Nonne-Milroy-Meige syndrome is a condition that is hereditary. This means that Nonne-Milroy-Meige syndrome is present from birth. As part of the disease, affected patients primarily suffer from lymphedema. As a result, various malformations develop. In addition, in some cases, the lymphedema causes the development of the affected individuals to be disturbed.
What is Nun-Milroy-Meige syndrome?
Nun-Milroy-Meige syndrome got its name in reference to the individuals who first described the condition. These were Max Nonne, a neurological specialist from Germany, William Milroy, an internist from the United States, and finally Henry Meige, a specialist in internal medicine from France. Nonne-Milroy-Meige syndrome is also referred to in some cases by the synonymous terms trophoedema hereditarium, hereditary chronic edema, or pseudoedematous hypodermic hypertrophy. In English, the condition is known primarily as tropholymphoedema. However, some physicians and researchers consider the term Nonne-Milroy-Meige syndrome to be outdated and redundant. It is assumed that the Nonne-Milroy-Meige syndrome is not an independent diagnosis. This is because the clinical picture of the Nonne-Milroy-Meige syndrome is not uniform in numerous cases, so that the existence of a delimitable syndrome is doubted by some sides. Instead, critics of the disease designation argue that the Nonne-Milroy-Meige syndrome and its symptoms should be counted among the lymphedemas, which have a hereditary component. For this reason, current scientific literature divides Nonne-Milroy-Meige syndrome into two types. Type 1 is the so-called Nonne-Milroy syndrome. The corresponding symptoms, in particular the lymphedema characteristic of the disease, appear before the patients reach the age of 35. As a rule, there are no malformations that are typical of type 2. Type 2 is also called Meige syndrome. In this case, the typical symptoms do not develop until after the affected person’s 35th birthday. Here, too, hereditary lymphedema is in the foreground. However, in this type, numerous malformations join the usual complaints.
Causes
No definite statements can yet be made about the exact causes for the development of the Nonne-Milroy-Meige syndrome. Basically, it is a predominantly hereditary disease, i.e. a form of hereditary disease. This means that affected patients suffer from the disease from birth. Depending on the type of Nonne-Milroy-Meige syndrome, symptoms may develop in childhood or in adults. Presumably, there is a genetic defect that leads to the development of the disease.
Symptoms, complaints, and signs
Basically, Nonne-Milroy-Meige syndrome is associated with a number of different complaints and symptoms. The main symptom of the disease is lymphedema, which takes on different manifestations. In principle, the edemas are multiple. In the majority of cases, they occur on the limbs of the affected persons. In addition, the patients may suffer from other deformities or complaints. Possible symptoms include mental retardation, reduced intelligence and short stature. Some individuals suffer from a malformed spine and are hard of hearing. There is also obesity, various trophic ulcerations and acromicriosis. Some individuals suffering from Nonne-Milroy-Meige syndrome have elephantiasis, which is associated with marked edema around the ankles. Finally, malformations of the heart or circulatory system are also possible.
Diagnosis and course of the disease
The diagnosis of Nonne-Milroy-Meige syndrome is made on the basis of clinical symptoms, which vary to a greater or lesser degree depending on the patient. In principle, consultation with a physician is indicated when characteristic symptoms of the disease occur. The patient first consults his or her family physician or an appropriate specialist. The diagnosis of the disease is made step by step, with the patient interview or anamnesis coming first.The sick person informs the attending physician about all complaints as well as his or her lifestyle, past illnesses and medications taken. In this way, it may already be possible for the physician to make a tentative diagnosis. Since the Nonne-Milroy-Meige syndrome is a hereditary disease, the family history must be taken particularly thoroughly. After the history is taken, clinical examination procedures are used. The physician examines the lymphedema and performs numerous blood analyses. In this way, decisive clues are collected, which eventually lead to the diagnosis.
Complications
Due to Nonne-Milroy-Meige syndrome, patients suffer from a number of different malformations and deformities. Furthermore, the child’s development is also significantly delayed, so that affected individuals suffer from mental retardation and are usually dependent on the help of other people in their daily lives. The patient’s quality of life is significantly limited and reduced by the Nonne-Milroy-Meige syndrome. Furthermore, patients also suffer from short stature and also hearing loss. The spine can also be affected, so that the patients suffer from restricted movement. Obesity can also occur in this syndrome. Due to the malformations, the heart is also affected in many cases, so that the patients suffer from circulatory problems and may possibly die of cardiac death. Furthermore, the parents of the child and the relatives are also affected by the Nonne-Milroy-Meige syndrome. They suffer from psychological complaints and depression. Unfortunately, it is not possible to treat the Nonne-Milroy-Meige syndrome. For this reason, treatment is focused exclusively on the respective symptoms. Possibly, the life expectancy of the patient is also limited and reduced in the process.
When should you see a doctor?
When ulcers, obesity, or infections occur, there may be an underlying cause of Nonne-Milroy-Meige syndrome. A visit to the doctor is necessary if symptoms do not resolve on their own or become progressively more severe. Symptoms that cannot be attributed to a specific cause must always be clarified by a doctor. People who lead an unhealthy lifestyle or regularly drink alcohol are particularly at risk. People with skin diseases or other conditions related to the immune system or hormone balance are also among the risk groups and should see a doctor immediately if skin complaints or other physical changes are noticed. In addition to the primary care physician, patients suspected of having Nonne-Milroy-Meige syndrome may also see an internist. Depending on the nature of the symptoms, the dermatologist, a gastroenterologist, or a doctor of hormonal disorders may be appropriate. Because of the variety of symptoms that usually occur with the chronic condition, several physicians are usually involved in the treatment. Close consultation with the specialist must be maintained during therapy because of the high risk of complications.
Treatment and therapy
In the current era, there is still no way to treat Nonne-Milroy-Meige syndrome causally. For this reason, therapy and alleviation of the existing symptoms become the focus of therapeutic efforts. First and foremost, lymphatic drainage is applied. This drainage is to be performed regularly and relieves the discomfort caused by the edema. Other therapeutic measures are similar to those generally considered for lymphedema, even if they are not associated with Nonne-Milroy-Meige syndrome. For example, patients are encouraged to follow a healthy lifestyle and reduce excess weight.
Outlook and prognosis
The course of the disease in Nun-Milroy-Meige syndrome turns out to be unfavorable. Recovery can be ruled out in such a health disorder. Legal requirements do not allow scientists to alter the patient’s genetics as things stand. Therefore, the cause of the syndrome cannot be treated. The treating physician concentrates on the individually severe symptoms and draws up a treatment plan. The affected person must undergo lifelong health care so that a rapid response can be made in the event of changes or abnormalities.Regular check-ups are basically necessary so that relief from the symptoms can be achieved. Due to medical progress, physicians are constantly succeeding in developing new therapy methods that contribute to an improvement of the overall situation. Nevertheless, the disease represents an immense burden for the patient as well as his relatives in coping with everyday life. Often, the circumstances lead to the development of a mental illness, to which all those affected are exposed in the long term Situation of overload. This possible development must be taken into account when making the prognosis. In severe cases, the cardiovascular system of the affected person is also damaged from birth. The risk of a reduced quality of life is therefore increased in these patients. They may develop a life-threatening situation at any time.
Prevention
To date, it is not possible to prevent Nonne-Milroy-Meige syndrome. The condition exists from birth, so preventive measures are moot. Effective therapeutic methods make it easier for patients to live with lymphedema.
Aftercare
In most cases, those affected by Nonne-Milroy-Meige syndrome have few, if any, special aftercare measures available to them. Because it is a hereditary disease, it usually cannot be completely cured, so the affected person should see a doctor early on. In the case of those who wish to have children, genetic testing and counseling may also be useful in order to prevent the recurrence of this disease. The disease is treated with the help of various therapies, although sufferers can also perform many of the exercises independently in their own homes. In general, a healthy lifestyle has a positive effect on the course of Nonne-Milroy-Meige syndrome. Ideally, those affected should avoid being overweight and pay attention to a healthy and balanced diet. Furthermore, regular check-ups and examinations by a doctor are very important to alleviate the symptoms and also to detect other damages at an early stage. Many patients depend on psychological support from their own family, whereby loving and intensive conversations in particular can have a positive effect on the further course of the disease and also prevent depression or other psychological upsets.
What you can do yourself
Since there are unfortunately no treatment options so far, the only thing left to do is to alleviate the symptoms and make everyday life easier for yourself. Basically helpful is to have a regular lymphatic drainage to relieve edema-related complaints such as feelings of tension and pain. Due to the variety of possible symptoms, there are hardly any generally valid recommendations. What is certain is that one should urgently try to avoid obesity and not put unnecessary strain on the cardiovascular system. For this purpose, a healthy lifestyle and a balanced diet are advisable, as well as sporting activity within the scope of possibilities. In particular, relatives of very young patients often suffer from self-doubt, even depression, which is why visiting self-help groups and also a psychologist again helps to provide the necessary stability in everyday life. Sometimes it is also recognized in such discussions that the family is overburdened with the care and the use of home care is advised. By applying for a severely disabled person’s card, the affected person can make use of the help of the integration specialist office and receives help there to help themselves, as well as help in work, care and authority matters. This is a great relief, especially in the initial period after the diagnosis and shows possibilities that were not considered before. Relatives of children with the disease also receive urgently needed help here for their new daily routine.
