"A forgotten disease": a case of Lemierre syndrome

Lemierre's syndrome is a rare but a life threatening condition which affects young healthy individuals, was first described by Dr.Andre Lemierre in 1936. Incidence rates are between 0.6 and 2.3 per million population. It is found more commonly in males, with a male to female ratio of approximately 2:1. Its pathogenesis consists of the development of infectious thrombophlebitis in the internal jugular vein or one of its branches caused by a focal sepsis, mostly localized in the oropharynx, leading to generalized multiorgan metastatic infections, generally to the lung. This computerized tomography (CT) neck with intravenous contrast is from a 24 year old female who presented with a two day history of fever, hypotension and respiratory failure. The physical exam was positive for diminished breath sounds bilaterally on lung exam. Complete blood count revealed a leukocytosis of 16,200 u/L with 70% neutrophils and 9% bands, hemoglobin of 13.4mg/dl and severe thrombocytopenia with a platelet count of 34,000 u/L; comprehensive metabolic panel revealed sodium 140mmol/L, potassium 2.9mmol/L, bicarbonate 26mmol/L, blood urea nitrogen (BUN) 16mg/dl, creatinine 0.8mg/dl, calcium 7.2 mg/dl, albumin 2.4g/dl, total bilurubin 3.1mg/dl, AST 81 U/L, ALK 101 U/L, ALT 35U/L. CT chest revealed multiple cavitary opacities in both lungs. Blood cultures were positive for Fusobacterium necrophorum. CT scan neck showed a filling defect of the right internal jugular vein consistent with a thrombus and multiple enlarged cervical lymph nodes. Treatment is medical with intravenous antibiotics and anticoagulation. References: 1. Carlson ER, Bergamo DF, Coccia CT. Lemierre's syndrome: two cases of a forgotten disease. J Oral Maxillofac Surg 1994; 52:74-78. 2. Moore-Gillon J, Lee TH, Eykyn SJ, Phillips I. Necrobacillosis: a forgotten disease. BMJ 1984;288:1526-1527. 3. Jones C, Siva TM, Seymour FK, O'Reilly BJ. Lemierre's syndrome presenting with peritonsillar abscess and VIth cranial nerve palsy. J Laryngol Otol 2006;120:502-504 4. Mohammed Iqbal Syed et al. Lemierre Syndrome: Two Cases and a Review. Laryngoscope, 117:1605-1610, 2007 5. Vohra A, Saiz E, Ratzan KR. A young woman with a sore throat, septicaemia, and respiratory failure. Lancet 1997; 350:928.