CLINICAL REPORT An Unusual Cause of Cardioembolic Stroke Isolated Left Ventricular Noncompaction Sevki Sahin, MD,* Ahmet Sekban, MD,† Sunay Ayalp, MD,* and Sibel Karsidag, MD* Background: Isolated left ventricular noncompaction (ILVNC) is a rare congenital condition that is the result of an intrauterine develop- mental arrest, which stops the compaction of the loose myocardial fiber meshwork of the left ventricle. This condition is recognized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Although these intertrabecular recesses are prone to thrombus formation, with resulting embolic sequelae, the literature describes only 1 adult case of a stroke caused by ILVNC. Case Report: We describe a case of cerebral cardioembolism in a 33-year-old man due to ILVNC. Because of echocardiography (ECHO) findings in this case, cardiac magnetic resonance imaging (cMRI) was performed, which allowed a definite diagnosis to be obtained. Conclusion: This case should highlight the importance of perform- ing cMRI especially in younger patients with embolic stroke, if the ECHO suggests a suspected myocardial disorder. Key Words: cardioembolic stroke, noncompaction, ECHO, cardiac MRI (The Neurologist 2008;14: 125–127) I solated left ventricular noncompaction (ILVNC) is a rare con- genital cardiomyopathy with prominent trabeculation and deep recesses of the left ventricular wall and no communication with the coronary arteries. 1,2 Systolic and diastolic ventricular dysfunction, conduction abnormalities, and cardioembolic events resulting from thrombus formation within the intertrabecular recesses are the most common clinical manifestations of this disorder. 3,4 CASE REPORT A previously healthy 33-year-old man presented with right hemiparesis and hemisensory loss which had developed 2 weeks previously. His neurologic examination showed right central facial paralysis, 4/5 right hemiparesis, right hemisen- sory loss, and an equivocal right plantar response. Brain magnetic resonance imaging (MRI) was performed and in T2 and fluid-attenuated inversion recovery (FLAIR) images, a hyperintense lesion which suggested ischemia was located in the posterior aspect of the left cortical parietal area. (Fig. 1). The biochemical, hematological, and vasculitic profiles were within normal limits. Duplex sonography of the carotid and vertebral arteries were also normal. No electrocardiographic (ECG) manifestations of dysrhythmia were present. Two- dimensional transthoracic echocardiography (ECHO) was performed and suspected hypokinesis was detected in the left ventricular wall. However, to investigate the possibility of a paradoxical embolism through a patent foramen ovale, trans- esophageal ECHO (TEE) was performed. TEE demonstrated that all closures are intact. Also suspected trabeculation was detected in the left ventricular wall by TEE. To further describe the probable myocardial disorder, cardiac MRI (cMRI) was performed. The cMRI demonstrated the presence of numerous, excessively prominent trabeculations and deep intertrabecular recesses in the mid and apical segments of the left ventricle (Fig. 2) with no thrombus formation, no peri- cardial thickening and no effusion. According to the findings of the cardiac MRI, a diagnosis of ILVNC was established. It was decided that the patient needed long-term anticoagulation treatment with warfarin to prevent any cardioembolic recur- rences. In addition, ECHO was performed on the patient’s first-degree relatives, with no findings of any abnormalities. After 10 days of warfarin treatment and physical therapy, the patient was discharged. Warfarin has been continued to maintain an international normalized ratio of between 2.0 and 2.5. His neurologic findings were almost completely resolved at the end of the 3-month follow-up period. DISCUSSION Cerebral embolism from cardiac sources is considered the cause of 15% to 45% of all strokes, and many studies suggest that it is more prevalent in patients younger than 45 years of age. 5,6 We describe the case of an ischemic stroke caused by isolated left ventricular noncompaction (ILVNC) in an adult male. ILVNC is a rare disorder of myocardial morphogenesis usually diagnosed at a pediatric age and associated with high mortality rates. 2 ILVNC is currently listed by the World Health Organization as an unclassified From the Departments of *Neurology and †Cardiology, Faculty of Medicine, Maltepe University, Istanbul, Turkey. Reprints: Sevki Sahin, MD, Baglarbasi Mah. Gumus Sk. No: 5/6, 34843 Maltepe/Istanbul, Turkey. E-mail: drsahin@gmail.com. Copyright © 2008 by Lippincott Williams & Wilkins ISSN: 1074-7931/08/1402-0125 DOI: 10.1097/NRL.0b013e3181618b08 Isolated left ventricular noncompaction is a rare congenital cardiomyopathy with prominent trabeculation and deep recesses of the left ventricular wall. The Neurologist • Volume 14, Number 2, March 2008 125