This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Lemierre syndrome is a rare and potentially life-threatening complication of bacterial infections that usually affects previously-healthy adolescents and young adults. It most commonly develops in association with a bacterial throat infection, but it may develop in association with an infection involving the ears, salivary glands (parotitis), sinuses, or teeth; or in association with an Epstein-Barr infection. The bacteria typically responsible for infection in Lemierre syndrome is Fusobacterium necrophorum, although a variety of bacteria can be responsible. In people with Lemierre syndrome, the initial infection spreads into tissues and deep spaces within the neck, leading to the formation of an infected blot clot (septic thrombophlebitis), sometimes made up of pus, in the internal jugular vein (the blood vessel that carries blood away from the brain, face, and neck). In addition to worsening symptoms of the initial infection, symptoms at this stage of the disease typically include persistent fever and chills (rigors), as well as pain, tenderness and swelling of the throat and neck. The infected clot then circulates in the blood (septicemia), resulting in the infection also spreading to the lungs (most commonly), skeletal system, and/or other parts of the body such as the spleen, liver, kidney, heart, or brain. This can lead to life-threatening complications such as respiratory distress syndrome due to pulmonary emboli (blood clots in the lung), damage to other affected organs, and/or septic shock (in about 7% of cases).
Lemierre syndrome may be diagnosed based on signs and symptoms, various blood tests, and imaging studies. Because most throat infections in young, healthy people do not cause severe health problems, diagnosis and treatment may be delayed. The main treatment involves intravenous antibiotic therapy over several weeks, but surgery may be necessary when there is abscess formation, respiratory distress, or severe clotting in the chest or brain. The long-term outlook and chance of survival in people with Lemierre syndrome varies depending on how much the syndrome progresses, but even with appropriate treatment, it is fatal in some cases.
For more information, visit GARD.