Rare disease

CASE REPORT

Isolated brachiocephalic artery dissection presenting
as acute stroke
Hariharasudan Mani,1 Sharat Ahluwalia2
1

Department of Internal
Medicine, Marshfield Clinic,
Eau Claire, Wisconsin, USA
2
Department of Neurology,
Marshfield Clinic, Eau Claire,
Wisconsin, USA
Correspondence to
Dr Hariharasudan Mani,
dr_harisudan@yahoo.co.in
Accepted 7 August 2015

SUMMARY
Isolated brachiocephalic artery dissection is an extremely
rare condition. Its presentation as an acute stroke can
pose a significant diagnostic challenge in patients
because of its rarity. We present a case of isolated
spontaneous brachiocephalic artery dissection presenting
as acute cerebrovascular accident. This case also
illustrates the treatment dilemma brachiocephalic artery
dissection can present, whether to choose
antithrombotic/anticoagulation therapy and/or surgery,
and also the dilemma in blood pressure management.

BACKGROUND
Spontaneous dissection of the internal carotid and
vertebral arteries is a common cause of ischaemic
strokes, particularly in children and young adults,
accounting for approximately 20% of these
strokes.1 In studies from North America and
Europe, the mean age of individuals affected by
dissection was 44–46 years;1–3 however, isolated
spontaneous brachiocephalic artery dissection is
very rare. Ischaemic stroke secondary to
supra-aortic dissection (SAD) accounts for approximately 10–25% of patients under 50 years of age.4
SAD commonly involves the internal carotid artery
and, less commonly, the vertebral and common
carotid arteries. Common causes of SAD are
trauma, sports injuries, neck manipulation and connective tissue or vascular disorders. Brachiocephalic
artery dissection is very rare, and few reports exist
in the literature.

cerebellar signs. On standing, he had a tendency to
drift to the left. Brain CT revealed acute ischaemic
infarct in the right basal ganglia. He was beyond
the window for treatment with recombinant tissue
plasminogen activator (rt-PA), so he was treated
with aspirin along with anticoagulation with
heparin.

INVESTIGATIONS AND TREATMENT
As part of the evaluation, the patient was noted
to have a difference in systolic blood pressure of
>20 mm Hg between his upper extremities. This
prompted further evaluation with angiography of
the chest, which revealed isolated brachiocephalic
artery dissection on the right with a slight extension into the proximal common carotid artery
(figure 1). The thoracic aorta was normal. The
patient’s other work-up including hypercoagulopathy and echocardiogram revealed no abnormalities. He was treated with heparin anticoagulation
and later bridged with Coumadin, to which he
responded fairly well. Except for minimal residual
dysarthria and facial asymmetry, which was persistent at the time of discharge 4 days later, the
patient’s overall symptoms improved. He was
involved in rehabilitation and is currently being
followed through the outpatient department.
His dissection is known to have happened
spontaneously.

CASE PRESENTATION

To cite: Mani H,
Ahluwalia S. BMJ Case Rep
Published online: [please
include Day Month Year]
doi:10.1136/bcr-2014208815

A 41-year-old obese man with undiagnosed hypertension presented to the emergency department for
symptoms of ataxia, slurred speech and left facial
weakness. He had noticed the symptoms the day
before at work, but did not seek immediate medical
attention. He sought medical help the next
morning because of persistence of the symptoms.
Family history was notable for his grandparents
having had strokes in their 60s, but the cause was
not known. He reported no history of coagulopathy, aortic dissection or connective tissue disorders
in his family. He had no history of antecedent
trauma. He was a non-smoker and denied using
illicit drugs. His blood pressure at presentation was
190/119 mm Hg, and he had frequent premature
ventricular contractions on ECG. On examination,
he was noted to have left facial asymmetry with
upper motor facial palsy. Minimal dysarthria was
noted, and no motor or sensory deficits in his
extremities were present. There was no presence of

Figure 1 Angiography of the chest showing isolated
brachiocephalic artery dissection on the right with a
slight extension into the proximal common carotid artery.

Mani H, Ahluwalia S. BMJ Case Rep 2015. doi:10.1136/bcr-2014-208815

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Rare disease
DISCUSSION
Spontaneous dissection of common carotid artery most commonly presents with headache and/or neck pain in 60–90% of
patients.1 5 Other manifestations include Horner’s syndrome,
cerebrovascular accidents and subarachnoid haemorrhage. In
adults, approximately 2% of ischaemic strokes are caused by
cervical artery dissection. In one study of spontaneous cervical
artery dissection, cerebral ischaemia was noted in 67% of
patients, with transient ischaemic attacks (TIAs) and cerebral
infarction in 23% and 56%, respectively.1
Aortic dissection commonly presents with tearing chest
pain;6 7 other presenting symptoms include syncope, cerebrovascular accident, aortic insufficiency, cardiac tamponade or
heart failure. In one study, up to 10% of patients presented with
neurological symptoms, but without chest pain.8 SAD is characterised by the presence of an intramural haematoma due to an
intimal tear in the artery wall. Subsequent ischaemic stroke is
believed to be caused due to the thromboembolism from the
intimal tear rather than by haemodynamic factors.
Antithrombotic therapy, with either anticoagulation or antiplatelet drugs, along with possible endovascular techniques such an
angioplasty or stenting,9 10 has been used as treatment for
ischaemic stroke and TIA caused by cervical arterial dissection.
In theory, anticoagulation may prevent occlusion of a stenotic
vessel and minimise distal embolisation. Antithrombotic/anticoagulation is the treatment of choice in such situations, since
expansion of the intramural haematoma is rather a theoretical
concern.4 Type A ascending aortic dissection is a surgical emergency11 and is treated with a surgical graft. Any anticoagulation/
antiplatelet is discontinued with involvement of aortic
dissection.
There are few reports in the literature of isolated dissection of
the brachiocephalic artery presenting with stroke and treated
with anticoagulation. In one other reported case of spontaneous
brachiocephalic artery dissection, initial improvement with
thrombolysis did occur and subsequent worsening was noticed.
It was attributed possibly to the flow disturbance from intramural haematoma.4 In our case, the patient did have improvement of his symptoms with anticoagulation, although rt-PA was
not used. Brachiocephalic artery dissection presents the dilemma
of managing either with anticoagulation or surgery, depending
on the involvement of the base of the ascending aorta.12 13
A pulse deficit or a difference in blood pressure of >20 mm Hg
between arms could be a sign of aortic or brachiocephalic artery
dissection, although other conditions that could cause this
deficit include subclavian artery stenosis or peripheral artery
disease.14 There have been few cases of residual brachiocephalic
artery dissection in the setting of post-type A aortic dissection
repair;15 and there have been reports of brachiocephalic artery
aneurysm with contained rupture managed by surgical
graft.12 13
Increase in blood pressure is a common finding in patients
with acute stroke. For patients who will be receiving antithrombotic therapy, it is recommended to decrease systolic blood pressure below 185 mm Hg and diastolic blood pressure below
110 mm Hg. In patients not treated with antithrombotics,
decreasing the blood pressure is only recommended in the presence of systolic blood pressure >220 mm Hg or diastolic blood
pressure >120 mm Hg, or in the presence of aortic dissection,
acute ischaemic coronary disease, or heart failure.16 In our
patient, the blood pressure decreased to 150/82 mm Hg within
hours of presentation without treatment. We could not find any
guidelines in the literature for managing blood pressure in SAD.
2

This case represents a rare presentation of brachiocephalic
artery dissection and the treatment with antithrombotic and/or
anticoagulation therapy as the choice in the absence of aortic
involvement. Also, blood pressure targeting still remains controversial given the conflicting conditions such as ischaemic stroke
and arterial wall dissection.4

Patient’s perspective
I can’t express enough thanks and gratitude to Dr Mani for all
the help and care I was given. I didn’t know it was as serious
as it turned out. Other doctors have told me I was very lucky
Dr Mani found what he did when he did. It may have very well
been life saving. So my thanks from the bottom of my heart.
I hope my case can be published as something that can help
others.

Learning points
▸ This case illustrates the potential for spontaneous isolated
brachiocephalic artery dissection as a rare but possible cause
of stroke.
▸ Standard stroke evaluation protocol should still be
implemented.
▸ A pulse deficit or a >20 mm Hg difference in blood pressure
between arms should prompt further evaluation with
angiography. Routine CT angiography in all patients with
stroke is still not recommended.
▸ Anticoagulation and tissue plasminogen activator (rt-PA)
with/without antiplatelet is still a reasonable option in such
patient presentation.

Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.

REFERENCES
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2
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6
7

8
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Lee VH, Brown RD Jr, Mandrekar JN, et al. Incidence and outcome of cervical artery
dissection: population-based study. Neurology 2006;67:1809–12.
Arnold M, Kappeler L, Georgiadis D, et al. Gender differences in spontaneous
cervical artery dissection. Neurology 2006;67:1050–2.
Touze E, Gauvrit JY, Moulin T, et al., Multicenter Survey on Natural History of
Cervical Artery Dissection. Risk of stroke and recurrent dissection after a cervical
artery dissection; a multicenter study. Neurology 2003;61:1347–51.
Sansone T, Baroncelli S, Tozzetti C, et al. Challenges in management of ischemic
stroke secondary to brachiocephalic artery dissection. Intern Emerg Med
2013;8:181–3.
Mokri B, Sundt TM Jr, Houser OW, et al. Spontaneous dissection of the cervical
internal carotid artery. Ann Neurol 1986;19:126–38.
Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161
cases. Am J Cardiol 1984;53:849–55.
Hagan PG, Nienaber CA, Isselbacher EM, et al. The international registry of acute
aortic dissection (IRAD): new insights into an old disease. JAMA
2000;283:897–903.
Gaul C, Dietrich W, Friedrich I, et al. Neurological symptoms in type A aortic
dissection. Stroke 2007;38:292–7.
Donas KP, Mayer D, Guber I, et al. Endovascular repair of extracranial carotid artery
dissection; current status and level of evidence. J Vasc Interv Radiol
2008;19:1693–8.

Mani H, Ahluwalia S. BMJ Case Rep 2015. doi:10.1136/bcr-2014-208815

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10
11
12

13

Engelter ST, Brandt T, Debette S, et al. Antiplatelets versus anticoagulation in
cervical artery dissection. Stroke 2007;38:2605–11.
Nienaber CA, Eagle KA. Aortic dissection: new frontiers in diagnosis and management:
part 1: from etiology to diagnostic strategies. Circulation 2003;108:628–35.
HIhashi S, Yoshida Y, Mitsuoka H. Dissecting aneurysms at the bases of the
brachiocephalic artery and the left common carotid artery due to localized dissection
of the aortic arch; report of a case. Kyobu Geka 2007;60:575–8.
Hirose T, Tabayashi N, Yoshikawa Y, et al. Isolated dissecting aneurysm of the
brachiocephalic artery associated with contained rupture. Gen Thorac Cardiovasc
Surg 2012;60:225–7.

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15

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Clark CE, Taylor RS, Shore AC, et al. Association of a difference in systolic blood
pressure between arms with vascular disease and mortality: a systemic review and
meta-analysis. Lancet 2012;379:905–14.
Neri E, Sani G, Massetti M, et al. Residual dissection of the brachiocephalic arteries:
significance, management, and long-term outcome. J Thorac Cardiovasc Surg
2004;128:303–12.
Jauch EC, Saver JL, Adams HP Jr, et al. Guidelines for the early management of
patients with acute ischemic stroke: a guideline for healthcare professionals from
the American Heart Association/American Stroke Association. Stroke
2013;44:870–947.

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Mani H, Ahluwalia S. BMJ Case Rep 2015. doi:10.1136/bcr-2014-208815

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