J Neurosurg 65:115-119,1986 Alveolar hydatid disease of the brain Report of four cases YUNUS AYDIN, M.D., ORHAN BARLAS, M.D., CO~KUN YOLA~, M.D., ]SMAiL HAKKI AYDIN, M.D., ADNAN CEViZ, M.D., ARiF ALADAG, M.D., DURKAYA OREN, M.D., AND DURSUN AKDEMiR, M.D. Departments of Neurosurgery and General Surgery, School of Medicine, Atatftrk University, Erzurum, and Department of Neurosurgery, Istanbul School of Medicine, University of lstanbul, ~apa, Istanbul, Turkey ~" Four cases of alveolar hydatid disease of the brain encountered within 27 months in eastern Turkey are reported. All of the patients were male farmers who presented with signs of cerebral tumor. Two of the patients were shown to harbor hepatic lesions and one of them had pulmonary metastases. The cerebral lesions were removed in toto and neurological recovery was obtained in all four patients. A review of the literature revealed only five previously reported cases treated surgically. It is concluded that cerebral Echinococcus multilocularis lesions are amenable to surgery, and that their removal provides useful prolongation of life despite the presence of hepatic or pulmonary disease. KEY WORDS 9 alveolar hydatid disease 9 cerebral echinococcosis 9 Echinococcus alveolaris 9 Echinococcus multilocularis N EUROSURGEONS are rarely called upon to treat alveolar hydatid disease. One of the most dan- gerous of human infections, alveolar hydatid disease is caused by Echinococcus multilocularis, also known as Echinococcus alveolaris. This organism is a parasite known in northern regions including Alaska, Canada, Siberia, and the Alps, ~'~2 but it has recently been increasingly observed in the more temperate re- gions of eastern France, Nebraska, and Illinois. 2'22 Un- like Echinococcus granulosus, which produces cystic lesions, E. multilocularis produces invasive lesions of firm consistency, full of connective tissue and a jelly- like material. The favorite location of these destructive cysts is the liver; cerebral localization is rare. We report four cases of alveolar hydatid disease of the brain that were surgically treated at AtatiJrk Uni- versity Medical School in Erzurum, Turkey, during a period of 27 months. In each case, the cerebral lesion was removed in toto and remission of neurological deficits was obtained. The purpose of this paper is to describe the clinical picture and surgical therapy of this infestation and to alert neurosurgeons and pathologists, particularly those in regions south of known endemic areas of E. multilocularis, to the possibility of encoun- tering such lesions. Cerebral E. multilocularis lesions lend themselves to total removal with useful prolonga- tion of life in spite of hepatic involvement. Case Reports Case 1 This 55-year-old man was admitted on September 29, 1981, with a 4-month history of progressive head- aches and left-sided weakness. His medical history and general physical examination were unremarkable. Neu- rological examination revealed a mild left hemiparesis with increased deep-tendon reflexes, and a positive left Babinski response. Skull x-ray films were normal. Erythrocyte sedimentation rate (ESR) was 82 mm/hr. Weinberg and Casoni tests were positive. Right carotid arteriography demonstrated a mass in the posterior parietal region. On October 11, 1981, a right parietal craniotomy was performed. When the dura was opened, the cerebral convolutions were seen to be strikingly flattened and widened. A firm multilobulated mass, 7 cm in diameter, was found located subcortically and was totally re- moved (Fig. 1). Histological examination of the speci- J. Neurosurg. / Volume 65 ~July, 1986 115