Article, Cardiology

Acute psychosis presenting as a sole manifestation of left atrial myxoma: a new paradigm

Unlabelled imageAcute psychosis presenting as a sole man”>American Journal of Emergency Medicine 32 (2014) 1556.e3-1556.e5

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Case Report

Acute psychosis presenting as a sole manifestation of left atrial myxoma: a new paradigm?,??,?


Atrial myxomas are the most common benign cardiac tumors. neurological manifestations associated with left atrial myxoma are seen infrequently in the form of embolic stroke of intracranial hemorrhage. Psychiatric manifestations are however exceedingly rare. We hereby present a case who presented with acute psychosis as a sole manifestation of an underlying left atrial myxoma.

Atrial myxomas, being the commonest benign tumor of mesenchy- mal origin, represent roughly 50% of all cardiac tumors, occurring mainly in third to sixth decade of life [1]. It is a well-described and a treatable cause of stroke. Patients with atrial myxoma present characteristically with a triad of constitutional symptoms (60%), embolic phenomenon (30%-40%), and obstructive symptoms (30%) [2]. Constitutional symp- toms include weight loss, fever, arthralgia, or Raynaud phenomenon. Embolic symptoms include stroke, pulmonary embolism, or paradoxical embolism. The embolization of thrombotic material covered with tumor cells occurs in up to 30% to 45% of cases, out of which half generally present as an embolic stroke [3]. Obstructive symptoms include atrioventricular valve obstruction resulting in tachypnea, dyspnea, syncope, or sudden cardiac death. Neurological manifestations of atrial myxoma may vary widely, ranging from recurrent embolic stroke and progressing to multi-infarct dementia, seizures, and cerebral hemor- rhage. The proposed mechanism for hemorrhagic stroke involves rupture of cerebral aneurysms that results from myxomatous embolization damaging the vessel walls [4,5]. Psychiatric manifestations are however seen very infrequently; and further, to the best of our knowledge, there has been no mention of acute psychosis as a sole manifestation of left atrial myxoma, which we hereby highlight in our case.

A 35-year-old woman presented with acute-onset psychosis of 10 days’ duration. The patient had anger spells, intense rage, irrelevant talking with delusions, and mood changes and was verbally abusive as well. She was hospitalized in a peripheral setup initially, was prescribed tranquilizers, and was referred thereafter. She was taken over by the neurology department; and on further enquiry, it was revealed that she also had suffered right hemiparesis with dysarthria 1 year back, from which she recuperated within 3 weeks. She had no history of recurrent headaches, seizures, fever, vomitings, visual symptoms, palpitation, or dyspnea. She was neither taking any drugs nor treated for any chronic illnesses in the past. Her family history for a similar illness was also unremarkable. On examination, the patient was afebrile and normo- tensive; and all peripheral pulses were well palpable. She was conscious

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?? Conflicts of interest: nil.

? Presentation at meeting: none.

but agitated and was talking irrelevantly. She had a tendency to run away from bedside and was difficult to curtail. She was having no apparent focal weakness, but her right plantar was extensor. No meningeal signs were present. Curiously, her cardiac examination revealed a loud S1 and “plop.” All her routine hematological and Biochemical parameters and the results of electroencephalograph were normal. Her chest radiograph revealed straightening of the left heart border, whereas result of her electrocardiogram was normal. Her thyroid profile, serum ammonia levels, and vasculitis markers were normal. Her non-contrast CT scan of head showed hypodensities in the right high parietal and left internal capsular regions suggestive of chronic infarcts (Fig. 1A). Magnetic resonance imaging of the brain with diffusion-weighted images showed multiple chronic infarcts in the right parietal and left internal capsular region and an acute infarct with restricted diffusion in the left periventricular region (Fig. 1B and C). Considering the clinical profile with infarcts in multiple vascular territory, a possibility of central nervous system (CNS) vasculitis was there. A cerebrospinal fluid examination was undertaken, the result of which was normal. Subsequently, carotid Doppler and computed tomographic (CT) angiography of brain were also done, the results of which were normal (Fig. 1D). We confirmed our finding on cardiac examination with a transthoracic echocardiography that interestingly revealed a pedunculated left atrial mass (Fig. 2A) attached to interatrial septum prolapsing through mitral valve with an ejection fraction of 55%. Subsequently, excision of left atrial mass was done under cardiopul- monary bypass; and the mass was subjected to biopsy. Interestingly, the biopsy was suggestive of atrial myxoma (Fig. 2B). The patient showed a dramatic recovery in her psychosis following the surgery and did well on anticoagulants that were titrated as per prothrombin time and antiplatelets during follow-up visits. There was no recurrence of a strokelike episode or any behavioral change.

Atrial myxomas may remain asymptomatic in 10% to 20% of cases

and can be only found as an incidental finding on echocardiography. Further auscultation abnormalities may be absent in up to 36% of cases [6]; and overall, neurological complications may be seen in 26% to 45% of cases, out of which only 0.5% of cases present with ischemic stroke [7]. Our patient did not have any cardiac symptoms whatsoever; but auscultation findings guided us to the diagnosis, which was later proved by echocardiography and subsequently confirmed by tissue diagnosis. There has been a mention only in the report by Ekinci and Donnan [8] where they reported that psychiatric manifestations such as acute confusional states, chronic cognitive impairment, or behavioral abnormalities were seen in around 23% of the cases. However, acute psychosis as a sole presenting feature has never been described to the best of our knowledge. Differentiating it from other disorders such as infections of the CNS, CNS vasculitis, reversible cerebral vasoconstriction syndrome, or toxin exposure is prudent. Investigational techniques such

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Fig. 1. A, noncontrast CT of the head showing hypodensities in the left caudate, globus pallidus, and right temporoparietal cortex suggestive of chronic infarcts. B, Magnetic resonance imaging of the brain showing multiple hyperintense signals in the left thalamus, left caudate, and right internal capsular region suggestive of chronic infarcts. C, Diffusion-weighted image showing hyperintense signals in the left periventricular region suggestive of acute infarct (black arrow). D, Normal intracranial CT angiography result of the patient.

as preferably transesophageal echocardiography combined with neu- roimaging are essential to diagnose left atrial myxoma in case of clinical suspicion. Surgery in such cases should not be delayed even if patients are asymptomatic because a complication of systemic embolization is

always there. Neurological sequelae after resection are rare, but recurrences of myxoma have been reported in various reports [9]. Our patient, however, has been on regular follow-up for 3 years; and results of repeated echocardiography studies have been normal, and there has

Fig. 2. A, Two-dimensional echocardiography showing a pedunculated left atrial mass attached to interatrial septum prolapsing through mitral valve. B, High-magnification histopathology section showing elongated fusiform cells in an abundant myxoid background suggestive of atrial myxoma.

R.S. Jain et al. / American Journal of Emergency Medicine 32 (2014) 1556.e31556.e5

been no deterioration in her neurological status either. Otherwise, annual review to detect for myxoma recurrence is advised for up to 3 to 4 years, during which the risk is greatest [10].


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Psychiatric manifestations as presenting feature can always pose a challenge for the treating clinician, masquerading the underlying illness. A thorough clinical examination is of paramount importance even if the presenting manifestation is primarily a psychiatric symptom, as it may herald an occult disorder in its incipient stage with grave implications.



Rajendra Singh Jain, MD, DM?

Kadam Nagpal, MD Rahul Jain, MD Swayam Prakash, MD Rahul Handa, MD

Department of Neurology, SMS Medical College, Jaipur, Rajasthan India

? Corresponding author. 126, Janakpuri II, Imli Ka Phatak Jaipur, Rajasthan, India 302005. Tel.: +91 9414073579

E-mail addresses: [email protected]

[email protected] [email protected]


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