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East Florida


HCA Florida Kendall Hospital


Internal Medicine

Document Type


Publication Date



Aicardi Syndrome, fever, bacterial infection, endocarditis


Bacterial Infections and Mycoses | Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Internal Medicine | Medicine and Health Sciences | Nervous System Diseases


INTRODUCTION Aicardi syndrome is a rare neurodevelopmental disorder predominantly affecting females and characterized by agenesis of the corpus callosum, infantile spasms, and distinctive chorioretinal lacunae. We present the case of a 24-year-old female with a known history of Aicardi syndrome who presented with fever of unknown origin, ultimately attributed to Staphylococcus Epidermidis infective endocarditis involving a native valve.

CASE DESCRIPTION A 24-year-old female with a history significant for Aicardi syndrome and epilepsy presented to the emergency department for fever during the past week; her primary care physician sent her for outpatient blood work a few days prior, including blood culture, which resulted in positive gram-positive coccus; otherwise the patient was acting at her baseline. On examination, the patient was bed-bound, non-verbal, and febrile, with poor dentition. Following admission, an exhaustive workup to determine the source of fever was initially inconclusive, including transthoracic echocardiography, a multidisciplinary approach involving cardiology, infectious disease specialists, and neurology was essential for optimizing patient care; ultimately, transesophageal echocardiography revealed vegetation on the mitral valve. Staphylococcus Epidermidis oxacillin resistant was reported in the final microbiology report. Once the patient started Vancomycin and the fever resolved, the patient was discharged to continue antibiotic therapy for six weeks.

DISCUSSION This case underscores the importance of considering rare presentations of infective endocarditis, even in individuals with complex neurodevelopmental conditions like Aicardi syndrome. Although Staphylococcus Epidermidis is known as an infrequent cause of native valve endocarditis (NVE), representing approximately 11% of all the microorganisms implicated lately, have emerged as an important cause of NVE in both community and healthcare settings and is associated with poor outcomes. Timely recognition, a comprehensive diagnostic approach, and collaborative care are crucial in achieving positive outcomes for patients with complex medical histories and emphasize the need for a multidisciplinary approach to diagnosis and management, ultimately leading to successful treatment and recovery.

Fever of Unknown Origin Secondary to Staphylococcus Epidermidis Infective Endocarditis in a Patient with Aicardi Syndrome



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