A 52-year-old man, with a history of active smoking and alcohol abuse, was admitted to hospital for a febrile coma. He had complained of headaches with fever and chills for a few days before admission. Behavioral disorders and seizures were observed. The clinical examination revealed bilateral miosis and a stiff neck. Status epilepticus required intubation and mechanical ventilation. The cerebrospinal fluid (CSF) was purulent with pleocytosis (9100 cells/mm3, neutrophils 98%), elevated CSF protein (3.51 g/L), hypoglycorrhachia (1.17 mmol/L; CSF/serum glucose ratio: 0.13), and numerous gram-positive diplococci. Cerebrospinal fluid and blood cultures grewS. pneumoniae. Results from whole-body computed tomodensitometry scan were normal. Cefotaxime was started, and prompt improvement led to extubation on the second day. Fever disappeared and inflammatory parameters declined. Two days later, fever recurred and a typical murmur of aortic valve regurgitation appeared. Transthoracic echocardiography showed aortic endocarditis, with a 6-mm vegetation on the right coronary cusp and severe aortic regurgitation. Gentamicin was added. The occurrence of congestive heart failure required aortic valve replacement with a mechanical prosthesis. No immune deficiency was found. Antibiotics were discontinued after 6 weeks, no relapse occurred, and the patient recovered fully.
Discussion
Pneumococcal infections can involve several organs. The association of pneumonia, meningitis, and endocarditis is called Austrian syndrome, first described by Osler in 1881. This syndrome is more frequent in alcoholic, middle-aged patients.
Pneumonia was absent in our case, although pleural effusion was observed on the computed tomography scan. Neurologic symptoms preceded cardiac signs, suggesting that bacterial adherence to the aortic valve may have occurred through bacteremia of neurologic origin. We can also hypothesize that endocarditis may have initially been present and transmitted septic meningeal emboli.
In Austrian syndrome, infection starts with pneumonia in more than 80% of cases, and endocarditis is present at the time of diagnosis in 60% of cases. The aortic valve is involved in 75% of cases, frequently associated with rapid valvular destruction. Alcoholism is a risk factor and may act by directly inhibiting immunity.
Pneumococcal endocarditis cases are rare but are associated with concomitant meningitis in 40%-76% of cases. Conversely, 7.5% of pneumococcal meningitis cases are associated with endocarditis. Diagnosis of endocarditis can be delayed, with a median time of 7-16 days. Mortality rates still remain high despite antimicrobial agents, up to 63%, as a result of frequent systemic complications.
Antimicrobial treatments should be initiated as soon as possible and can require surgical intervention, especially if the aortic valve is involved. Early surgery has shown superior results and seems to be associated with decreased mortality. Antibiotic treatment is administered for 4 weeks and combines β-lactam antibiotics with gentamicin.
Conclusion
This case underlines the importance of careful, repeated patient examination in S. pneumoniae infections. Screening for endocarditis should take place when pneumococcal meningitis or pneumonia occurs, especially in middle-aged men with a history of alcohol abuse.
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-Samuel Deshayes, MDcorrespondenceemail, Hubert de Boysson, MD, MSc, Arnaud Salmon-Rousseau, MD, Aurélie Baldolli, MD, Christophe Auzary, MD, Loïk Geffray, MD
This article originally appeared in the March 2016 issue of The American Journal of Medicine.
