Lemierre’s syndrome occurs when bacteria from a throat infection spread to a major blood vessel and then poison the bloodstream and cause blood clots.
The condition can be severe but has a high survival rate among people who seek immediate medical attention. Treatment includes several courses of antibiotics and can require surgery.
Fewer than 4 in every 1 million people develop Lemierre’s syndrome worldwide each year. However, cases have steadily increased since 1998.
In this article, we take a look at the symptoms and causes of the condition, along with how Lemierre’s syndrome is diagnosed and treated.
A sore throat may be particularly concerning if it does not improve with antibiotic medication.
Lemierre’s syndrome may also cause neck swelling and pain on one side of the neck that starts at the ear and spreads downward. The infection can make the voice sound hoarse or muffled.
In general, a person with a sore throat should become concerned about Lemierre’s syndrome if their symptoms last longer than expected or become severe.
As the disease progresses, additional symptoms may include:
- nausea, loss of appetite, diarrhea, or vomiting
- coughing or pain while breathing
- severe headaches
- dental pain
- pain, swelling, and stiffness of the joints, such as knees or hips
- yellowish skin tone
- on rare occasions, coughing up blood
Without treatment, a person with Lemierre’s syndrome will typically develop one or more additional health problems. The most common is a condition called sepsis, where organs become inflamed as they fight against bacteria in the blood.
Symptoms associated with sepsis include:
- fever above 101°F
- shortness of breath
- mental confusion
- increased heart rate
Symptoms of advanced sepsis include:
- difficulty breathing
- incoherent speech
- irregular heartbeats
- stomach pain
- decrease in urination
- patches of discolored skin
- low blood pressure
Two other conditions are sometimes associated with Lemierre’s syndrome:
Pneumonia symptoms include:
- stabbing chest pains
- clammy skin or excessive sweating
- fast or shallow breathing
Meningitis is an infection of the fluid surrounding the brain and spine. Meningitis associated with Lemierre’s syndrome occurs when bacteria enter the spinal fluid from the bloodstream.
Meningitis symptoms include:
- head swelling
- seizure or stroke
- stiff neck
- irritability or confusion
Lemierre’s syndrome usually develops from a bacterial infection inside the throat. Less often, the infection begins in the ears, nose, or other areas inside the head.
The bacteria spread to the inner jugular vein, a major blood vessel that connects the brain to the heart and lungs.
From there, the infection enters the bloodstream, causing infected blood clots that travel throughout the body, endangering other organs.
In general, the bacteria that cause Lemierre’s syndrome live naturally in the human body but have rapidly spread and become harmful. Around 90 percent of cases occur from the spread of Fusobacterium necrophorum, a bacterium found in the stomach, large intestine, small intestine, colon, and throat.
Doctors are uncertain why these bacteria spread. One theory suggests that other diseases in the throat weaken the body’s defense system, making it easier for bacteria to move around.
Medical experts largely blame antibiotic overuse for bacteria becoming stronger. As a result, doctors prescribe this type of medication less often. While good for public health, the decrease in antibiotic use could make it easier for bacteria to spread.
Who gets Lemierre’s syndrome?
Anyone with a sore throat or upper respiratory infection has a slight chance of developing Lemierre’s syndrome. Several cases of Lemierre’s Syndrome have occurred in people with strep throat or mono.
Lemierre’s syndrome can affect a person at any age. However, people under 30 years old account for most cases and are more likely to develop the disease than older adults.
A person diagnosed with Lemierre’s syndrome may be otherwise healthy with no history of health problems.
Lemierre’s syndrome was relatively common before the discovery of antibiotics. In the 1960s, however, antibiotics almost eliminated the disease. For this reason, Lemierre’s syndrome is sometimes called the “forgotten disease.” Many doctors find diagnosing it difficult.
Diagnosis begins with a blood sample to confirm the presence of harmful bacteria. Next, the doctor may use a contrast computed tomography (CT) scan or an ultrasound to check for a blood clot in the inner jugular vein.
A chest X-ray may also be used to identify whether the infection has spread to the lungs.
Once Lemierre’s syndrome is diagnosed, it is treated immediately and aggressively using antibiotics.
Treatment may include multiple courses of antibiotics taken over several weeks. This drug regimen is often pumped directly into the bloodstream instead of being taken by mouth for a faster response.
A doctor will prescribe antibiotics based on the type of bacteria that are causing the infection. Treatment for F. necrophorum may include:
- clindamycin (Cleocin)
- metronidazole (Flagyl)
Treating this disease is not always straightforward. Sometimes, Lemierre’s syndrome is caused by a different type of bacteria and sometimes, multiple infections occur. In these cases, a doctor may treat the disease with penicillin or a different set of targeted antibiotics.
Advanced cases of Lemierre’s syndrome can require surgery to drain abscesses, which are the pockets where pus and bacteria have built up. Abscesses may develop in the neck, head, lungs, or brain.
Limited evidence suggests that, in some cases, blood thinners may play a role in an individual making a full recovery from Lemierre’s syndrome.
In one study, 100 percent of people who were treated survived after receiving blood thinners in combination with antibiotics. Many of them also had surgery.
People who seek immediate medical attention for Lemierre’s syndrome have a high survival rate. Relief from symptoms may begin after several days of antibiotics. Full recovery can be expected in 3 to 6 weeks.
The disease is often more serious than its symptoms suggest and may continue after the initial throat infection goes away. And, while many people with Lemierre’s syndrome experience extreme symptoms that lead them to seek medical attention, not everyone does.
Anyone with a recent sore throat and who is experiencing pain, fever, or difficulty breathing should contact a doctor immediately.