What is Lemierre Syndrome & How is it Treated?| Causes,Symptoms, Diagnosis of Lemierre Syndrome

What is Lemierre Syndrome?

Lemierre Syndrome is a pathological condition that occurs when a bacterial infection in the throat spreads to the blood vessels and infiltrates the bloodstream causing a serious complication like septicemia and even blood clots. Lemierre Syndrome is an extremely rare and serious condition but incidentally most people from this condition survive provided they get immediate medical treatment. Treatment for Lemierre Syndrome revolves around extended course of antibiotics and may at times even require surgical procedures. The prevalence of Lemierre Syndrome was much higher before antibiotics were introduced. However, with the introduction of penicillin the incidence of this condition fell down significantly [1].

There has been of late a steady increase in the cases of Lemierre Syndrome due to decreased use of antibiotics in treatment of infections involving the throat and the pharynx area. Despite a steady increase in the cases of Lemierre Syndrome, it still remains rather rare with an estimated 1 case in every 100,000 people. This condition affects mainly the younger generation with adolescents who have a perfectly normal health history being mostly affected [1].

The age of onset of Lemierre Syndrome is between 19 and 21 years and males tend to have it more than females. Lemierre Syndrome starts as a normal throat infection that then spreads to the soft tissues of the neck and then ultimately infects the blood vessels [1].

What is Lemierre Syndrome?

What Causes Lemierre Syndrome?

The primary cause for Lemierre Syndrome is a bacterial infection. This infection normally originates from the throat but it can also start in the ears as well as the nose. The bacteria then start to spread to the inner jugular veins. This vein connects the brain to the lungs and the heart and thus is extremely vital for the body. It is from this vein that the bacteria infiltrates the blood causing clots that traverse throughout the body and can get lodged anywhere posing a threat to other vital organs of the body. The bacteria responsible for causing Lemierre Syndrome are present in the body naturally but become harmful and start spreading to cause symptoms as seen with this condition [2].

Majority of the cases of Lemierre Syndrome are caused by Fusobacterium Necrophorum bacteria which is normally present in the dormant form in the stomach, intestines, throat and colon. There is no clear cut reason as to why these bacteria become active and start spreading. However, the most favored hypothesis, according to scientists, is that certain medical conditions that make the immune system weak and make it easier for the bacteria to spread is the primary cause [2].

They are also of the opinion that prolonged use of antibiotics makes the bacteria resistant to most medications necessitating the physicians to use medications sparingly. This again makes it easier for the bacteria to spread and cause symptoms [2].

Who is at Risk for Lemierre Syndrome?

Lemierre Syndrome may be a rare medical condition but there are certain risk factors which increase the likelihood of an individual developing this condition. An individual with frequent upper respiratory infections and bouts of sore throat is more likely to develop Lemierre Syndrome than others. There have been many cases where people have developed Lemierre Syndrome due to strep throat [2].

There is no age prevalence related to the development of Lemierre Syndrome; however, people under the age of 30 are more vulnerable for getting this disease. In rare instances, a perfectly healthy individual can at times also develop Lemierre Syndrome [2].

What Are The Symptoms of Lemierre Syndrome?

The primary presenting feature of Lemierre Syndrome in the initial stages is a throat infection or a sore throat that lasts for more than a week. This will be accompanied by muscle weakness, lethargy, and fever. The sore throat will not be responsive to standard antibiotic treatments. There may also be swelling observed in the neck with pain in the ear radiating downwards. There are also certain changes in the voice of the individual with it sounding more muffled. There will be gradual worsening of the symptoms as the disease condition progresses [2].

There will be additional symptoms of nausea along with loss of appetite. Diarrhea and vomiting may also be observed. The breathing of the affected individual also gets infected in that the individual finds it painful to take in air and may even have coughing. There will be episodes of sever unrelenting headaches along with chills. Some people also have pain in the teeth as well. Some people also complain of pain and stiffness of the joints of the knees and hips. At the times, the skin may also look jaundiced [2].

Hemoptysis is observed in rare cases of Lemierre Syndrome. If the condition is left untreated then the individual will go on to develop sepsis which may have life threatening complications. If the individual goes on to develop sepsis then he or she will have high fever, difficulty breathing, lack of mental alertness, tachycardia, dizziness, and fatigue. As sepsis progresses, the patient will start having hallucinations, speech difficulties, skin discoloration, hypotension, and decreased urinary frequency [2].

If these symptoms develop then it is mandatory to take the patient to the nearest emergency room for treatment as any delay may pose a threat to the life of the patient. An individual with Lemierre Syndrome in most cases will develop two major health conditions, namely pneumonia and meningitis. Thus it is important to be on the lookout for the symptoms of these conditions so that prompt treatment can be given before any complications set in [2].

For an individual with pneumonia due to Lemierre Syndrome, there will be complaints of chest pains that will be stabbing in nature. The individual will sweat a lot. The breathing will be shallow with audible wheezing. Meningitis on the other hand is caused when the bacterial enters the spinal fluid resulting in infection of the fluid around the brain and spine. An individual with meningitis caused due to Lemierre Syndrome will have swelling of the head [2].

There will also be episodes of seizures and strokes. The individual will have an extremely stiff neck. The affected individual will have lack of mental awareness and confusion. If these symptoms are observed then it is mandatory for the patient to be taken to the hospital for immediate treatment [2].

How is Lemierre Syndrome Diagnosed?

Lemierre Syndrome was quite common prior to the discovery of antibiotics. However, by the late 1960s, this disease was completely eliminated. This is the reason that makes it difficult to diagnose Lemierre Syndrome. However, to diagnose this disease, based on the symptoms, the treating physician will order a series of blood tests to confirm the presence of a bacterial infection [2].

Once confirmed, the physician will order radiographs in the form of CT scan or ultrasound. This will show the presence of a clot within the inner jugular vein confirming the diagnosis of Lemierre Syndrome. Additionally, an x-ray of the chest may be done to look for the presence of the spread of the disease [2].

How is Lemierre Syndrome Treated?

With regard to treatment options, it is important to diagnose Lemierre Syndrome first. Once a diagnosis is made then the patient will be given an extensive course of antibiotics. The administration of antibiotics will go on for many weeks before the infection can be controlled. In most cases, the medication is directly injected into the blood instead of the usual way of giving it orally. This will hasten the recovery process and allow the infection to clear up quickly and prevent further complications [2].

The medications given for treatment of Lemierre Syndrome include cephalosporin, Cleocin, and metronidazole. Treating Lemierre Syndrome is not an easy task as this condition at times may be caused by different forms of bacteria which will be responsible for the condition and will require different approaches to treating them. In addition, at times there may be more than one infection occurring simultaneously in Lemierre Syndrome which further complicates the picture and as each infection will need different medication to treat it [2].

In severe cases, there may be formation of abscess in the brain, neck, or lungs as a result of accumulation of bacteria and pus due to Lemierre Syndrome. This will require surgical procedures for drainage of the abscess. Some studies have suggested the use of blood thinners in the successful treatment of Lemierre Syndrome [2].

In the study, 100 people with Lemierre Syndrome were treated with blood thinners along with antibiotics and completely recovered from the condition without any sequelae. However, more research needs to be done to understand the role of blood thinners in the treatment of Lemierre Syndrome [2].

In conclusion, Lemierre Syndrome is a rare medical condition in which bacteria from the throat infiltrates into the adjacent oropharyngeal region and ultimately infects the blood causing sepsis. Even though Lemierre Syndrome causes potentially life threatening complications the survival rate of patients with this condition is quite good once a confirmed diagnosis is made and treatment is started with antibiotics. The individual can find relief of symptoms within a few days of starting antibiotics [1, 2].

This condition was quite prominent when antibiotics were not in use but declined sharply after introduction of penicillin. However, Lemierre Syndrome has again started to raise its head in recent times due to less use of empiric antibiotics in the treatment of throat infections. The treatment usually involves aggressive administration of antibiotics which is given directly to the blood instead of orally to hasten the recovery process. Once treatment has started is normally takes about a couple of months of the individual to get back to normal life [2].

Thus it is important to get checked up by a physician if an individual has a sore throat or infection that remains persistent despite standard antibiotic treatment as this may be a classic case of Lemierre Syndrome which will require further more aggressive care. If Lemierre Syndrome remains untreated, it may lead to pneumonia, meningitis, or even septicemia which can be quite serious and emergent treatment will be required in such cases [2].

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