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 Türkiye 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.