Ménétrier syndrome is characterized by hyperplastic mucosal folds in the stomach area and is characterized by protein loss and upper abdominal pain. The risk of degeneration of the mucosal folds is around ten percent, so the patients have to take part in close controls. Treatment is symptomatic.
What is Ménétrier Syndrome?
In Ménétrier’s syndrome, the gastric mucosa shows conspicuously large hyperplasia, which gives the stomach a roughly wrinkled appearance. The syndrome is also called Ménétrier’s disease or hypertrophic gastropathy and is a rather rare disease with no precise information on its prevalence. The disease is counted among the exudative gastroenteropathies, which are characterized by a loss of protein in the gastrointestinal tract. See sciencedict for Introduction to Metamorphopsia.
The syndrome primarily affects middle-aged men and owes its name to Pierre E. Ménétrier, who first described it. Due to its low prevalence, the disease has not yet been fully researched. So far, for example, we can only speculate about the causes. The disease can have different degrees of severity and therefore does not always have to be symptomatic, but can also take place largely silently.
Causes
The causes of Ménétrier’s syndrome have so far been rather speculative. For example, there is a connection with a Helicobacter pylori infection, which is often symptomatic of the disease. Those affected also often report an infection with the cytomegalovirus that they suffered as children.
Both the accompanying finding of Helicobacter pylori and the cytomegalovirus infection do not necessarily have to be causally related to Ménétrier’s syndrome. In order to establish such a connection, there has so far been a lack of controlled studies and case reports. A genetic disposition does not appear to be associated with the disease. Familial clusters, for example, were not observed. A sporadic occurrence is to be assumed. This largely rules out a genetic cause with a hereditary basis.
Symptoms, Ailments & Signs
The stomach changes pathologically in the context of Ménétrier’s syndrome. Macroscopically, the gastric mucosa appears thickened and shows significantly coarser folds, which are particularly visible on the greater curvature of the stomach. Cystic dilatations and lengthening are evident at the foveolae. The cysts are often covered with mucus. The gastric glands are affected by degeneration and lose their acid-forming parietal cells. The mucous membrane of the stomach expands due to edema and is populated by inflammatory cells. Eosinophilia is often present.
Individual beams of muscle cells extend into the gastric mucosa. Achlorhydria can also be symptomatic. Inflammatory reactions in the gastric mucosa are associated with the loss of plasma protein and often result in hypoproteinemia. Anemia is particularly common when parietal cells are destroyed. Diarrhea is one of the most common symptoms. Depending on the severity, individual symptoms may be absent.
Diagnosis & course of disease
To diagnose Ménétrier’s disease, the doctor performs a gastroscopy. He takes biopsies from the most conspicuous areas. The histological examination of the biopsies should be accompanied by an examination for Helicobacter pylori. In individual cases, the diagnosis can be made on suspicion by an ultrasound examination of the stomach. This can be followed by a gastroscopy to secure the suspected diagnosis.
The course of the disease depends on the severity. The most serious complication of Ménétrier syndrome is malignant hyperplasia. The risk of developing gastric cancer is around ten percent. However, regular check-ups can largely prevent serious complications of this type. Lethal consequences are not to be expected for this reason.
Complications
First and foremost, those affected by Ménétrier syndrome suffer from relatively severe pain in the upper abdomen. These can also lead to a loss of appetite and further to underweight or deficiency symptoms. In many cases, this pain has a very negative effect on the psyche of those affected, so that the patients suffer from depression or other mental disorders.
The risk of cancer is also significantly increased due to Ménétrier’s syndrome, so that those affected are usually dependent on various regular examinations in order to avoid complications. In addition, cysts or inflammation can form. In most cases, these are associated with pain in the stomach.
Diarrhea can also be associated with Ménétrier syndrome and have a very negative effect on the quality of life of those affected. This sometimes leads to permanent dehydration. The treatment of Ménétrier syndrome is symptomatic in most cases and is not associated with complications.
Those affected can fight the infection with the help of antibiotics. However, in the case of a tumor, it must be surgically removed. The patient’s life expectancy may also be reduced.
When should you go to the doctor?
Ménétrier syndrome does not always cause noticeable symptoms. In some cases, a longer period of freedom from symptoms is documented, which makes diagnosis difficult. Disorders of the digestive tract, diarrhea or a general feeling of illness must be examined and treated. The pain is often persistent until the sufferer notices it. As soon as changes or irregularities appear, a doctor should be consulted. People need help if they feel pain, lose their appetite, or lose weight. If various deficiency symptoms occur, if there are diffuse functional disorders, cramps or an inner weakness, a doctor’s visit is advisable.
If the physical symptoms are accompanied by emotional or psychological abnormalities, if there is a withdrawal from social life or if the person concerned suffers from mood swings, a doctor is needed. In the event of behavioral problems, an increased need for sleep or if you tire quickly when performing light activities, a doctor’s visit is recommended. There are irregularities, the cause of which must be determined.
Swellings on the body, palpable lumps or hardening should always be presented to a doctor. If they increase in scope or frequency, a doctor’s visit must be initiated immediately.
Treatment & Therapy
A causal therapy for Ménétrier’s syndrome does not yet exist. The disease is therefore treated symptomatically. Drug therapy can be used to treat upper abdominal pain. If an infection with Helicobacter pylori has been detected and chronic type B gastritis is present, treating the infection is the focus of symptomatic therapy.
The finding can possibly regress after successful treatment of the helicobacter. A few weeks after the therapy, the patient’s stomach is checked for improvements. If the original finding persists despite successful treatment of the infection, the patient then takes part in frequent gastroscopies, which serve to check the findings. If these check-ups show any changes, biopsies are taken.
If these biopsies show progressive changes and progressively increasing dysplasia, a gastrectomy must be performed as a precaution. This resection of the stomach corresponds to a surgical procedure in which all altered areas are removed. If necessary, the affected passages can then be reconstructed. The possible degeneration is counteracted preventively by the resection.
If the regular check-ups of the stomach no longer show any changes and the disease is therefore not progressive but comes to a standstill, the stomach is usually not resected. In this case, the patient receives symptomatic treatments depending on his symptoms, which are primarily intended to improve his quality of life.
Outlook & Forecast
Ménétrier syndrome is treated purely symptomatically. The patient can support the therapy by adopting a conservative lifestyle and changing their diet. Due to the severe gastrointestinal complaints, warming pads are recommended. Natural painkillers can also provide acute relief. The intake should first be discussed with the doctor in order to avoid complications. Rest and protection are indicated after an operation. Patients should follow the doctor’s instructions to avoid complications. This includes changing your diet.
After a gastrectomy, heavy or particularly irritating foods must first be avoided. Alcohol, nicotine and caffeine should also be avoided so as not to further irritate the gastrointestinal tract. The details of the change in diet should be discussed with a nutritionist or the responsible specialist. Sporting activity may be resumed a few days after the procedure. Here, too, it is advisable to consult your doctor first, as there is a risk of serious complications if the body is put under stress again too soon. If, despite these measures, gastrointestinal complaints, upper abdominal pain or other typical symptoms occur again, the doctor must be informed.
Prevention
Ménétrier’s syndrome is of unknown etiology. For this reason, the disease cannot be successfully prevented. Patients with the syndrome can at least prevent malignant degeneration of the hyperplastic gastric mucosa through regular check-ups and biopsies of the stomach and, if necessary, have a gastric resection carried out in good time.
Aftercare
In most cases, those affected by Ménétrier syndrome have very few measures or options for aftercare. First and foremost, an early diagnosis is necessary in order to prevent further deterioration of the symptoms. Self-healing is usually not possible with Ménétrier syndrome, so ideally the patient should see a doctor at the first signs and symptoms.
Those affected depend on the intake of various medications. Regular intake with the correct dosage should always be observed. If anything is unclear or if you have any questions, you should always consult a doctor first. Furthermore, with this disease, frequent checks and regular examinations by a doctor are very important in order to detect further damage at an early stage.
The diet of the person affected should also be adjusted, and the doctor can also draw up a nutrition plan. If Ménétrier’s syndrome is to be treated by surgery, it is advisable for those affected to rest after the operation and take care of their bodies. Efforts or physical activities should be avoided. Likewise, only light food should be taken, whereby the body can adapt to the usual food over time.
You can do that yourself
These patients are followed up closely because there is a high risk that their disease will degenerate. Other complications of the disease can also be detected and treated at an early stage by the controls, which is why these examination appointments should be observed.
The fact that the disease is rare and still largely unexplored can unsettle those affected. However, you can find the latest information on the self-help portal for rare diseases (www.orpha- selbsthilfe.de). There you can also contact other people affected, which experience has shown can be helpful.
Depending on the severity of the disease, those affected suffer from sometimes severe pain and diarrhea. This may be why they take little part in normal social life. This can wear patients down and trigger depression or other mental illnesses. Psychological therapy support is therefore urgently recommended. Patients with Ménétrier syndrome need to be careful to drink enough fluids, especially if they are prone to diarrhea. Otherwise your body will become dehydrated, which will weaken it at the same time.
Despite possible pain, patients should also eat enough food to avoid provoking any deficiency symptoms. The diet should consist of healthy and natural foods, but also be easily digestible. In times of crisis, cooked and pureed foods are recommended. Old home recipes, such as oatmeal, can be spiced up in a tasty way, are nutritious and well tolerated.
