Lemierre's syndrome

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Lemierre's syndrome (or Lemierre's disease, also known as postanginal sepsis and human necrobacillosis) is a form of thrombophlebitis[1] usually caused by the bacterium Fusobacterium necrophorum, and occasionally by other members of the genus Fusobacterium (F. nucleatum, F. mortiferum and F. varium etc.) and usually affects young, healthy adults. Lemierre's syndrome develops most often after a sore throat caused by some bacterium of the Streptococcus genus, has created a peritonsillar abscess, a pocket filled with pus and bacteria near the tonsils. Deep in the abscess, anaerobic bacteria (microbes that do not require oxygen) like Fusobacterium necrophorum can flourish. The bacteria penetrate from the abscess into the neighboring jugular vein in the neck and there they cause an infected clot (thrombosis) to form, from which bacteria are seeded throughout the body by the bloodstream (bacteremia). Pieces of the infected clot break off and travel to the lungs as emboli blocking branches of the pulmonary artery bringing the heart's blood to the lungs. This causes shortness of breath, chest pain and severe pneumonia. Fusobacteria are normal inhabitants of the oropharyngeal flora. This is a very rare disease with only approximately 160 reported cases in the last 100 years.[2]

Sepsis following from a throat infection was described by Scottmuller in 1918.[3] However it was Andre Lemierre, in 1936, who published a series of 20 cases where throat infections were followed by identified anaerobic septicemia, of whom 18 patients died.[4]


Signs and symptoms

The symptoms vary per person, but usually start with a sore throat, fever, and general body weakness. These are followed by extreme lethargy, spiked fevers, rigors, swollen cervical lymph nodes and a swollen, tender or painful neck. Often there is abdominal pain, diarrhea, nausea and vomiting during this phase. These symptoms usually occur several days to 2 weeks after the initial symptoms.

Symptoms of pulmonary involvement can be shortness of breath, cough and painful breathing (pleuritic chest pain). Rarely there is hemoptysis. Painful or inflamed joints (arthralgia or arthritis, respectively) exist when the joints are involved.

Septic shock can occur which presents itself with hypotension, tachycardia, oliguria and tachypnea. Nuchal rigidity, headache and photophobia occur in case there is meningitis.

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