclap") onset [120]. (See "Overview of thunderclap
headache".)
Isolated orbital or monocular pain is a rare presentation of carotid artery dissection [121,122]
Horner syndrome — Horner syndrome ( figure 4) occurs in approximately 25 percent of

cases [6] and is due most often to distension of sympathetic fibers spanning the external
surface of the internal carotid artery [6,123,124]. The Horner syndrome seen with internal
carotid artery dissection is usually partial, involving ptosis and miosis but not anhidrosis. This
occurs because the sympathetic fibers responsible for facial sweating and vasodilation branch
off at the superior cervical ganglion from the remainder of the oculosympathetic pathway and
travel with the external carotid artery. (See "Horner syndrome", section on 'Third-order
syndrome'.)
Cranial or cervical neuropathies — Carotid dissection may cause single or multiple

compressive cranial neuropathies in up to 12 percent of cases [7,125,126]. Cranial nerve XII is
most commonly affected ( figure 5), followed by IX; involvement of cranial nerves III, V, and VI
is less common [125-128]. Cervical nerve root involvement is a rare complication of vertebral
artery dissection [129-132].
Cranial or cervical neuropathies due to arterial dissection may occur without associated
ischemia.
Pulsatile tinnitus — Pulsatile tinnitus can occur in isolation or in combination with other
manifestations of cervical artery dissection [133-135]. In the Cervical Artery Dissection and
Ischemic Stroke Patients (CADISP) study of 778 patients with cervical artery dissection, pulsatile
tinnitus was present in 8 percent [135].
Ischemic stroke or TIA — Ischemic symptoms are a common manifestation of cerebral and
cervical artery dissection. In a population-based report of 48 patients with dissection of the
internal carotid and vertebral arteries, cerebral ischemia was noted in 67 percent, with transient
ischemic attack (TIA) and cerebral infarction in 23 and 56 percent, respectively [6].
Neurologic symptoms and signs of ischemic stroke or TIA due to carotid or vertebral artery
dissection are not specific to dissection but are related to the different vascular territories
involved.
● Carotid artery dissection may cause anterior circulation stroke syndrome, transient
monocular blindness, retinal artery occlusion, or ischemic optic neuropathy
[7,112,136,137].
● Vertebral artery dissection may lead to lateral medullary infarction (Wallenberg syndrome),
other posterior circulation stroke syndromes, or cervical spinal cord ischemia [129]. In a
2012 systematic review of 75 studies that described over 1900 patients with vertebral

artery dissection, dizziness/vertigo was the most common symptom, although it is not
certain that ischemia was the cause [138].
Subarachnoid hemorrhage — Intracranial artery dissection may result in subarachnoid

hemorrhage. In a 2015 systematic review of retrospective case series, subarachnoid
hemorrhage was associated with intracranial artery dissection in 8 to 69 percent of cases [111].
In rare cases, subarachnoid hemorrhage is found in combination with ischemic stroke [5].
EVALUATION AND DIAGNOSIS
When to suspect dissection — The diagnosis of cervical or intracranial artery dissection should
be suspected in patients presenting with acute or subacute headache, neck pain, and/or stroke
symptoms, particularly in following settings [139]:
● History of recent potential triggering events:

• Trauma, even if minor or trivial
• Participation in sports or physical activities
• Intense sneezing or coughing
● Peripartum Valsalva
● Personal or family history of connective tissue or vascular disorders or migraine
● Acute or subacute symptoms:

• Neurologic symptoms suggestive of ischemic stroke involving the anterior or posterior
circulation stroke or suggestive of subarachnoid hemorrhage
• Horner syndrome
• Cranial or cervical neuropathies
• Pulsatile tinnitus
• Tooth pain without a dental cause
Headache or neck pain at or prior to stroke onset may suggest underlying dissection, especially
as a cause of stroke in the young. The acute onset of Horner syndrome in association with neck
pain and an ischemic stroke or transient ischemic attack (TIA) in the territory of the ipsilateral
internal carotid artery is suggestive of carotid artery dissection [58]. However, patients age ≥60
years with cervical artery dissection may be less likely to present with neck pain, headache,
preceding trauma, or a mechanical triggering event [140]. Therefore, the possibility of
dissection should not be disregarded when these features are absent in older individuals with
unexplained TIA or acute ischemic stroke.

Confirming the diagnosis — We obtain urgent noninvasive multimodal imaging with brain and
neck magnetic resonance imaging (MRI) and head and neck magnetic resonance angiography
(MRA), or head computed tomography (CT) and computed tomography angiography (CTA) of
the head and neck to confirm an initial diagnosis of cervicocephalic dissection and to guide
serial treatment decisions. While clinical features may raise suspicion for dissection, the
diagnosis is confirmed by neuroimaging findings, particularly the demonstration of a long
tapered arterial stenosis, a tapered occlusion, a dissecting aneurysm (pseudoaneurysm), an
intimal flap, a double lumen, or an intramural hematoma. (See 'Choice of neuroimaging study'
below.)
Choice of neuroimaging study — MRI should be ordered with standard axial T1-weighted, T2-
weighted, fluid attenuation inversion recovery (FLAIR), and diffusion-weighted sequences.
Cervical and cranial T1 fat-saturation imaging is useful for identifying small intramural
hemorrhages [141]. MRA of the head and neck should be obtained with contrast-enhanced and
time-of-flight MRA. Alternatively, a noncontrast head CT with CTA (which requires injection of
contrast media) of the head and neck can be ordered. Axial source images and three-
dimensional reconstructions are useful for detecting dissection, intimal tears, and medial or
subendothelial hemorrhage [141]. The choice between these modalities is based primarily on
availability and experience at local hospitals. There is no "gold standard," and the diagnosis
often requires complementary imaging modalities, which may need repeating over time.
These imaging studies should be performed urgently as part of a comprehensive stroke

evaluation for patients who present with a clinical diagnosis of acute ischemic or hemorrhagic
stroke, particularly for patients with ischemic stroke who may be candidates for reperfusion
therapy using intravenous thrombolysis or mechanical thrombectomy. In addition, we obtain
multimodal MRI/MRA or CT/CTA if dissection is suspected on the basis of local symptoms in the
absence of ischemic stroke or subarachnoid hemorrhage.
We reserve the use of conventional angiography for younger patients when clinical suspicion
for dissection remains high despite negative noninvasive imaging. There is no need for
conventional angiography if the diagnosis of cerebral or cervical artery dissection is clear using
CTA or MRA. In most centers, conventional angiography has been supplanted by noninvasive
approaches, particularly brain MRI with MRA and cranial CT with CTA [142-144]. A systematic
review published in 2009 found that the sensitivity and specificity of MR techniques and CTA for
the diagnosis of cervicocephalic arterial dissection were relatively similar [142].
Carotid duplex and transcranial Doppler ultrasonography (TCD) may be used to screen for
suspected dissection, or to monitor therapy [145-148]. However, carotid duplex detects
abnormalities in only 68 to 95 percent of cases [145,149]. Duplex and transcranial Doppler have
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a suboptimal yield for identifying arterial dissection near the skull base and vertebral artery
dissection within the transverse foramina [58,144]. In addition, ultrasound is unreliable for
detecting carotid artery dissection in patients with an isolated Horner syndrome [150].
Therefore, confirmation with MRA or CTA should be pursued in ultrasound-negative cases when
the clinical history is suggestive of dissection [151].
Characteristic imaging findings — Angiographic findings of dissection include:
● String sign ( image 1)

● Tapered stenosis or occlusion or flame-shaped occlusion ( image 2)
● Intimal flap ( image 3)
● Dissecting aneurysm ( image 4)
● Intramural hematoma – crescent sign ( image 5)
● Distal pouch
In a population-based study of 48 consecutive patients with cervical artery dissection, the

diagnostic neuroimaging patterns were an elongated tapered stenosis, a tapered occlusion, and
a dissecting aneurysm in 48, 35, and 17 percent, respectively [6]. In a prospective European
study of patients with vertebral artery dissection, the most frequent diagnostic neuroimaging
finding on MRI was intramural hematoma, which was observed in 91 percent of 157 vertebral
artery dissections [5].
The pathognomonic crescent sign of intramural hematoma is formed by an eccentric rim of

hyperintensity surrounding a hypointense arterial lumen on MRI ( image 5). This crescent
sign has traditionally been described on T1-weighted fat-saturation MRI sequences but may be
apparent on other sequences such as diffusion-weighted imaging [152] or CTA. The degree of
MRI hyperintensity and the methemoglobin content of the intramural hematoma varies with
age of the lesion. Dissections of the horizontal vertebral artery segment may be difficult to
diagnose as the classic crescent may be missing due to orientation of the vessel and the
vertebral venous plexus may also appear hyperintense. The orientation of the vertebral artery
may also limit delineation of a crescent, as the lumen may be patent yet surrounded by a more
irregular "suboccipital rind" sign [153]. Assessment of multimodal CT or MRI source images is
crucial to define vessel wall abnormalities.
The pattern of brain ischemia on diffusion-weighted MRI may be influenced by the patency of
the dissected artery, with territorial rather than borderzone infarcts apparent when there is
complete occlusion of the vessel [154]. The advent of high-resolution 3 Tesla MRI has made it
possible to detail interval recanalization, degree of stenosis, formation of dissecting aneurysms
and the appearance of new dissections as part of serial imaging evaluations [155]. Periarterial

inflammation associated with dissection may also be visualized with such high-resolution MRI
techniques [156].
Evaluation for connective tissue disorders — As noted previously (see 'Associated conditions
and risk factors' above), the proportion of patients with dissection who are affected by a known
connective tissue or vascular disorder is low. Therefore, we generally do not pursue additional
testing for such disorders unless there is heightened clinical suspicion because of suggestive
symptoms, signs or family history (eg, joint hypermobility, multiple joint dislocations,
translucent skin, poor wound healing, easy bruising, and unusual scars consistent with Ehlers-
Danlos syndrome). (See "Clinical manifestations and diagnosis of Ehlers-Danlos syndromes".)
The diagnosis of fibromuscular dysplasia (FMD) is made from angiography, so typically no

additional testing is needed if extracranial arteries show no signs of FMD on MRA or CTA. An
exception might be patients with clinical manifestations suggestive of renal FMD, where
evaluation of renal arteries could prove diagnostic. (See "Clinical manifestations and diagnosis
of fibromuscular dysplasia".)
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of cervicocephalic dissection is broad, given that the manifestations
may include local symptoms (primarily head and neck pain, Horner syndrome, lower cranial
nerve palsy), brain ischemia, or subarachnoid hemorrhage, either in isolation or in combination.
(See 'Clinical manifestations' above.)
● Head and neck pain – Entities to be considered in the differential diagnosis of head and
neck pain include various types of headache, particularly those with unilateral head pain
and those accompanied by autonomic symptoms such as ptosis and miosis. The list
includes migraine, cluster headache and other trigeminal autonomic cephalalgias (eg,
paroxysmal hemicrania and short-lasting unilateral neuralgiform headache with
conjunctival injection and tearing [SUNCT] syndrome), and Raeder paratrigeminal
neuralgia [115]. Migraine should be suspected when there is a characteristic march of
transient neurological deficits, although this pattern has been reported as well in rare
patients with internal carotid artery dissection [157]. Cluster headache typically occurs
without focal deficits. (See "Pathophysiology, clinical manifestations, and diagnosis of
migraine in adults" and "Pathophysiology, clinical features, and diagnosis of migraine in
children" and "Cluster headache: Epidemiology, clinical features, and diagnosis" and
"Paroxysmal hemicrania: Clinical features and diagnosis" and "Overview of craniofacial
pain", section on 'Paratrigeminal oculosympathetic syndrome'.)

● Thunderclap headache – Thunderclap headache, a severe headache of sudden onset,

occurs in a minority of patients with cervicocephalic dissection. In addition, thunderclap
headache is characteristic of the pain associated with the onset of subarachnoid
hemorrhage and can be associated with multiple other causes as listed in the table
( table 1). (See "Overview of thunderclap headache".)
● Ischemic stroke and TIA – The differential diagnosis for the cause of brain ischemia
includes cardiogenic embolism, large artery atherosclerosis, small vessel disease, and a
host of less common mechanisms such as a vasculopathy other than dissection.
Intracranial vertebral artery occlusive disease due to atherosclerosis is a more common
cause of lateral medullary ischemia than is vertebral dissection. (See "Differential diagnosis
of transient ischemic attack and acute stroke" and "Stroke: Etiology, classification, and
epidemiology" and "Ischemic stroke in children and young adults: Epidemiology, etiology,
and risk factors" and "Posterior circulation cerebrovascular syndromes", section on 'Lateral
medullary infarction'.)
● Subarachnoid hemorrhage – The differential diagnosis of subarachnoid hemorrhage

includes saccular aneurysm rupture, bleeding from a vascular malformation,
perimesencephalic nonaneurysmal subarachnoid hemorrhage, and a number of less
common causes. Angiography is the key to determining whether subarachnoid
hemorrhage is caused by intracranial dissection or another vascular abnormality. (See
"Aneurysmal subarachnoid hemorrhage: Clinical manifestations and diagnosis" and
"Nonaneurysmal subarachnoid hemorrhage" and "Perimesencephalic nonaneurysmal
subarachnoid hemorrhage".)
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Stroke in adults" and
"Society guideline links: Stroke in children" and "Society guideline links: Fibromuscular
dysplasia".)
SUMMARY AND RECOMMENDATIONS
● Location – Extracranial carotid dissections typically occur 2 cm or more beyond the carotid
bifurcation, near or adjacent to the level of the skull base. Intracranial carotid dissections
are most frequent in the supraclinoid segment. Vertebral artery dissection most often

occurs in the cervical transverse processes of C6 to C2 (V2 segment) or the extracranial

segment between the transverse process of C2 and the foramen magnum at the base of
the skull (V3 segment) ( figure 3). (See 'Anatomy and pathology' above.)
● Pathophysiology – Separation of the arterial wall layers results in dissection. A false

lumen arises in the space where blood seeps into the vessel wall ( figure 1). Hemorrhage
may be due to an intimal tear or result from rupture or other pathology in the vasa
vasorum. Subintimal dissections cause luminal stenosis or occlusion whereas
subadventitial dissections largely result in dissecting aneurysm formation ( figure 2).
(See 'Pathophysiology' above.)
● Etiology – Dissection may result from a combination of intrinsic deficiencies of vessel wall
integrity and extrinsic factors, including minor trauma. Numerous proposed risk factors
and inciting activities have been associated with dissection. (See 'Etiology' above.)
● Epidemiology – Dissection of the cervical and cerebral arteries occurs in about 3 cases per
100,000 individuals across all ages yet accounts for up to one quarter of all strokes in the
young. (See 'Epidemiology' above.)
● Clinical manifestations – Evidence from population and hospital-based reports suggests

that dissection most often results in ischemic stroke or transient ischemic attack, usually
preceded or accompanied by local symptoms such as neck pain, headache, Horner
syndrome ( figure 4), and/or cranial neuropathies ( figure 5). However, these studies
may underestimate the proportion of cases that are asymptomatic or associated with local
symptoms only. Uncommonly, intracranial dissection results in subarachnoid hemorrhage.
(See 'Clinical manifestations' above.)
● Evaluation and diagnosis – We obtain urgent noninvasive multimodal imaging with

magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the
head and neck, or computed tomography (CT) and computed tomography angiography
(CTA) of the head and neck to confirm an initial diagnosis of cervicocephalic dissection and
to guide serial treatment decisions. While clinical features may raise suspicion for
dissection, the diagnosis is confirmed by neuroimaging findings, particularly the
demonstration of a long tapered arterial stenosis, a tapered occlusion, a dissecting
aneurysm (pseudoaneurysm), an intimal flap, a double lumen, or an intramural
hematoma. (See 'Evaluation and diagnosis' above.)
● Treatment and prognosis – The treatment and prognosis of cervicocephalic dissection is

reviewed in detail separately. (See "Cerebral and cervical artery dissection: Treatment and
prognosis".)
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REFERENCES
1. Pannone A, Lucchetti G, Stazi G, et al. Internal carotid artery dissection in a patient with
Behçet's syndrome. Ann Vasc Surg 1998; 12:463.
2. Völker W, Dittrich R, Grewe S, et al. The outer arterial wall layers are primarily affected in
spontaneous cervical artery dissection. Neurology 2011; 76:1463.
3. Downer J, Nadarajah M, Briggs E, et al. The location of origin of spontaneous extracranial

internal carotid artery dissection is adjacent to the skull base. J Med Imaging Radiat Oncol
2014; 58:408.
4. Chaves C, Estol C, Esnaola MM, et al. Spontaneous intracranial internal carotid artery
dissection: report of 10 patients. Arch Neurol 2002; 59:977.
5. Arnold M, Bousser MG, Fahrni G, et al. Vertebral artery dissection: presenting findings and
predictors of outcome. Stroke 2006; 37:2499.
6. Lee VH, Brown RD Jr, Mandrekar JN, Mokri B. Incidence and outcome of cervical artery
dissection: a population-based study. Neurology 2006; 67:1809.
7. Arnold M, Kappeler L, Georgiadis D, et al. Gender differences in spontaneous cervical artery

dissection. Neurology 2006; 67:1050.
8. Touzé E, Gauvrit JY, Moulin T, et al. Risk of stroke and recurrent dissection after a cervical
artery dissection: a multicenter study. Neurology 2003; 61:1347.
9. Hassan AE, Zacharatos H, Mohammad YM, et al. Comparison of single versus multiple
spontaneous extra- and/or intracranial arterial dissection. J Stroke Cerebrovasc Dis 2013;
22:42.
10. Arnold M, De Marchis GM, Stapf C, et al. Triple and quadruple spontaneous cervical artery
dissection: presenting characteristics and long-term outcome. J Neurol Neurosurg
Psychiatry 2009; 80:171.
11. Besselmann M, Vennemann B, Lowens S, et al. Internal carotid artery dissection. Neurology
2000; 54:442.
12. Ulbricht D, Diederich NJ, Hermanns-Lê T, et al. Cervical artery dissection: An atypical
presentation with Ehlers-Danlos-like collagen pathology? Neurology 2004; 63:1708.
13. Brandt T, Morcher M, Hausser I. Association of cervical artery dissection with connective
tissue abnormalities in skin and arteries. Front Neurol Neurosci 2005; 20:16.

14. Brandt T, Orberk E, Weber R, et al. Pathogenesis of cervical artery dissections: association
with connective tissue abnormalities. Neurology 2001; 57:24.
15. Lucas C, Moulin T, Deplanque D, et al. Stroke patterns of internal carotid artery dissection in
40 patients. Stroke 1998; 29:2646.
16. Morel A, Naggara O, Touzé E, et al. Mechanism of ischemic infarct in spontaneous cervical
artery dissection. Stroke 2012; 43:1354.
17. Arnold M, Cumurciuc R, Stapf C, et al. Pain as the only symptom of cervical artery
dissection. J Neurol Neurosurg Psychiatry 2006; 77:1021.
18. Mehdi E, Aralasmak A, Toprak H, et al. Craniocervical Dissections: Radiologic Findings,
Pitfalls, Mimicking Diseases: A Pictorial Review. Curr Med Imaging Rev 2018; 14:207.
19. Norris JW, Beletsky V, Nadareishvili ZG. Sudden neck movement and cervical artery
dissection. The Canadian Stroke Consortium. CMAJ 2000; 163:38.
20. Engelter ST, Grond-Ginsbach C, Metso TM, et al. Cervical artery dissection: trauma and
other potential mechanical trigger events. Neurology 2013; 80:1950.
21. De Giorgio F, Vetrugno G, De Mercurio D, et al. 1. Dissection of the vertebral artery during a
basketball game: a case report. Med Sci Law 2004; 44:80.
22. Stamboulis E, Raptis G, Andrikopoulou A, et al. Spontaneous internal carotid artery
dissection: an uncommon cause of recurrent postpartum headache. J Neuroimaging 2011;
21:76.
23. Paciaroni M, Bogousslavsky J. Cerebrovascular complications of neck manipulation. Eur
Neurol 2009; 61:112.
24. Reuter U, Hämling M, Kavuk I, et al. Vertebral artery dissections after chiropractic neck
manipulation in Germany over three years. J Neurol 2006; 253:724.
25. Leon-Sanchez A, Cuetter A, Ferrer G. Cervical spine manipulation: an alternative medical

procedure with potentially fatal complications. South Med J 2007; 100:201.
26. Smith WS, Johnston SC, Skalabrin EJ, et al. Spinal manipulative therapy is an independent
risk factor for vertebral artery dissection. Neurology 2003; 60:1424.
27. Ernst E. Adverse effects of spinal manipulation: a systematic review. J R Soc Med 2007;
100:330.
28. Biller J, Sacco RL, Albuquerque FC, et al. Cervical arterial dissections and association with
cervical manipulative therapy: a statement for healthcare professionals from the american
heart association/american stroke association. Stroke 2014; 45:3155.
29. Caso V, Paciaroni M, Bogousslavsky J. Environmental factors and cervical artery dissection.

Front Neurol Neurosci 2005; 20:44.

30. Faivre A, Abdelfettah Z, Rodriguez S, Nicoli F. Neurological picture. Bilateral internal carotid
artery dissection due to elongated styloid processes and shaking dancing. J Neurol
Neurosurg Psychiatry 2009; 80:1154.
31. Prabhakar S, Bhatia R, Khandelwal N, et al. Vertebral artery dissection due to indirect neck
trauma : an underrecognised entity. Neurol India 2001; 49:384.
32. Faivre A, Chapon F, Combaz X, Nicoli F. Internal carotid artery dissection occurring during
intensive practice with Wii video sports games. Neurology 2009; 73:1242.
33. Dharmasaroja P, Dharmasaroja P. Sports-related internal carotid artery dissection:
pathogenesis and therapeutic point of view. Neurologist 2008; 14:307.
34. Burneo JG, Shatz R, Papamitsakis NI, Mitsias PD. Neuroimages: amusement park stroke.
Neurology 2000; 55:564.
35. Sheikh SI, Singhal AB. Extension of "roller coaster dissection" after tissue plasminogen
activator. AJNR Am J Neuroradiol 2010; 31:E4.
36. Schneck M, Simionescu M, Bijari A. Bilateral vertebral artery dissection possibly precipitated
in delayed fashion as a result of roller coaster rides. J Stroke Cerebrovasc Dis 2008; 17:39.
37. Blacker DJ, Wijdicks EF. A ripping roller coaster ride. Neurology 2003; 61:1255.
38. Lascelles K, Hewes D, Ganesan V. An unexpected consequence of a roller coaster ride. J

Neurol Neurosurg Psychiatry 2001; 71:704.
39. Biousse V, Chabriat H, Amarenco P, Bousser MG. Roller-coaster-induced vertebral artery
dissection. Lancet 1995; 346:767.
40. Bartsch T, Palaschewski M, Thilo B, et al. Internal carotid artery dissection and stroke after
SCUBA diving: a case report and review of the literature. J Neurol 2009; 256:1916.
41. Walters GK. Cervicocranial artery dissection and scuba diving: Is there a link or is it
serendipity? Undersea Hyperb Med 2018; 45:65.
42. Delasobera BE, Osborn SR, Davis JE. Thunderclap headache with orgasm: a case of basilar
artery dissection associated with sexual intercourse. J Emerg Med 2012; 43:e43.
43. Karnik R, Rothmund T, Bonner G, et al. Inline skating as a possible cause of consecutive
bilateral vertebral artery dissection. Acta Neurol Scand 2000; 101:70.
44. Furtner M, Werner P, Felber S, Schmidauer C. Bilateral carotid artery dissection caused by
springboard diving. Clin J Sport Med 2006; 16:76.
45. Abe A, Nishiyama Y, Kamiyama H, et al. Symptomatic middle cerebral artery dissection in a
young tennis player. J Nippon Med Sch 2009; 76:209.

46. DeBehnke DJ, Brady W. Vertebral artery dissection due to minor neck trauma. J Emerg Med
1994; 12:27.
47. Wechsler B, Kim H, Hunter J. Trampolines, children, and strokes. Am J Phys Med Rehabil
2001; 80:608.
48. Slankamenac P, Jesic A, Avramov P, et al. Multiple cervical artery dissection in a volleyball
player. Arch Neurol 2010; 67:1024.
49. Low A, Dovey J, Ash-Miles J. Vertebral artery dissection in weightlifter with performance
enhancing drug use. BMJ Case Rep 2011; 2011.
50. Cassidy JD, Boyle E, Côté P, et al. Risk of Carotid Stroke after Chiropractic Care: A Population-
Based Case-Crossover Study. J Stroke Cerebrovasc Dis 2017; 26:842.
51. Paulus JK, Thaler DE. Does case misclassification threaten the validity of studies
investigating the relationship between neck manipulation and vertebral artery dissection
stroke? Yes. Chiropr Man Therap 2016; 24:42.
52. Chung CL, Côté P, Stern P, L'Espérance G. The Association Between Cervical Spine
Manipulation and Carotid Artery Dissection: A Systematic Review of the Literature. J
Manipulative Physiol Ther 2015; 38:672.
53. Debette S, Markus HS. The genetics of cervical artery dissection: a systematic review. Stroke
2009; 40:e459.
54. Grond-Ginsbach C, Debette S. The association of connective tissue disorders with cervical
artery dissections. Curr Mol Med 2009; 9:210.
55. Debette S, Goeggel Simonetti B, Schilling S, et al. Familial occurrence and heritable
connective tissue disorders in cervical artery dissection. Neurology 2014; 83:2023.
56. Giossi A, Ritelli M, Costa P, et al. Connective tissue anomalies in patients with spontaneous
cervical artery dissection. Neurology 2014; 83:2032.
57. Gdynia HJ, Kühnlein P, Ludolph AC, Huber R. Connective tissue disorders in dissections of
the carotid or vertebral arteries. J Clin Neurosci 2008; 15:489.
58. Debette S, Leys D. Cervical-artery dissections: predisposing factors, diagnosis, and

outcome. Lancet Neurol 2009; 8:668.
59. de Bray JM, Marc G, Pautot V, et al. Fibromuscular dysplasia may herald symptomatic
recurrence of cervical artery dissection. Cerebrovasc Dis 2007; 23:448.
60. Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia:
results in the first 447 patients. Circulation 2012; 125:3182.
61. Olin JW, Gornik HL, Bacharach JM, et al. Fibromuscular dysplasia: state of the science and
critical unanswered questions: a scientific statement from the American Heart Association.
Circulation 2014; 129:1048.

62. Kadian-Dodov D, Gornik HL, Gu X, et al. Dissection and Aneurysm in Patients
With Fibromuscular Dysplasia: Findings From the U.S. Registry for FMD. J Am Coll Cardiol
2016; 68:176.
63. Talarowska P, Dobrowolski P, Klisiewicz A, et al. High incidence and clinical characteristics of
fibromuscular dysplasia in patients with spontaneous cervical artery dissection: The
ARCADIA-POL study. Vasc Med 2019; 24:112.
64. Rouvière S, Michelini R, Sarda P, Pagès M. Spontaneous carotid artery dissection in two
siblings with osteogenesis imperfecta. Cerebrovasc Dis 2004; 17:270.
65. Thal DR, Schober R, Schlote W. Carotid artery dissection in a young adult: cystic medial
necrosis associated with an increased elastase content. Clin Neuropathol 1997; 16:180.
66. Hegedüs K. Reticular fiber deficiency in the intracranial arteries of patients with dissecting
aneurysm and review of the possible pathogenesis of previously reported cases. Eur Arch
Psychiatry Neurol Sci 1985; 235:102.
67. Weiss N, Demeret S, Sonneville R, et al. Bilateral internal carotid artery dissection in
cystathionine beta-synthase deficiency. Eur Neurol 2006; 55:177.
68. Larranaga J, Rutecki GW, Whittier FC. Spontaneous vertebral artery dissection as a
complication of autosomal dominant polycystic kidney disease. Am J Kidney Dis 1995; 25:70.
69. Vila N, Millán M, Ferrer X, et al. Levels of alpha1-antitrypsin in plasma and risk of
spontaneous cervical artery dissections: a case-control study. Stroke 2003; 34:E168.
70. Fantaneanu T, Veinot JP, Torres C, et al. Cervical arterial dissections due to segmental
mediolytic arteriopathy. Neurology 2011; 77:295.
71. Mawet J, Boukobza M, Franc J, et al. Reversible cerebral vasoconstriction syndrome and
cervical artery dissection in 20 patients. Neurology 2013; 81:821.
72. Rubinstein SM, Peerdeman SM, van Tulder MW, et al. A systematic review of the risk factors
for cervical artery dissection. Stroke 2005; 36:1575.
73. Barbour PJ, Castaldo JE, Rae-Grant AD, et al. Internal carotid artery redundancy is
significantly associated with dissection. Stroke 1994; 25:1201.
74. Kim BJ, Yang E, Kim NY, et al. Vascular Tortuosity May Be Associated With Cervical Artery
Dissection. Stroke 2016; 47:2548.
75. Schievink WI, Mokri B, Piepgras DG. Angiographic frequency of saccular intracranial
aneurysms in patients with spontaneous cervical artery dissection. J Neurosurg 1992; 76:62.
76. Schievink WI. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med
2001; 344:898.
77. Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-
Danlos syndrome type IV, the vascular type. N Engl J Med 2000; 342:673.
78. Schievink WI, Björnsson J, Piepgras DG. Coexistence of fibromuscular dysplasia and cystic
medial necrosis in a patient with Marfan's syndrome and bilateral carotid artery
dissections. Stroke 1994; 25:2492.
79. Harrer JU, Sasse A, Klötzsch C. Intimal flap in a common carotid artery in a patient with
Marfan's syndrome. Ultraschall Med 2006; 27:487.
80. Wityk RJ, Zanferrari C, Oppenheimer S. Neurovascular complications of marfan syndrome: a

retrospective, hospital-based study. Stroke 2002; 33:680.
81. Faivre L, Collod-Beroud G, Loeys BL, et al. Effect of mutation type and location on clinical
outcome in 1,013 probands with Marfan syndrome or related phenotypes and FBN1
mutations: an international study. Am J Hum Genet 2007; 81:454.
82. Guillon B, Berthet K, Benslamia L, et al. Infection and the risk of spontaneous cervical artery
dissection: a case-control study. Stroke 2003; 34:e79.
83. Hunter MD, Moon YP, Miller EC, et al. Influenza-Like Illness is Associated with Increased
Short-Term Risk of Cervical Artery Dissection. J Stroke Cerebrovasc Dis 2021; 30:105490.
84. Witsch J, Rutrick SB, Lansdale KN, et al. Influenza-Like Illness as a Short-Term Risk Factor for
Arterial Dissection. Stroke 2023; 54:e66.
85. Pezzini A, Caso V, Zanferrari C, et al. Arterial hypertension as risk factor for spontaneous
cervical artery dissection. A case-control study. J Neurol Neurosurg Psychiatry 2006; 77:95.
86. Debette S, Metso T, Pezzini A, et al. Association of vascular risk factors with cervical artery
dissection and ischemic stroke in young adults. Circulation 2011; 123:1537.
87. Metso TM, Metso AJ, Salonen O, et al. Adult cervicocerebral artery dissection: a single-
center study of 301 Finnish patients. Eur J Neurol 2009; 16:656.
88. Metso TM, Tatlisumak T, Debette S, et al. Migraine in cervical artery dissection and ischemic
stroke patients. Neurology 2012; 78:1221.
89. Rist PM, Diener HC, Kurth T, Schürks M. Migraine, migraine aura, and cervical artery
dissection: a systematic review and meta-analysis. Cephalalgia 2011; 31:886.
90. De Giuli V, Grassi M, Lodigiani C, et al. Association Between Migraine and Cervical Artery
Dissection: The Italian Project on Stroke in Young Adults. JAMA Neurol 2017; 74:512.
91. Magalhães JE, Sampaio Rocha-Filho PA. Migraine and cerebrovascular diseases:
Epidemiology, pathophysiological, and clinical considerations. Headache 2018; 58:1277.
92. Del Zotto E, Grassi M, Zedde M, et al. Risk Profile of Patients with Spontaneous Cervical

Artery Dissection. Ann Neurol 2023; 94:585.

93. Benninger DH, Herrmann FR, Georgiadis D, et al. Increased prevalence of
hyperhomocysteinemia in cervical artery dissection causing stroke: a case-control study.
Cerebrovasc Dis 2009; 27:241.
94. Ortiz J, Ruland S. Cervicocerebral artery dissection. Curr Opin Cardiol 2015; 30:603.
95. Arnold M, Pannier B, Chabriat H, et al. Vascular risk factors and morphometric data in
cervical artery dissection: a case-control study. J Neurol Neurosurg Psychiatry 2009; 80:232.
96. Raser JM, Mullen MT, Kasner SE, et al. Cervical carotid artery dissection is associated with
styloid process length. Neurology 2011; 77:2061.
97. Baldino G, Di Girolamo C, De Blasis G, Gori A. Eagle Syndrome and Internal Carotid Artery
Dissection: Description of Five Cases Treated in Two Italian Institutions and Review of the
Literature. Ann Vasc Surg 2020; 67:565.e17.
98. Salehi Omran S, Parikh NS, Poisson S, et al. Association between Pregnancy and Cervical
Artery Dissection. Ann Neurol 2020; 88:596.
99. Traenka C, Kloss M, Strom T, et al. Rare genetic variants in patients with cervical artery
dissection. Eur Stroke J 2019; 4:355.
100. Wang K, Zhao S, Zhang Q, et al. Whole-exome sequencing reveals known and novel variants
in a cohort of intracranial vertebral-basilar artery dissection (IVAD). J Hum Genet 2018;
63:1119.
101. Debette S, Kamatani Y, Metso TM, et al. Common variation in PHACTR1 is associated with
susceptibility to cervical artery dissection. Nat Genet 2015; 47:78.
102. Martin JJ, Hausser I, Lyrer P, et al. Familial cervical artery dissections: clinical, morphologic,
and genetic studies. Stroke 2006; 37:2924.
103. Schievink WI, Mokri B, Piepgras DG, Kuiper JD. Recurrent spontaneous arterial dissections:
risk in familial versus nonfamilial disease. Stroke 1996; 27:622.
104. Grond-Ginsbach C, Klima B, Weber R, et al. Exclusion mapping of the genetic predisposition
for cervical artery dissections by linkage analysis. Ann Neurol 2002; 52:359.
105. Wiest T, Hyrenbach S, Bambul P, et al. Genetic analysis of familial connective tissue
alterations associated with cervical artery dissections suggests locus heterogeneity. Stroke
2006; 37:1697.
106. Atalay YB, Piran P, Chatterjee A, et al. Prevalence of Cervical Artery Dissection Among
Hospitalized Patients With Stroke by Age in a Nationally Representative Sample From the
United States. Neurology 2021; 96:e1005.

107. Beletsky V, Nadareishvili Z, Lynch J, et al. Cervical arterial dissection: time for a therapeutic
trial? Stroke 2003; 34:2856.
108. Schievink WI, Mokri B, O'Fallon WM. Recurrent spontaneous cervical-artery dissection. N
Engl J Med 1994; 330:393.
109. Paciaroni M, Georgiadis D, Arnold M, et al. Seasonal variability in spontaneous cervical
artery dissection. J Neurol Neurosurg Psychiatry 2006; 77:677.
110. Kim BM, Kim SH, Kim DI, et al. Outcomes and prognostic factors of intracranial unruptured
vertebrobasilar artery dissection. Neurology 2011; 76:1735.
111. Debette S, Compter A, Labeyrie MA, et al. Epidemiology, pathophysiology, diagnosis, and
management of intracranial artery dissection. Lancet Neurol 2015; 14:640.
112. Debette S, Grond-Ginsbach C, Bodenant M, et al. Differential features of carotid and
vertebral artery dissections: the CADISP study. Neurology 2011; 77:1174.
113. Morris NA, Merkler AE, Gialdini G, Kamel H. Timing of Incident Stroke Risk After Cervical
Artery Dissection Presenting Without Ischemia. Stroke 2017; 48:551.
114. Mokri B, Sundt TM Jr, Houser OW, Piepgras DG. Spontaneous dissection of the cervical
internal carotid artery. Ann Neurol 1986; 19:126.
115. Manabe H, Yonezawa K, Kato T, et al. Incidence of intracranial arterial dissection in non-
emergency outpatients complaining of headache: preliminary investigation with MRI/MRA
examinations. Acta Neurochir Suppl 2010; 107:41.
116. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid
and vertebral artery dissections. Neurology 1995; 45:1517.
117. Gallerini S, Marsili L, Bartalucci M, et al. Headache secondary to cervical artery dissections:
practice pointers. Neurol Sci 2019; 40:613.
118. Diamanti S, Longoni M, Agostoni EC. Leading symptoms in cerebrovascular diseases: what
about headache? Neurol Sci 2019; 40:147.
119. Headache Classification Committee of the International Headache Society (IHS) The
International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018; 38:1.
120. Mitsias P, Ramadan NM. Headache in ischemic cerebrovascular disease. Part I: Clinical
features. Cephalalgia 1992; 12:269.
121. Guillon B, Biousse V, Massiou H, Bousser MG. Orbital pain as an isolated sign of internal
carotid artery dissection. A diagnostic pitfall. Cephalalgia 1998; 18:222.
122. Richoz O, Scott Schutz J, Mégevand P. Pearls & Oy-sters: unusual manifestations of bilateral
carotid artery dissection: deep monocular pains. Neurology 2012; 78:e16.

123. Shankar Kikkeri N, Nagarajan E, Sakuru RC, Bollu PC. Horner Syndrome Due to
Spontaneous Internal Carotid Artery Dissection. Cureus 2018; 10:e3382.
124. Flaherty PM, Flynn JM. Horner syndrome due to carotid dissection. J Emerg Med 2011;
41:43.
125. Mokri B, Silbert PL, Schievink WI, Piepgras DG. Cranial nerve palsy in spontaneous
dissection of the extracranial internal carotid artery. Neurology 1996; 46:356.
126. Mattioni A, Paciaroni M, Sarchielli P, et al. Multiple cranial nerve palsies in a patient with
internal carotid artery dissection. Eur Neurol 2007; 58:125.
127. English SW, Passe TJ, Lindell EP, Klaas JP. Multiple cranial neuropathies as a presentation of
spontaneous internal carotid artery dissection: A case report and literature review. J Clin
Neurosci 2018; 50:129.
128. Song JX, Lin XM, Hao ZQ, et al. Ocular manifestations of internal carotid artery dissection.
Int J Ophthalmol 2019; 12:834.
129. Crum B, Mokri B, Fulgham J. Spinal manifestations of vertebral artery dissection. Neurology
2000; 55:304.
130. Wagner F, Mono ML, Köndgen H, et al. Cervical polyradiculopathy caused by vertebral
artery dissection. Lancet 2013; 381:1510.
131. Quinn C, Salameh J. Vertebral artery dissection causing an acute C5 radiculopathy.
Neurology 2013; 81:1101.
132. Eberhardt O, Topka H. Compressive Cervical Radiculopathy due to Vertebral Artery

Dissection. J Stroke Cerebrovasc Dis 2015; 24:e115.
133. Pelkonen O, Tikkakoski T, Luotonen J, Sotaniemi K. Pulsatile tinnitus as a symptom of
cervicocephalic arterial dissection. J Laryngol Otol 2004; 118:193.
134. Shimizu Y, Yagi M. Pulsatile tinnitus and carotid artery dissection. Auris Nasus Larynx 2018;
45:175.
135. Kellert L, Kloss M, Pezzini A, et al. Prognostic significance of pulsatile tinnitus in cervical
artery dissection. Eur J Neurol 2016; 23:1183.
136. Biousse V, Touboul PJ, D'Anglejan-Chatillon J, et al. Ophthalmologic manifestations of
internal carotid artery dissection. Am J Ophthalmol 1998; 126:565.
137. Biousse V, Schaison M, Touboul PJ, et al. Ischemic optic neuropathy associated with internal
carotid artery dissection. Arch Neurol 1998; 55:715.
138. Gottesman RF, Sharma P, Robinson KA, et al. Clinical characteristics of symptomatic
vertebral artery dissection: a systematic review. Neurologist 2012; 18:245.

139. Cadena R. Cervical artery dissection: early recognition and stroke prevention. Emerg Med
Pract 2016; 18:1.
140. Traenka C, Dougoud D, Simonetti BG, et al. Cervical artery dissection in patients ≥60 years:
Often painless, few mechanical triggers. Neurology 2017; 88:1313.
141. Hakimi R, Sivakumar S. Imaging of Carotid Dissection. Curr Pain Headache Rep 2019; 23:2.
142. Provenzale JM, Sarikaya B. Comparison of test performance characteristics of MRI, MR
angiography, and CT angiography in the diagnosis of carotid and vertebral artery
dissection: a review of the medical literature. AJR Am J Roentgenol 2009; 193:1167.
143. Vertinsky AT, Schwartz NE, Fischbein NJ, et al. Comparison of multidetector CT angiography
and MR imaging of cervical artery dissection. AJNR Am J Neuroradiol 2008; 29:1753.
144. Provenzale JM. MRI and MRA for evaluation of dissection of craniocerebral arteries: lessons
from the medical literature. Emerg Radiol 2009; 16:185.
145. Benninger DH, Baumgartner RW. Ultrasound diagnosis of cervical artery dissection. Front
Neurol Neurosci 2006; 21:70.
146. Imray CH, Pattinson KT. Potential role for TCD-directed antiplatelet agents in symptomatic
carotid artery dissection. Stroke 2006; 37:767.
147. Sengelhoff C, Nebelsieck J, Nassenstein I, et al. Neurosonographical follow-up in patients
with spontaneous cervical artery dissection. Neurol Res 2008; 30:687.
148. Wessels T, Mosso M, Krings T, et al. Extracranial and intracranial vertebral artery dissection:
long-term clinical and duplex sonographic follow-up. J Clin Ultrasound 2008; 36:472.
149. Nebelsieck J, Sengelhoff C, Nassenstein I, et al. Sensitivity of neurovascular ultrasound for

the detection of spontaneous cervical artery dissection. J Clin Neurosci 2009; 16:79.
150. Arnold M, Baumgartner RW, Stapf C, et al. Ultrasound diagnosis of spontaneous carotid
dissection with isolated Horner syndrome. Stroke 2008; 39:82.
151. Dittrich R, Dziewas R, Ritter MA, et al. Negative ultrasound findings in patients with cervical
artery dissection. Negative ultrasound in CAD. J Neurol 2006; 253:424.
152. Choi KD, Jo JW, Park KP, et al. Diffusion-weighted imaging of intramural hematoma in
vertebral artery dissection. J Neurol Sci 2007; 253:81.
153. Lum C, Chakraborty S, Schlossmacher M, et al. Vertebral artery dissection with a normal-
appearing lumen at multisection CT angiography: the importance of identifying wall
hematoma. AJNR Am J Neuroradiol 2009; 30:787.
154. Bonati LH, Wetzel SG, Gandjour J, et al. Diffusion-weighted imaging in stroke attributable to
internal carotid artery dissection: the significance of vessel patency. Stroke 2008; 39:483.

155. Bachmann R, Nassenstein I, Kooijman H, et al. High-resolution magnetic resonance

imaging (MRI) at 3.0 Tesla in the short-term follow-up of patients with proven cervical
artery dissection. Invest Radiol 2007; 42:460.
156. Naggara O, Touzé E, Marsico R, et al. High-resolution MR imaging of periarterial edema
associated with biological inflammation in spontaneous carotid dissection. Eur Radiol 2009;
19:2255.
157. Silverman IE, Wityk RJ. Transient migraine-like symptoms with internal carotid artery
dissection. Clin Neurol Neurosurg 1998; 100:116.
Topic 14082 Version 38.0

GRAPHICS
The progression of a dissection, thrombus development, and total vessel

occlusion
Courtesy of Dr. Mounzer Kassab.
Graphic 57866 Version 2.0

Mechanism of dissecting aneurysm formation
Lateral and cross-sectional schematic view of the carotid artery.
(A) Aneurysmal dilatation of the vessel related to a hemorrhage between the media and the adventitia
(subadventitial dissection).
(B) Communication between the arterial lumen and the dissection cavity or disappearance of the
hematoma may lead to the formation of an extraluminal pouch or a fusiform dilatation.
Reproduced with permission from: Guillon B, Brunereau L, Biousse V, et al. Long-term follow-up of aneurysms developed during
extracranial internal carotid artery dissection. Neurology 1999; 53:117. Copyright © 1999 Lippincott Williams & Wilkins.

Segments of the vertebral artery
The vertebral artery is divided into four anatomic segments:
V1 – Origin of the vessel to the foramina of the sixth cervical (C6) transverse process.
V2 – Intraforaminal segment from the sixth to the second cervical vertebral body (C6 to C2).
V3 – From the second cervical (C2) foramina to the base of the skull.
V4 – Intracerebral segment of the vertebral artery. The vertebral arteries merge to form the basilar
artery and are intradural.

Pathology of internal carotid artery dissection
Elastica van Gieson stain of specimens from a 61-year-old man with internal carotid artery dissection and
fibromuscular dysplasia.
(A) Postmortem exposition of intramural hematoma of the right internal carotid artery above the
bifurcation (arrow).
(B) Cross-section of the dissected artery shows subintimal and intramedial hematoma (small arrows).
(C) Longitudinal section with zipper-like separation within the arterial wall (large arrow) corresponding to
the false lumen and myxoid degeneration of the media (small arrows).
Asterisk: true lumen; Scale bar: 0.25 mm.
Reproduced with permission from: Besselmann M, Vennemann B, Lowens S, et al. Internal carotid artery dissection. Neurology
2000; 54:442. Copyright © 2000 Lippincott Williams & Wilkins.

Horner syndrome due to internal carotid artery dissection
(A) Horner syndrome (miosis and ptosis).
(B) Multiple left frontoparietal acute ischemic lesions on diffusion-weighted MRI.
(C) Carotid angiography with evidence of left extracranial internal carotid artery occlusion.
(D) Internal carotid artery mural hematoma due to dissection evident on MRI scan.
(E) Intimal flap (arrows) appearance on Duplex scan, a pathognomonic sign of dissection.
Reproduced with permission from: Mazzucco S, Rizzuto N. Teaching NeuroImage: Horner syndrome due to internal carotid artery
dissection. Neurology 2006; 66:E19. Copyright © 2006 Lippincott Williams & Wilkins.


Isolated hypoglossal nerve palsy due to internal carotid artery dissection
(A) The patient's tongue deviated to the left.
(B) T1-weighted MRI demonstrates narrowing of the lumen and intramural hematoma in the left internal
carotid artery.
(C) True fast MRI with steady-state precession reveals the anatomic juxtaposition of the hypoglossal
nerve (arrowhead) and the dissected internal carotid artery (arrow).
Reproduced with permission from: Okunomiya T, Kageyama T, Suenaga T. Teaching NeuroImages: Isolated hypoglossal nerve
palsy due to internal carotid artery dissection. Neurology 2012; 79:e37. Copyright © 2012 Lippincott Williams & Wilkins.

String sign on MR angiography
Left internal carotid artery dissection in a 43-year-old man. A hemorrhagic crescent on axial T1 cervical
MRI (A) and the string sign on follow-up neck MR angiography (B) are consistent with dissection.
MRI: magnetic resonance imaging; MR: magnetic resonance.
Reproduced with permission from: Pary LF, Rodnitzky RL. Traumatic internal carotid artery dissection associated with taekwondo.
Neurology 2003; 60:1392. Copyright © 2003 Lippincott Williams & Wilkins.

Flame-shaped occlusion of internal carotid artery from dissection
Digital subtraction angiogram (lateral projection) shows an internal carotid artery occlusion secondary to
dissection, as indicated by the typical flame-shaped appearance and location of the occlusion distal to the
carotid bifurcation.
Reproduced with permission from: Koch S, Lorenzo D, Rabinstein AA, Lam B. Ischemic optic neuropathy and carotid dissection.
Neurology 2005; 64:827. Copyright © 2005 Lippincott Williams & Wilkins.

Intracranial dissection with intimal flap
(A, B) Diffusion-weighted sequence of MRI of the brain shows acute infarction in the right MCA territory.
(C, D) Catheter angiography showed irregularities of the right supraclinoid internal carotid artery,
extending into the M1 segment of the right MCA. An intimal flap (arrow) indicates dissection.
MRI: magnetic resonance imaging; MCA: middle cerebral artery.
Reproduced with permission from: Suter B, El-Hakam LM. Child neurology: stroke due to nontraumatic intracranial dissection in a
child. Neurology 2009; 72:e100. Copyright © 2009 Lippincott Williams & Wilkins.

Extracranial aneurysm of the internal carotid artery
Arteriography of an extracranial aneurysm of the internal carotid artery at the skull base resulting from
carotid dissection in a 34-year-old man.
Reproduced with permission from: Meairs S, Hennerici M. Long-term follow-up of aneurysms developed during extracranial
internal carotid artery dissection. Neurology 2000; 54:2190. Copyright © 2000 Lippincott Williams & Wilkins.

MRI of ICA dissection
Axial T1-weighted MRI showing a right internal carotid artery dissection as a crescent-shaped hypersignal
within the internal carotid artery wall.
MRI: magnetic resonance imaging; ICA: internal carotid artery.

Etiologies of thunderclap headache
Most common causes of thunderclap headache:
Subarachnoid hemorrhage
Reversible cerebral vasoconstriction syndromes (RCVS)
Conditions that less commonly cause thunderclap headache:

Cerebral infection (eg, meningitis, acute complicated sinusitis)
Cerebral venous thrombosis
Cervical artery dissection
Spontaneous intracranial hypotension
Acute hypertensive crisis
Posterior reversible leukoencephalopathy syndrome (PRES)
Intracerebral hemorrhage
Ischemic stroke
Conditions that uncommonly or rarely cause thunderclap headache:

Pituitary apoplexy
Colloid cyst of the third ventricle
Aortic arch dissection
Aqueductal stenosis
Brain tumor
Giant cell arteritis
Pheochromocytoma
Pneumocephalus
Retroclival hematoma
Spinal epidural hematoma
Varicella zoster virus vasculopathy
Vogt-Koyanagi-Harada syndrome
Disputed causes of thunderclap headache:
Sentinel headache (unruptured intracranial aneurysm)*
Primary thunderclap headache ¶

* Sentinel headache due to an unruptured intracranial aneurysm is a possible cause of thunderclap

headache, but supporting data are weak.
¶ There is controversy as to whether thunderclap headache can occur as a benign and potentially
recurrent headache disorder in the absence of underlying organic intracranial pathology.

Contributor Disclosures
David S Liebeskind, MD Consultant/Advisory Boards: Cerenovus [Stroke]; Genentech [Stroke]; Medtronic
[Stroke]; Stryker [Stroke]. Speaker's Bureau: Astra-Zeneca [Stroke]. All of the relevant financial relationships
listed have been mitigated. Scott E Kasner, MD Grant/Research/Clinical Trial Support: Bayer [Stroke];
Bristol Meyers Squibb [Stroke]; Medtronic [Stroke]; WL Gore and Associates [Stroke]. Consultant/Advisory
Boards: Abbvie [Stroke]; AstraZeneca [Stroke]; BMS [Stroke]; Diamedica [Stroke]; Medtronic [Stroke]. All of
the relevant financial relationships listed have been mitigated. John F Dashe, MD, PhD No relevant
financial relationship(s) with ineligible companies to disclose.
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.
Conflict of interest policy

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1/6/24, 3:06 PM Cerebral and cervical artery dissection: Clinical features and diagnosis - UpToDate

Official reprint from UpToDate®

Cerebral and cervical artery dissection: Clinical features

Literature review current through: Dec 2023.

ANATOMY AND PATHOLOGY

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Multiple simultaneous cervicocephalic dissections are found in 13 to 22 percent of cases [6-9]

There is a high prevalence of ultrastructural connective tissue abnormalities in patients with

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Subadventitial dissections with aneurysm or hematoma formation and vessel dilatation

● Sexual intercourse [42]

• Fibromuscular dysplasia [59-63]

The most common association is with fibromuscular dysplasia, a nonspecific arteriopathy,

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● Other conditions – A number of other conditions have been associated with

• Recent infection [58,72,82-84]

• Pregnancy, mainly in the postpartum period [98]

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Horner syndrome — Horner syndrome ( figure 4) occurs in approximately 25 percent of

Cranial or cervical neuropathies — Carotid dissection may cause single or multiple

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Subarachnoid hemorrhage — Intracranial artery dissection may result in subarachnoid

EVALUATION AND DIAGNOSIS

● History of recent potential triggering events:

● Personal or family history of connective tissue or vascular disorders or migraine

● Acute or subacute symptoms:

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These imaging studies should be performed urgently as part of a comprehensive stroke

Characteristic imaging findings — Angiographic findings of dissection include:

● String sign ( image 1)

In a population-based study of 48 consecutive patients with cervical artery dissection, the

The pathognomonic crescent sign of intramural hematoma is formed by an eccentric rim of

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The diagnosis of fibromuscular dysplasia (FMD) is made from angiography, so typically no

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● Thunderclap headache – Thunderclap headache, a severe headache of sudden onset,

● Subarachnoid hemorrhage – The differential diagnosis of subarachnoid hemorrhage

SOCIETY GUIDELINE LINKS

SUMMARY AND RECOMMENDATIONS

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occurs in the cervical transverse processes of C6 to C2 (V2 segment) or the extracranial

● Pathophysiology – Separation of the arterial wall layers results in dissection. A false

● Clinical manifestations – Evidence from population and hospital-based reports suggests

● Evaluation and diagnosis – We obtain urgent noninvasive multimodal imaging with

● Treatment and prognosis – The treatment and prognosis of cervicocephalic dissection is

Use of UpToDate is subject to the Terms of Use.

3. Downer J, Nadarajah M, Briggs E, et al. The location of origin of spontaneous extracranial

7. Arnold M, Kappeler L, Georgiadis D, et al. Gender differences in spontaneous cervical artery

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25. Leon-Sanchez A, Cuetter A, Ferrer G. Cervical spine manipulation: an alternative medical

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Front Neurol Neurosci 2005; 20:44.

38. Lascelles K, Hewes D, Ganesan V. An unexpected consequence of a roller coaster ride. J

https://www.uptodate.com/contents/cerebral-and-cervical-artery-dissection-clinical-features-and-diagnosis/print?search=carotid artery dissection&source=search_r… 17/40

58. Debette S, Leys D. Cervical-artery dissections: predisposing factors, diagnosis, and

Circulation 2014; 129:1048.

80. Wityk RJ, Zanferrari C, Oppenheimer S. Neurovascular complications of marfan syndrome: a

https://www.uptodate.com/contents/cerebral-and-cervical-artery-dissection-clinical-features-and-diagnosis/print?search=carotid artery dissection&source=search_r… 20/40