Unusual presentation of more common disease/injury

Atrial myxoma: a rare cause of cardioembolic stroke
Junaid Akhtar, Mohammad Wasay, Javeria Rauf
Neurology Department, Aga Khan University Hospital, Karachi, Sindh, Pakistan
Correspondence to Dr Junaid Akhtar, drjunaid.akhtar@hotmail.com

Summary
The authors present the case of a 45-year-old lady who presented to the emergency department with sudden onset of right hemiparesis,
numbness, dysarthria, dysmetria, left upper motor neuron facial palsy and left extensor plantar. Initial laboratory investigations, ECG and MR
angiography were normal. MRI brain revealed multiple ischaemic strokes in middle cerebral artery and posterior cerebral artery territory.
Transthoracic echocardiography revealed large mobile mass in left atrium which was resected and the patient remained well postoperatively.
Anticoagulants do not play any protective role making resection as the only effective treatment.

BACKGROUND
At least 20% of all ischaemic strokes are cardioembolic.
Cardiac myxoma is a source of emboli to the central nervous system and elsewhere in the vascular tree.1 It is a welldescribed cause of stroke that usually occurs in young
people and is a potential treatable condition.2 Therefore,
it is important for the clinicians to detect the myxoma and
prevent its complications like syncope, recurrent strokes or
sudden cardiac death. In this case, a middle aged lady with
no known co-morbids presented with acute stroke and her
investigation revealed atrial myxoma on transesophageal
echocardiography which was successfully resected.

confirming acute stroke in the territory of bilateral middle cerebral artery and posterior cerebral artery (figure 1).
The results of MRA were unremarkable. Her carotid
doppler ultrasound and lipid profile were unremarkable.
Transthoracic echocardiography (TTE) was thereby done
to find out a cardiac source of embolism which revealed
large (4.5×3×2.5 cm) mobile echogenic density noted in
the left atrium attached to the interatrial septum and was
consistent with an atrial myxoma of size. She was diagnosed as a case of cardioembolic infarction secondary to
atrial myxoma.

DIFFERENTIAL DIAGNOSIS
CASE PRESENTATION

▶

A 45-year-old female with no known comorbids, was
brought to our emergency department with sudden onset
of left hemiparesis and numbness for 1 day after an episode
of nausea and vomiting. Neurological examination revealed
that she was drowsy but arousable and was following two
steps commands. Neck was supple and speech was dysarthric. She had left upper motor neuron facial palsy and
rest of the cranial nerves were normal. Motor examination
revealed left-sided hemiparesis with a power of 4/5 in left
upper and lower limbs with left extensor plantar. Reflexes
were +1 in all over the limbs. Cerebellar examination
revealed bilateral finger nose dysmetria. Sensory examination was normal. On cardiovascular examination, S1 and
S2 were audible in all four areas with no added sounds and
pulse of 90 beats per min. Rest of the systemic examination was unremarkable.

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▶
▶
▶
▶
▶

Acute cardioembolic stroke
Acute thrombotic stroke
Cerebral venous thrombosis
Intracerebral haemorrhage
Subarachnoid haemorrhage
Cerebral abscess
Chronic central nervous system infection

TREATMENT

INVESTIGATIONS

The patient was managed on the line of acute stroke care.
She was admitted to the highly specialised stroke unit
and the supportive management including intravenous
fluids, oxygen, deep vein thrombosis stockings, antacids,
antiemetics etc along with antiplatelets and statins were
given. Investigation of stroke revealed atrial myxoma.
Cardiothoracic team was involved and they planned to
resect the lesion. She underwent the surgery after 7 days
of admission and was well postoperatively. Later histopathology confirmed the diagnosis.

Her initial laboratory investigations including complete
blood count, electrolytes, renal and liver functions were
normal. ECG revealed normal sinus rhythm. MRI brain
with time of flight MR angiography (MRA), was done
which showed diffusion restricted infarctions in bilateral
cerebellar hemisphere, vermis, bilateral pons, left-sided
occipital, parietal and basal ganglia. These areas appeared
dark on apparent diffusion coefficient mapping, thus

She remained in the hospital 4 days after the surgery and
was discharged without any poststroke complication.
Rehabilitation was started including physiotherapy, speech
therapy and occupational therapy for her residual deficits.
Postoperatively, she remained well without any surgical
complication.

BMJ Case Reports 2012; doi:10.1136/bcr.2012.006176

OUTCOME AND FOLLOW-UP

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Figure 1 (A, B) Multiple areas of diffusion restricted in middle cerebral artery and posterior cerebral artery territories. (C, D) Subsequent
areas that are dark on apparent diffusion coefficient mapping. (E) Normal anterior and posterior circulation vessels.

DISCUSSION
Atrial myxoma is a benign neoplasm of mesenchymal origin. Histologically, these tumours are composed of scattered cells within a mucopolysaccharide stroma. It is the
most frequent (70%) primary cardiac tumour in adults3
and its incidence is reported to be between 0.0017% and
0.19%.4 About 75%–90% of the cardiac myxomas occurs
in the left atrium, and is found more commonly between
3rd to 6th decades of life.5 Tumours vary widely in size,
ranging from 1 to 15 cm in diameter, and weigh between
15 and 180 g.6 About 35% of myxomas are friable or
villous, and these tend to present with emboli. Larger
tumours are more likely to have a smooth surface and to
be associated with cardiovascular symptoms. In at least
half of the cases, cerebral arteries are affected, leading to
embolic ischemic stroke.6 The male to female ratio is 1:2
with a slight female preponderance. Delay in the diagnosis after presentation is usually variable and may range
from 1 to 126 months.7
Most cases (90%) of atrial myxoma are sporadic with no
known cause.8 In remaining (10%), a familial pattern occurs
having an autosomal dominant pattern known as Carney
complex which is characterised by multiple tumors, including atrial and extracardiac myxomas, cutaneous spotty
pigmentation, non-myxomatous extracardiac tumours,
schwannomas and various endocrine tumours, through a
causative mutation of the PRKAR1α gene located on the
long arm of chromosome 17 (17q22-24 region).7 9
Patients with cardiac myxoma presents characteristically with a triad of obstructive symptoms (30%), constitutional symptoms (60%) and embolic phenomenon
(30–40%).8 Obstructive symptoms include atrioventricular
valve obstruction, dyspnoea, orthopnea, paraoxysmal nocturnal dyspnoea, oedema, dizziness, syncope, atrial fibrillation or sudden cardiac death. Embolic phenomenon such
as systemic embolisation presents with stroke, pulmonary
embolism or paradoxical embolism while constitutional
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symptoms presents as fever, weight loss, arthralgia or
Raynaud’s phenomena.2 10
Neurological manifestations of atrial myxoma vary
widely ranging from embolic,6 haemorrhagic or recurrent infarctions11 and progressive multi-infarct dementia12
to massive embolic stroke causing sudden death.13 Since
tumour fragments may embolise, early anticoagulation may
not be protective in reducing disability and mortality.1
Laboratory investigations may reveal anaemia, polycythaemia, thrombocytopenia with raise erythrocyte
sedimentation rate, C reactive protein and imunoglobulins.14 Echocardiography is widely available and provides
a simple, non-invasive technique for the initial evaluation.
Echocardiography images both the myocardium and the
cardiac chambers and can usually identify the presence of a
mass and its mobility. Transesophageal echocardiography
which has been reported as having 100% sensitivity for
cardiac myxoma,7 15 is preferred over TTE. Cardiac MRI
can help out in demarcating tumour size, attachment and
its mobility7 16 and this information may be further used
during surgical resection. Surgery should not be delayed
even in asymptomatic cases which are revealed incidentally because of the risk of having further embolisation.
Neurological sequelae after resection are usually rare but
possiblity exist.7 Positron emission tomography scan has
been useful in identifying cardiac involvement in patients
with metastatic tumours17 and atrial myxoma.18
Once a presumptive diagnosis of myxoma has been
made on imaging studies, prompt resection is required
because of the risk of embolisation or cardiovascular complications including sudden death.19 The results of surgical
resection are generally very good, with most series reporting an operative death rate of <5%.20
There is a recurrence rate of 1–3% which is associated with sporadic cases of atrial myxoma, often because
of inadequate resection.8 These patients require annual
review for a period of 3–4 years with echocardiography,
BMJ Case Reports 2012; doi:10.1136/bcr.2012.006176

when the risk of recurrence is greatest.21 For Carney complex, which has a recurrence rate of up to 25%, lifetime
annual review with familial screening is recommended.22

Learning points
Cardiac echocardiography should always be
considered in young patients presenting with embolic
ischaemic stroke with usually normal MRA of neck
vessels.
▶ Patients with cardiac myxoma can present with
symptoms of atrioventricular valvular dysfunction,
stroke and neurological disorder or with any other
constitutional symptoms.
▶ Transesophageal echocardiography should be preffered
over transthoracic echocardiography.
▶ Once the diagnosis is made, surgery should not be
delayed.
▶

Competing interests None.
Patient consent Obtained.

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Akhtar J, Wasay M, Rauf J. Atrial myxoma: a rare cause of cardioembolic stroke. BMJ Case Reports 2012;10.1136/bcr.2012.006176, Published XXX
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