Fetal radiation exposure and moyamoya disease R. Kansal, A. Mahore, T. Nadkarni * , A. Goel Department of Neurosurgery, King Edward Memorial Hospital, Seth G.S. Medical College, Acharya Donde Marg, Parel, Mumbai 400 012, India article info Article history: Received 28 May 2009 Accepted 17 June 2009 Keywords: Fetus Moyamoya disease Pregnancy Radiotherapy abstract Moyamoya disease is a progressive occlusive disease of the cerebral vasculature with particular involve- ment of the circle of Willis. We present the first reported patient with moyamoya disease possibly due to radiation exposure during fetal development. A 10-year-old male, whose mother had undergone radio- therapy when pregnant, presented with moyamoya disease. The relevant literature is discussed. Ó 2009 Elsevier Ltd. All rights reserved. 1. Case report A 10-year-old male presented with acute onset right hemipare- sis. Routine hematological and thrombophilic profiles were nor- mal. A CT scan revealed a left internal capsule infarct. The patient underwent a cerebral angiogram, which demonstrated chronic occlusive arteriopathy involving medium-sized vessels typical of Moyamoya disease. In addition to the occlusion of both the supraclinoid internal carotid arteries there was abnormal for- mation of collateral vessels (Figs. 1 and 2). On further enquiry it was discovered that the child’s mother had suffered from breast carcinoma for which she had received radiotherapy during her sec- ond trimester of pregnancy. The patient showed gradual improve- ment in hemiparesis with physiotherapy. On follow-up at the end of 3 months, there was residual weakness of right grip but the child could walk unaided. A encephaloduroarteriosynangiosis procedure was recommended. 2. Discussion Moyamoya disease is a rare disorder of the blood vessels of the brain. It is characterized by a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the medium-sized feeding arteries. The changes affect the major blood vessels of the brain including the anterior, middle, and posterior cerebral arteries. The term ‘‘moyamoya” is the Japa- nese word for ‘‘puff of smoke”. This distinctive name describes the angiographic appearance of the abnormal vascular collateral net- works that develop adjacent to the stenotic vessels. The symptoms and clinical course vary widely and depend upon the location and severity of infarction or hemorrhage. The steno- occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. Thus, signs and symptoms may range from transient events to severe permanent neurologic defi- cits. In younger patients, the initial symptoms are usually ischemic attacks with or without infarction, whereas older patients tend to develop intraventricular or intracerebral hemorrhage. Fragile, en- gorged basal moyamoya vessels with or without small intraparen- chymal aneurysms near the lateral ventricle are thought to be responsible for the hemorrhages. The exact etiology of moyamoya disease is unknown. The dis- ease may be hereditary and multifactorial. Some genetic predispo- sition is apparent because it is familial in 10% of patients. Because there was no family history of such illness for our patient, his moyamoya disease may be either idiopathic or caused by the ef- fects of radiotherapy. Radiation may contribute to degeneration of the internal elastic tissue and degradation of the vascular wall. Fig. 1. Lateral views of (A) right carotid angiogram and (B) left carotid angiogram showing narrowing of the petrous and cavernous internal carotid artery. The carotid artery is totally occluded after the origin of the ophthalmic artery. The collateral vessels are noted around the ophthalmic artery. * Corresponding author. Tel.: +91 22 24129884; fax: +91 22 24143435. E-mail address: tdnadkarni@hotmail.com (T. Nadkarni). 406 Case Reports / Journal of Clinical Neuroscience 17 (2010) 406–407