LAUSR

INTRODUCTION Echinococcosis is a chronic disease caused by Echinococcus species. The central nervous system (CNS) hydatidosis is still a major concern, especially in endemic countries, due to non-specific features and late diagnosis and treatment. This study aimed to provide a systematic review to elucidate the epidemiology and clinical characteristics of CNS hydatidosis worldwide over the past decades. EVIDENCE ACQUISITION PubMed, Scopus, EMBASE, Web of Science, Ovid, and Google Scholar were systematically searched. The gray literature and the references of included studies were searched as well. EVIDENCE SYNTHESIS Our results showed that the CNS hydatid cyst was more prevalent in the male gender, and it is known as a recurrent disease with a rate of 26.5%. CNS hydatidosis was more common in the supratentorial region and was also significantly common in developing countries, including Turkey and Iran. CONCLUSIONS It was demonstrated that the disease would be more prevalent in developing countries. Also, there would be a trend toward a male predominance of CNS hydatid cyst, younger age involvement, and the recurrence rate of 25% in general. There is no consensus about chemotherapy unless in recurrent disease and the patients who experienced cyst rupture intraoperatively, recommended for a wide range of 3 to 12 months.