Netherlands Journal of Critical Care

Copyright © 2011, Nederlandse Vereniging voor Intensive Care. All Rights Reserved. Received April 2010; accepted July 2010 Case Report

A patient with the Austrian syndrome

RJ de Haas1,2, J Kruik3, AEL van Golde4, TF Fraatz1, B Mulder5, ThF Veneman1,6

1 Department of Intensive Care Medicine, Twenteborg Hospital Almelo, The Netherlands 2 Department of Surgery, Twenteborg Hospital Almelo, The Netherlands 3 Department of Cardiology, Twenteborg Hospital Almelo, The Netherlands 4 Department of Neurology, Twenteborg Hospital Almelo, The Netherlands 5 Laboratory for Medical Microbiology, Enschede, The Netherlands 6 Department of Internal Medicine, Twenteborg Hospital Almelo, The Netherlands

Abstract - Background: The combination of endocarditis, and caused by , currently known as the Austrian syndrome, is a rare condition with a high mortality rate. Case: We present the case of a 44-year-old man with a history of abuse who was recently treated with antibiotics for otitis media. The patient was admitted to our Intensive Care Unit with an impaired level of consciousness, respiratory insufficiency and sepsis. A pneumonia was diagnosed radiologically and molecular analysis of cerebrospinal fluid was positive for Streptococcus pneumoniae which necessitated treatment with intravenous antibiotics. Echocardiography showed a large mitral valve vegetation with severe mitral regurgitation. After two days on the ICU, due to increasing congestive heart failure, a mitral valve replacement was necessary. Unfortunately, our patient died four weeks post-admission due to a major myocardial infarction. Conclusion: The present case underlines the need to consider the possibility of endocarditis with potential valve damage in every patient with pneumococcal meningitis or bacteremia, particularly in those with a history of chronic and in patients who are immunocompromised. By combining antibiotic treatment with valve replacement at an early stage, an improve- ment in mortality rates might be observed.

Keywords - Austrian syndrome, Streptococcus pneumoniae, meningitis, endocarditis

Introduction unknown cause. The patient had a history of alcohol abuse and Pneumococcal endocarditis as well as pneumonia and meningitis hypertension, and had been treated by his general practitioner that are caused by Streptococcus pneumoniae are potentially with amoxicillin in a dose of 500 mg twice daily during the 2 life-threatening diseases. The combination of these clinical weeks before presentation because of otitis media. problems is a rare condition, first described in 1957 by Robert Austrian and currently known as the Austrian syndrome [1]. This syndrome appears to be more prevalent in alcoholic patients. In Figure 1. Transthoracic echocardiography showing a large addition, intravenous drug use has also been reported as a risk mitral valve vegetation (arrow). Abbreviations: LV = left ventricle; factor for the development of this syndrome [2,3]. The rarity of LA = left atrium. this syndrome might, among others, be related to the current use of effective antibiotics. Nevertheless, it still occurs, and the mortality rate remains high [4]. In this case report we describe the presentation, pathogenesis, prognosis and therapeutic options for this condition.

Case report A 44-year-old man was admitted from his home to the Intensive Care Unit of the Twenteborg Hospital with an impaired level of consciousness, respiratory insufficiency and sepsis of

Correspondence

ThF Veneman E-mail: [email protected]

84 NETH J CRIT CARE - VOLUME 15 - NO 2 - APRIL 2011 Netherlands Journal of Critical Care A patient with the Austrian syndrome

At physical examination the patient was confused and restless, mg per day) was started and maintained for 7 days. Subsequently with a Glasgow Coma Scale score of 10. No additional blood cultures were positive for Streptococcus pneumoniae. meningeal signs were present and cardiac auscultation revealed Therefore, antibiotic treatment was switched to 12 no abnormalities. The initial laboratory results of a venous and million U/day intravenously. Because of increasing congestive arterial blood sample are shown in Table 1. heart failure and ongoing deterioration of renal and liver function, A chest radiograph showed a left lower lobe consolidation. The the mitral valve was replaced with a mechanical valve on day 2 patient was intubated and mechanically ventilated, and inotropic post-admission. The postoperative course was complicated by agents were started for haemodynamic support. Because renal insufficiency which necessitated haemodialysis. Further, an electrocardiogram showed a suspected diffuse cardiac blood cultures then turned positive for Candida albicans which ischaemia, transthoracic echocardiography was performed, was treated with intravenous fluconazole. Unfortunately, our which showed a large mitral valve vegetation with severe mitral patient died 4 weeks post-admission due to a major myocardial insufficiency (Figure 1). Repeated electrocardiograms on days 1 infarction. and 2 post-admission showed disappearance of ischemia, and therefore, the observed electrocardiographic abnormalities were Discussion most probably due to sepsis. In addition, a lumbar puncture Streptococcus pneumoniae is a relatively rare cause of endo­ was performed with analysis of the cerebrospinal fluid (CSF) as carditis. Risk groups are immunocompromised patients and follows: leukocyte count 6360/μL, glucose concentration 1.7 alcoholics [5,6,7]. The patient described in this case report had mmol/L with blood glucose of 11.4 mmol/L, and Gram-positive been suffering from severe otitis media, which is a well known cocci in pairs on Gram stain. Molecular analysis was positive factor in the aetiology of meningitis and was initially treated by for Streptococcus pneumoniae. A computed tomography (CT) the GP with amoxicillin. Streptococcus pneumoniae is also scan of the brain showed 2 ischaemic appearing lesions in the the most frequent aetiological microbial agent of community occipital and right temporal lobe. acquired bacterial meningitis in adults [8,9]. Moreover, our patient Empirical intravenous antibiotic treatment was started, which had a history of chronic , which rendered him more consisted of ceftriaxon, flucloxacillin, and one dose of gentamycin. susceptible to S. pneumoniae. In addition, treatment with high-dose steroids (hydrocortisone 300

Table 1. Laboratory results on admission.

Laboratory test Value Reference value

Venous blood sample Leucocyte count 26.0 × 109/L 4.0-10.0 × 109/L C-reactive protein 202 mg/L 0-10 mg/L Thrombocytes 228 × 109/L 150-400 × 109/L Troponin-I 1.69 μg/L 0.00-0.01 μg/L pro-BNP 2030 pmol/L 0-12 pmol/L Creatinin 171 μmol/L 65-105 μmol/L Urea 21.2 mmol/L 2.5-6.4 mmol/L Natrium 133 mmol/L 136-146 mmol/L Potassium 2.7 mmol/L 3.5-5.0 mmol/L Alanine aminotransferase (ALT) 100 U/L 0-45 U/L Aspartate aminotransferase (AST) 117 U/L 0-40 U/L Gamma-glutamyl transferase (GGT) 49 U/L 0-55 U/L Alkaline phosphatase (ALP) 78 U/L 35-120 U/L Lactate 2.4 mmol/L 0.5-2.5 mmol/L Arterial blood sample* pH 7.31 7.35-7.45 PaCO2 4.6 kPa 4.5-6.0 kPa PaO2 9.3 kPa 11.1-14.4 kPa Bicarbonate 16.9 mmol/L 21.0-28.0 mmol/L Base excess -8.0 mmol/L -4.0-3.0 mmol/L

* FiO2 35%

NETH J CRIT CARE - VOLUME 15 - NO 2 - APRIL 2011 85 Netherlands Journal of Critical Care RJ de Haas, J Kruik, AEL van Golde, TF Fraatz, B Mulder, ThF Veneman

A review of the scarce literature on the Austrian syndrome Early recognition of Austrian syndrome is needed in order shows that it generally occurs in alcoholic men with native to be able to take therapeutic measures at an early stage. infectious endocarditis. Other common risk factors for invasive Echocardiography plays an important role both in confirming the pneumococcal infections are human immunodeficiency virus diagnosis and assessing the valve status in order to determine (HIV) infection, , connective tissue disease, the need for surgical intervention in infective endocarditis. The steroid use, diabetes mellitus, and intravenous drug use [5]. combination of a prolonged course of antibiotics and early valve The majority of patients suffer from severe heart disease, in replacement results in a lower mortality rate compared with particular, congestive heart failure due to valve dysfunction that that observed after antibiotic treatment alone [6]. Therefore, requires valve replacement. The aetiology of the ischaemic brain therapy should be focused on the treatment of sepsis with lesions observed on the CT scan of the brain in this patient is antibiotic therapy and high-dose steroids, combined with not clear, although it cannot be excluded that they are caused early valve replacement in cases of major valve damage. Early by emboli originating from the mitral valve endocarditis. The treatment with high-dose steroids has been shown to improve mortality rate for this condition is high, ranging between 19% the outcome in adults with acute bacterial meningitis [9]. Of and 63% [6,7,10,11]. The prognosis depends, amongst other note, only observational data are available that report on the factors, on how soon the condition is recognised. Obviously, outcome following antibiotic treatment combined with early valve in this patient, the development of a Candida sepsis worsened replacement, which might introduce some selection bias. the prognosis. The aetiology of the Candida septicaemia was The present case report underlines the need to consider not clear. However, a number of risk factors were present, the possibility of endocarditis with potential valve damage in such as the presence of an immunocompromised state due to every patient with pneumococcal meningitis or bacteremia, chronic alcoholism, administration of total parenteral nutrition, particularly in those with a history of chronic alcohol abuse and treatment with high-dose steroids. Because our patient and in immunocompromised patients. Early recognition is died from a major myocardial infarction, it would have been crucial for the optimization of treatment results. By combining interesting to know the degree of coronary artery disease before antibiotic treatment with valve replacement at an early stage, an valve replacement as this procedure could have been combined improvement in mortality rates might be observed. with a coronary artery bypass graft. However, because of rapid deterioration in the patient, due to significant congestive heart Conflicts of interest failure, our cardiac surgeon decided to treat the patient’s most No financial or ethical conflicts of interest were encountered for lethal problem, namely mitral valve insufficiency, to avoid a too all authors. extensive surgical procedure.

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