Epidemiological, Diagnostic, and Clinical Features of Intracranial Cystic Echinococcosis: A Systematic Review.

Cystic Echinococcosis (CE) is a rare but serious parasitic disease caused by Echinococcus granulosus sensu lato, representing only 1-2% of all hydatid disease cases. Due to its nonspecific clinical presentation, its diagnosis and management pose significant challenges. This study aimed to provide a comprehensive overview of intracranial CE cases reported globally over the past 35 years, focusing on demographic characteristics, clinical presentation, diagnostic approaches, treatment modalities, and outcomes. METHODS: A systematic review was conducted in accordance with PRISMA guidelines and was registered in PROSPERO (CRD 42024608624). Relevant studies published between 1990 and 2024 were identified from PubMed, Scopus, and Web of Science databases. RESULTS: After screening and eligibility assessment, 392 studies involving 718 intracranial CE cases were included. The majority of patients were children (65%) and male (59.2%). The most frequent presenting symptoms were signs of increased intracranial pressure (79.4%), followed by motor deficits (37.9%) and visual disturbances (23.2%). Most cysts were located in the supratentorial region (88.9%), predominantly in the parietal lobe, and were solitary (88.4%). Surgical intervention was performed in 95.8% of cases, often combined with albendazole therapy. Complete recovery was observed in 85.5% of patients, while 8.7% died-primarily due to cyst rupture-related complications such as septicemia and anaphylaxis. Recurrence was reported in 26% of cases with follow-up. CONCLUSIONS: This review presents one of the most extensive analyses of intracranial CE to date. Despite being a rare manifestation, intracranial CE should be considered in the differential diagnosis of space-occupying brain lesions in endemic areas, particularly in paediatric patients.