A Forgotten Disease

1
A Forgotten Disease

• A Case Study about Lemierres Disease
• by Brandy Harkins

2
Patient Presentation

• 20 year old female
• Diagnosed with infectious mononucleosis 2 days
  prior to admission
• No remarkable previous medical history
• Blood pressure 101/72
• Pulse 167 beats/min
• Respiratory rate 52/min

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Presentation continued

• Shortness of breath and chest pain with shallow
  breathing
• Sore throat
• Headache
• Fever
• Decreased appetite
• Abdominal pain (no nausea, vomiting, diarrhea or
  constipation)
• Pale
• Initial diagnosis was pneumonia

4
Laboratory Findings

• Blood culture positive at 24hrs (Fusobacterium
  necrophorum)
• Monospot negative
• EBV-VCA IgG positive
• Increased fibrinogen, PT PTT
• Increased bilirubin
• Liver enzymes AST 74 (19-45), ALT 44 (8-37)
• WBCs 15.3 (4.0-10.9)
• Plts 106 (150-400)

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Fusobacterium necrophorum

• Normal flora in oral cavity, female genital
  tract, and gastrointestinal tract
• Pleomorphic gram negative bacillus (GNB)
• Non-motile
• Non-spore forming
• Strict anaerobe

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Disease Association

• Can cause parotitis, otitis media, sinusitis,
  odontogenic infection, mastoiditis and Lemierres
  syndrome (necrobacillosis)
• Produces lipopolysaccharide endotoxin,
  hemagglutinin, leukocidin, and hemolysin
• Invasion usually from intra-oral disease
  (bacterial tonsillitis, EBV, dental disease)

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Questions to Consider

• What organism is usually responsible for
  Lemierres sydrome?
• Why has Lemierres become the forgotten
  disease?
• What are the symptoms of the syndrome?
• What age group is most commonly affected?
• What are the stages commonly seen with Lemierres
  and at which stage does the red flag appear?

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Lemierres Syndrome

• Thrombophlebitis of the internal jugular vein
  (IJV) due to anaerobic infection (usually F.
  necrophorum)
• Virulent toxin production with platelet
  aggregation ? IJV thrombosis
• Causes severe disease as primary pathogen in
  healthy individuals
• Generally affects young adults 16-29 y/o
• 1 in 1,000,000 infected per year
• Common in the early 20th century, but disappeared
  with antibiotics
• Used to have 100 mortality ratetodays rate is
  6-20

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Disease Presentation

• Sore throat
• Tender/swollen lymph nodes
• Prolonged fever
• May experience abdominal pain, nausea or
  vomitting
• Bacteremia
• Increased WBCs or left shift
• Hyperbilirubinemia and slight increase in liver
  enzymes

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Classical Characterization

• Primary infection in oropharynx
• Septicemia documented by at least one positive
  blood culture bottle
• Evidence of internal jugular vein thrombosis
• At least one metastatic focus (usually pulmonary)

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Stages

• Patient generally exhibits three stages
• 1. Pharyngitis sore throat (lt 1 week) ?
• 2. Local invasion of lateral pharyngeal
  space and IJV septic thrombophlebitis ?
  swollen/tender neck red flag
• 3. Metastatic complications fever,
  pulmonary infiltrates or possible joint
  involvement

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Treatment

• Fatal if untreated
• 1-2 weeks IV antibiotics and 2-4 weeks oral
  antibiotics
• Aggressive approach when patient has pharyngitis
  and tender/swollen neck
• Get blood culture
• Look for evidence of IJV thrombophlebitis with
  CT, MRI, ultrasound
• Use antibiotics affective against anaerobes
  (clindamycin, metronidazole, etc.)
• Anticoagulant therapy controversial
• May require surgery to remove the IJV because of
  continuing sepsis, localized collection of pus,
  or embolism

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So whys it so hard to diagnose?

• Rarely seen in the antibiotic-eramost physicians
  have never seen it
• Can present with pneumonia-like or
  meningitis-like clinical picture
• Many sore throats have a viral etiology and are
  not treated with antiobiotics, therefore a
  patient can be misdiagnosed and untreated for
  long periods of time before clinicians suspect
  Lemierres
• More severe with longer duration of symptoms than
  viral sore throat!

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Summary

• Lemierres syndrome is usually caused by
  Fusobacterium necrophorum
• Affects healthy young adults
• Patient presents with fever, sore throat,
  swollen/tender neck (red flag)
• 3 stages pharyngitis, IJV thrombosis, and
  metastatic complications
• Disease severity is often underestimated and left
  untreated or is treated as a case of pneumonia or
  meningitis

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References

• 1. Chirinos J et al. The evolution of Lemierres
  syndrome report of 2 cases and review of the
  literature. Medicine. 200281458.
• 2. Deadly sore throat ailment on the rise in UK.
  Clinical Infectious Diseases. 2002351.
• 3. Harrisons Online. www.harrisons.accessmedicin
  e.com
• 4. Moore B, Dekle C, Werkhaven J. Bilateral
  Lemierres syndrome a case report and literature
  review. Ear, Nose and Throat Journal.
  200281234.
• 5. Singhal A, Morris D. Lemierres syndrome.
  Southern Medical Journal. 200194886.
• 6. Woywodt A et al. A swollen neck. The Lancet.
  20023601838.

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Credits

• This case study was created by
  
  Brandy Harkins, MT(ASCP) while she was a Medical
  Technology student in the 2004 Medical Technology
  Class at William Beaumont Hospital, Royal Oak, MI.