272 Scientific Programme 22. European Stroke Conference © 2013 S. Karger AG, Basel 10 Interesting and challenging cases Recurrent hemodynamic ischemic attacks as adult manifestation of the PHACES syndrome R. Werner1, T.E. Emmerich2, R.P. Kunz3, I. Kureck4, C.A.S. Herber5, J.C. Wöhrle6 Katholisches Klinikum Koblenz-Montabaur, Koblenz, GERMANY1, Katholisches Klinikum Koblenz-Montabaur, Koblenz, GERMANY2, Radiologisches Institut Dr. von Essen, Koblenz, GER-MANY3, Radiologisches Institut Dr. von Essen, Koblenz, GERMANY4, Katholisches Klinikum Koblenz-Montabaur, Koblenz, GERMANY5, Katholisches Klinikum Koblenz-Montabaur, Koblenz, GERMANY6 Background: The PHACES syndrome is a rare cause of juvenile stroke and usually diagnosed in ear-ly childhood. It specifies a neurocutaneous disorder of unknown etiology that predominantly affects girls. The acronym stands for Posterior fossa and other brain malformations, facial Hemangiomas, Arterial cerebrovascular anomalies, Cardiac and aortic abnormalities, Eye anomalies, and Sternal defects. Most patients do not express the full phenotype. Case report: A 21-year-old female was admitted after two episodes of sudden weakness of both legs and alien limb syndrome with involuntary movements of the right arm. Since childhood, she complained of “migraine” with strictly left-sided headache and preceding sensory and visual auras as well as isolated visual auras. Her father, the father’s sister and the aunt’s daughter were reported to have “similar attacks”. Duplex ultrasound showed a narrowing of the left internal carotid artery (ICA) and a decreased flow velocity of the left middle cerebral artery (MCA). Magnetic resonance imaging displayed Moyamoya-like flow voids at the level of the circle of Willis but no acute or old-er cerebral infarction. After having ruled out other causes of stroke, and with the extended history of a fusion anomaly of the sternum and several strabismus operations in early childhood, we diagnosed a possible PHACES syndrome with ocular abnormalities, sternal cleft and ICA hypoplasia leading to hemodynamic ischemic attacks in the territory of the left MCA. We started a medication with as-pirin. In case of further attacks, an extra-intracranial arterial bypass has to be considered analogous to the treatment concepts of Moyamoya disease. Conclusion: Adult patients with cryptogenic stroke and hypo- or aplasia of large cerebral arteries should be carefully checked for other developmental anomalies, as the most prominent feature of the PHACES syndrome, the facial hemangioma, might be missing and is not mandatory for the diagno-sis. 9 Interesting and challenging cases Ischemic stroke as the first manifestation of hepatic epithelioid hemangioendothelioma P. Zis1, M. Mpalomenaki2, A. Assi3, D. Kravaritis4, V.A. Sevastianos5 Department of Neurology, Evangelismos General Hospital, Athens, GREECE1, 4th Department of Internal Medicine, Evangelismos General Hospital, Athens, GREECE2, 4th Department of Inter-nal Medicine, Evangelismos General Hospital, , 3, Department of Neurology, Evangelismos General Hospital, Athens, GREECE4, 4th Department of Internal Medicine, Evangelismos General Hospital, Athens, GREECE5 Background Cryptogenic stroke accounts for 30 to 40 percent of ischemic strokes in most modern stroke regis-tries and databases. Identifying a unifying theory and mechanism for cryptogenic stroke is likely to be difficult, if not impossible. Cancer-related mechanisms have been associated with cryptogenic stroke. Hepatic epithelioid hemangioendothelioma is an uncommon neoplasm of vascular origin first de-scribed in 1982. Right upper quadrant abdominal pain and abdominal discomfort are the most com-mon symptoms at presentation. Case A 38-year-old obese woman, with a past medical history of cholecystectomy and dyslipidaemia, pre-sented with acute occipital headache, vomiting and rotational vertigo which lasted 8 hours. On admission neurological examination was unremarkable, however general physical examination revealed hepatomegaly. Routine blood tests showed elevated abnormal liver function tests. Brain CT and MRI scans indicated an acute ischemic infarct in the right cerebellum. Further investigation, which included carotid and vertebral artery doppler ultrasound, 24-hour holter monito, magnetic resonance angiography of the brain, lumbar puncture and screening for collagen related diseases, was negative. Transesophageal heart ultrasound did not reveal the presence of a left heart thrombus. Moreover, antithrombin III, factor V Leiden, PCGlob-FVNR and protein C and S levels were normal. However, liver MRI revealed multiple lesions in both liver lobes. Fine needle aspiration, histologi-cal examination and immunohistochemistry confirmed the diagnosis of hepatic hemangioendothelio-ma. Conclusion We report for a first time a case of a young patient presenting with an ischemic stroke of the cerebel-lum as the sole manifestation of a hepatic hemangioendothelioma. Thorough physical examination in addition to neurological is extremely important in stroke patients.
Karger_ESC London_2013
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