More than a fever: Encephalitis due to typhoid fever

Worldwide, 10.9 million cases of typhoid fever occur every year, with 116,000 deaths. The largest burden of disease occurs in Africa and Asia but imported cases in travelers, including those visiting friends and relatives, are common. Although the majority of cases are uncomplicated and respond well to antibiotics alone, severe disease may occur, particularly, in incompletely or untreated cases. Mortality is particularly common where complications, including shock, intestinal perforation, or neurologic involvement, occur. A case of typhoid fever with severe neurologic involvement resulting in coma, seizures, and prolonged disability is presented. Despite prompt initiation of appropriate empiric antibiotics, neurologic deterioration occurred, with a prolonged requirement for mechanical ventilation, severe cerebral edema, and disability. The role for adjunctive corticosteroids and data supporting these therapies and the uncertainty around optimal dose and duration are reviewed. The pathophysiology of neurologic involvement in typhoid fever, with a focus on recent data from animal models, is presented.