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Lemierre's syndrome due to Fusobacterium necrophorum

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Summary

We present a case of a patient with Lemierre's syndrome caused by Fusobacterium necrophorum who developed a right frontal lobe brain abscess. We summarise the epidemiology, microbiology, pathogenesis, clinical presentation, diagnosis, complications, therapy, and outcomes of Lemierre's syndrome. F necrophorum is most commonly associated with Lemierre's syndrome: a septic thrombophlebitis of the internal jugular vein. Patients usually present with an exudative tonsillitis, sore throat, dysphagia, and unilateral neck pain. Diagnosis of septic thrombophlebitis is best confirmed by obtaining a CT scan of the neck with contrast. Complications of the disease include bacteraemia with septic abscesses to the lungs, joints, liver, peritoneum, kidneys, and brain. Treatment should include a prolonged course of intravenous beta-lactam antibiotic plus metronidazole.

Introduction

Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein (IJV) that typically begins with an oropharyngeal infection. Most cases are caused by fusobacterium, which are normal human microflora of the oropharynx, genitourinary tract, and gastrointestinal tract. The term postanginal sepsis is used interchangeably with Lemierre's syndrome, which was initially reported by Courmont and Cade in 19001, 2 but best described by André Lemierre in 1936.3, 4 Before the development of antibiotics, this syndrome was common and had a rapidly fatal course within 7–15 days.

We describe a case of Lemierre's syndrome caused by Fusobacterium necrophorum in a college student. He developed septicaemia and a brain abscess as a complication of his infection, which necessitated prolonged intravenous antibiotics. We review clinical features, outcomes, and other complications associated with Lemierre's syndrome.

Section snippets

Case presentation

The patient was an 18-year-old white male college student. He presented to the emergency department in December, 2008, with a sore throat and fever, was diagnosed with viral pharyngitis, and discharged home. He presented to the emergency department again 4 days later with a worsening sore throat, persistent fever, and malaise. A monospot test was negative and he was diagnosed with tonsillitis. At this time, he was placed on corticosteroids, azithromycin, and ibuprofen without relief of

Epidemiology

Lemierre's syndrome is a rare disease. A prospective study in Denmark detected an annual incidence of 3·6 cases of Lemierre's syndrome per million people from 1998 to 2001, with a substantially higher annual rate of 14·4 cases per million people aged 14–24 years.5

For unknown reasons, Lemierre's syndrome commonly occurs in young adults. A review of 38 cases published between 1974 and 1988 reported a mean age of those affected to be 20 years.6 Another review of 53 cases published in the 1990s

Microbiology

Anaerobic oropharyngeal infections comprise 1–5% of all anaerobic bacteraemias, with F necrophorum the most common anaerobe in sepsis originating from the oropharynx.14 A study by Batty and colleagues15 showed that 24 of 248 patients were positive for F necrophorum on throat swabs and isolation with the organism correlated with a clinical diagnosis of persistent sore throat syndrome. F necrophorum has also been associated with meningitis, endocarditis, sinusitis, abscesses, and appendicitis.6,

Pathogenesis

Primary infection in most cases of Lemierre's syndrome is associated with pharyngitis affecting the palatine tonsils or peritonsillar tissue.20, 21 Rare primary sources of infection are odontogenic infection, mastoiditis, otitis media, sinusitis, and parotitis.6, 22 Primary infection is followed by local invasion to the pharyngeal space and IJV causing septic thrombophlebitis, with a 1–3 week time interval.6, 9, 20

How F necrophorum causes septic thrombophlebitis is not known, although several

Clinical presentation

When originally describing this disease, Lemierre noted that “the appearance and repetition, several days after the onset of a sore-throat (and particularly of a tonsillar abscess) of severe pyrexial attacks and an initial rigor, or still more certainly the occurrence of pulmonary infarcts and arthritic manifestations, constitute a syndrome so characteristic that a mistake is almost impossible”.3, 6 Despite this classic description, diagnosis of Lemierre's syndrome is often missed until an

Diagnosis

The key to diagnosis of Lemierre's syndrome is a high clinical suspicion since physical examination might not be helpful in recognising the disease. During the early stages of the disease, persistently high fevers can be the only clue. Since F necrophorum is an anaerobic Gram-negative rod, cultures can take 5–8 days to grow delaying initiation of adequate antimicrobial treatment.6, 9 The following criteria are accepted as strong evidence for the presence of Lemierre's syndrome: anaerobic

Complications

Once infection has reached the IJV, haematogenous spread to other sites can occur causing various complications and ultimately death if antibiotics are delayed (panel), which is best illustrated by a series of patients in the preantibiotic era who all died within 12 days of disease onset.4 The lungs are most commonly affected in up to 85% of cases.14 Lung lesions commonly appear as necrotic cavitary lesions, but can also present as infiltrates, pleural effusions or empyema, lung abscesses,

Therapy

A multidisciplinary approach is necessary to treat patients with Lemierre's syndrome. Collaboration with microbiologists, radiologists, otolaryngologists, and thoracic surgeons is essential to reach a prompt diagnosis and achieve a good therapeutic outcome. Treatment involves use of appropriate antibiotics and, in many cases, surgical drainage. Anticoagulation should be considered in some cases.

No randomised clinical trials have been done to determine optimal antimicrobial therapy for patients

Conclusions

Patients presenting with Lemierre's syndrome tend to be young adults with conflicting data regarding the effect of gender. Once thrombosis of the IJV has occurred, pain, swelling, or indurations at the ipsilateral angle of the mandible of the neck extending along the sternocleidomastoid muscle can occur along with high fevers or trismus. Confirmation of IJV thrombosis is best made by a CT scan with contrast showing luminal filling defects, distension, and enhancement of the IJV with swelling of

Search strategy and selection criteria

A Medline search of the literature was done to identify relevant English language articles. The following search terms were used: “Lemierre's syndrome,” “Lemierre's disease,” “postanginal sepsis,”“necrobacilliosis,” “F necrophorum and CNS complications”, “Lemierre's syndrome and CNS complications”, and “Lemierre's syndrome and complications.” The references of retrieved articles were also reviewed to identify additional sources. Larger studies and case series were preferred over case reports.

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      There are also reports that the incidence of Lemierre's syndrome is increasing, which makes having it in the differential for necrotizing soft tissue infection an important consideration. This may be attributed both to decreased rates of antibiotic prescriptions in children, and decreased rates of tonsillectomies performed [3,4]. As was the case in our patient, the pathogen most commonly isolated in this syndrome is Fusobacterium necrophorum, an anaerobic, Gram-negative bacilli commonly found in the oropharynx.

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