Lemierre's syndrome, or postanginal sepsis, is the most common life-threatening manifestation. Tonsillitis is followed by septic thrombophlebitis of the internal jugular vein and then a septicemia with septic emboli in lungs and other sites. Recent evidence suggests that F. necrophorum can be limited to the throat and cause persistent or recurrent tonsillitis. F. necrophorum is unique among non-spore-forming anaerobes, first for its virulence and association with Lemierre's syndrome as a monomicrobial infection and second because it seems probable that it is an exogenously acquired infection. The source of infection is unclear; suggestions include acquisition from animals or human-to-human transmission. Approximately 10% of published cases are associated with infectious mononucleosis, which may facilitate invasion. Recent work suggests that underlying thrombophilia may predispose to internal jugular vein thrombophlebitis. Lemierre's syndrome was relatively common in the preantibiotic era but seemed to virtually disappear with widespread use of antibiotics for upper respiratory tract infection. In the last 15 years there has been a rise in incidence, possibly related to restriction in antibiotic use for sore throat. [3]
Most reviewers now regard the paper by Courmont and Cade in 1900 as the first description of Lemierre's syndrome, i.e., a human postanginal septicemic infection with F. necrophorum. The patient complained of cough and sore throat, and a few days later, when the throat had settled, had sudden onset of rigors and progressed to an overwhelming sepsis, likened to plague because of the presence of a large abscess in the supraclavicular fossa. The autopsy showed multiple lung abscesses which were thought to be septic embolic infarcts. [3]
Case series suggest that there may be an association between infectious mononnucleosis (IM) and subsequent Lemierre's disease. As an example, in one series that evaluated five patients diagnosed with Lemierre’s disease over a six-year period, three had evidence of acute EBV infection, including one related to Fusobacterium necrophorum. However, in a larger series that evaluated 23 patients with Lemierre's syndrome related to F. necrophorum, only one patient had evidence of concomitant mononucleosis. In that series, another patient who died from Lemierre's syndrome had a high number of EBV copies in the CSF and serum; however, it was unclear if the patient had acute EBV since anti-EBV antibodies were not obtained. [2]
Of the five patients diagnosed with Lemierre's syndrome, two had concomitant acute infection with Epstein-Barr virus. Additionally, a 19-year-old adolescent was admitted during this period with acute infectious mononucleosis, Fusobacterium necrophorum sepsis, sinusitis, frontal lobe abscess and ophthalmic vein thrombosis. The clinical presentation of all patients included fever, sore throat, and ear or neck pain. The duration of symptoms ranged from two days to three weeks prior to admission. The patients with acute Epstein-Barr virus infection had been diagnosed with infectious mononucleosis prior to admission, and tested positive for heterophile antibody. These patients subsequently underwent more extensive in-patient treatment, including intensive care management and ventilator support. The patients who tested negative for heterophile antibody experienced a milder course of illness, with a shorter duration of in-patient management. [1]
[1] Chacko EM, Krilov LR, Patten W, Lee PJ. Lemierre's and Lemierre's-like syndromes in association with infectious mononucleosis. J Laryngol Otol. 2010 Dec;124(12):1257-62. doi: 10.1017/S0022215110001568. Epub 2010 Jul 6. PMID: 20602850.
[2] J Sullivan. Clinical manifestations and treatment of Epstein-Barr virus infection. Uptodate
[3] Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre's syndrome. Clin Microbiol Rev. 2007 Oct;20(4):622-59. doi: 10.1128/CMR.00011-07. PMID: 17934077; PMCID: PMC2176048.