RESUMEN
BACKGROUND: No universal protocol exists for treating cerebral abscesses in Down syndrome. An illustrative case supplemented with a systematic literature review on brain abscesses in Down syndrome is presented, comprising a total of 16 cases. Preoperative infectious disease workups, cardiac examinations including echocardiography, as well as reported surgical and antibiotic treatments were correlated in the reported cohorts. OBSERVATIONS: Overall, 18.8% of cases (n = 3) had no reported cardiac evaluation. The majority of cases were treated surgically (n = 8), with aspiration (n = 3), drainage (n = 2), or other operations (n = 3); 25% (n = 4) were treated with antibiotics only. Strikingly, 25% of cases (n = 4) reported neither surgical nor antibiotic therapy, a significantly higher rate compared to 0%-3% of patients with brain abscess in other reported cohorts. Half of the patients (n = 8) who died either lacked a cardiac evaluation or had existing heart conditions. This mortality rate was about 4 times higher than the rates observed in other studies. LESSONS: Down syndrome patients with cerebral abscess have a high morbidity rate, mainly due to cardiac disease. Therefore, early diagnostic workup, including echocardiography, allows proactive management with an improved outcome. https://thejns.org/doi/10.3171/CASE23394.