Definition

Lemierre's syndrome is a rare bacterial infection of the throat. If left untreated, the infection can spread to the lymphatic vessels, impairing their ability to drain fluids. These fluids originate from excess fluid inside and outside of cells and must be returned to the bloodstream. Lemierre's syndrome can also cause swelling in the jugular vein, leading to the formation of blood clots. If not treated promptly, this condition can be life-threatening.

This rare syndrome typically affects healthy teenagers and young adults. While it is often linked to bacterial throat infections, it can also develop from infections of the ear, salivary glands, sinuses, or teeth.

Causes

Lemierre's syndrome is most commonly caused by the bacterium Fusobacterium necrophorum, which is frequently found in the throat without causing infection. However, if the outer lining (mucosa) of the throat is damaged, the bacteria can enter and infect the throat.

Other bacteria known to cause Lemierre's syndrome include Staphylococcus aureus, which is commonly found on the skin and in the nasal cavity. Staphylococcus infections can occur if the bacteria enter body tissues or the bloodstream and can spread through shared objects like towels and razors or contaminated food.

Other bacterial causes of Lemierre's syndrome include Streptococcus, Bacteroides, and Klebsiella pneumoniae. Additionally, the Epstein-Barr virus, part of the herpesvirus family, can also cause this syndrome.

Risk Factor

Lemierre's syndrome begins as a bacterial throat infection that spreads. Since bacterial throat infections are common, anyone can be at risk if the infection is not properly treated. The progression of bacterial throat infections into Lemierre's syndrome is usually found in previously healthy young adults, occurring more frequently in males.

Symptoms

Symptoms of Lemierre's syndrome may not appear immediately after infection. The condition usually starts in the throat, with a sore throat being the most likely initial symptom. Other early symptoms include:

• Swelling around the lymph nodes in the neck
• Unusual headaches
• Sharp neck pain
• High fever
• Stiffness, weakness, or fatigue
• Sensitivity to light (photophobia)
• Difficulty breathing
• Difficulty swallowing
• Inflammation of the tonsils (tonsillitis), the two lymph nodes that protect the body from infection
• Coughing up blood or blood-streaked phlegm
• Loss of appetite or nausea
• Vomiting

As the infection spreads, these symptoms can worsen. Affected individuals should seek immediate emergency care if they experience these symptoms. The infection can spread to the neck, causing painful neck swelling, typically on one side. It can also lead to cellulitis, an infection beneath the skin.

In later stages, the infection can spread to the veins in the neck, allowing bacteria to spread to organs such as the lungs, joints, liver, kidneys, brain, bones, heart, and meninges (the membranes surrounding the brain). At this stage, patients may experience shortness of breath and fever, potentially requiring breathing support.

Diagnosis

Lemierre's syndrome is diagnosed based on medical history and various examinations, primarily focusing on a history of prolonged sore throat. Diagnostic procedures include laboratory tests and imaging.

Laboratory Tests

Laboratory tests may include a complete blood count (CBC), liver function tests, kidney function tests, and inflammation markers. A CBC may reveal a bacterial infection, which could be accompanied by a decrease in platelets if widespread blood clotting has occurred. Liver and kidney function tests help determine the spread of the infection, as these organs may be affected. Inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) can also be measured.

Blood cultures may be performed to identify the bacteria causing the infection. However, if Fusobacterium is the cause, the culture may be negative, as this bacterium is difficult to grow.

Imaging

Imaging primarily targets the lungs, as they are the most frequent site of infection. Chest X-rays can reveal complications in the lungs. Infections of the jugular vein, a common target, may be examined using ultrasound, contrast-enhanced CT scans of the neck, or MRI. Ultrasound is the safest and least expensive option but may struggle to distinguish structures in the neck. CT scans and MRI are more effective at detecting infections and blood clots in the jugular vein, though their availability is limited in some regions.

Management

Initial treatment for Lemierre's syndrome involves antibiotics to combat the bacterial infection. The choice of antibiotic is based on the most likely infection source or blood culture results. If the infection does not respond to antibiotics, surgery may be required to treat the throat or neck. In cases where an abscess (pus-filled pocket) forms, it must be drained. The doctor may also perform a ligation of the jugular vein, redirecting blood flow by closing off the affected vein to allow it to heal.

Blood thinners may also be prescribed, as Lemierre's syndrome is strongly associated with increased blood clot formation. Blood clots can block vessels to critical organs such as the heart and brain, making this a dangerous condition. Blood thinners prevent clot formation but carry a risk of excessive bleeding. This can cause wounds to bleed for longer than normal. Consult your doctor before starting this treatment.

Complications

Complicated Lemierre's syndrome is life-threatening, with mortality rates ranging from 5% to 18%. Complications include:

• Spread of infection to the bones (osteomyelitis)
• Infection of the meninges (meningitis)
• Shortness of breath leading to respiratory distress (inability to breathe without assistance)
• Shock or organ failure due to lack of oxygen

Prevention

Lemierre's syndrome can be prevented by treating throat infections promptly. If you have a throat infection, see a doctor. If the infection causes difficulty swallowing, breathing, or coughing up blood, seek medical attention immediately. The infection may be bacterial and can be treated with antibiotics.

When to See a Doctor?

See a doctor immediately if you have a prolonged throat infection, difficulty swallowing or breathing, cough up blood, or experience neck swelling. The sooner the infection is treated, the higher the recovery rate for Lemierre's syndrome and the better your chances of avoiding complications. If left untreated, the infection can spread throughout the body, leading to organ damage (sepsis).

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