Morgagni-Stuart-Morel syndrome, a photo of the manifestations of which can be seen in this article, is quite rare - only one hundred and forty get sick for one million people. In addition, in practice, specialists are faced mainly with “fragments” of the disease, because the disease itself develops unnoticed for many years, and the symptoms manifest themselves at different rates. Patients have to be examined by more than one doctor before the syndrome manifests itself completely and an accurate diagnosis is determined.
The disease reaches a critical state when pathological irreversible changes in the body are already formed. After that, it is almost impossible to help the patient.
History
Morgagni-Stuart-Morel syndrome was carefully described in 1762, when the Italian pathologist Morgagni opened the corpse of a forty-year-old woman and found a thickening of the inner frontal plate of the bone. At the same time, he noted obesity and excessive body hair. Morgagni considered the signs interconnected and described his observations in detail. Morel and Stuart later drew attention to a combination of symptoms and were able to add various neurological and mental manifestations to the clinical picture.
What is an ailment?
Morgagni-Morel-Stuart syndrome is a disease in which hyperostosis of the internal frontal bone plate, obesity and a number of other endocrine and metabolic disorders are detected. The disease in the vast majority occurs in women, mainly after forty years, but sometimes it manifests up to thirty. Among the males, the syndrome is practically not found, with rare exceptions.
Etiology and pathogenesis
Morgagni-Morel-Stuart disease is a syndrome whose pathogenesis and etiology do not have a single definition. Some authors believe that pathology is genetically determined and is transmitted by a dominant-autosomal type. It is believed that the syndrome is closely related to the X chromosome. There have been cases when the disease has suffered in four generations, and mostly women.
There are isolated cases of the manifestation of the syndrome in men. But they have a sporadic character, and science does not yet know how the disease is transmitted through the male line. There was an opinion that the syndrome is more often manifested in women during the menopause. But the data accumulated over time puts this statement into question.
The disease can occur due to infections (mainly sinusitis), neuroinfections, and head injuries. Morgagni-Stewart-Morel syndrome was found in female castrates who underwent surgery. Individual symptoms of the syndrome may form after cancer treatment.
There are cases when the disease was detected in patients with:
- uterine myoma;
- sclerocystic ovary;
- persistent galactorrhea;
- genital infantilism.
Morgagni-Morel-Stuart syndrome: pathology
With the disease, not only does the inner frontal bone plate thicken, but sometimes hyperostosis in the occiput and fronto-occipital region is detected. Many microscopic changes appear in the endocrine glands. The number of eosinophilic and basophilic cells is increasing. Adenomatous growths begin (thyroid glands, adrenal cortex, etc.).
Symptoms
When a person develops Morgagni-Morel-Stuart syndrome, there are a number of symptoms characterizing him:
- Patients begin to experience regular headaches.
- Obesity, mainly in the abdomen.
- The skin remains normal, but pyoderma and weeping eczema often appear on it.
- Most patients have severe hypertrichosis.
- Pastosity is noted on the skin, lower extremities begin to swell, acrocyanosis appears.
- The muscles are weakening.
- There are changes in the cardiovascular system and respiratory system (expanding the borders of the heart, there is a dull tone and heart failure).
- Tendency to collapse, catarrh of the upper respiratory tract and bronchopneumonia.
- Changes in the endocrine glands do not have characteristic features. Exchange can be normal, increased or decreased.
- Tolerance to carbohydrates decreases. Often diabetes mellitus is clearly manifested.
- The menstrual cycle is broken. During pregnancy during this period, the patient's condition worsens.
- Nervous and mental disorders are observed. They manifest in the form of emotional lability, irritability, insomnia and a tendency to depression.
- The inner frontal plate has hyperostosis, often the bones of the skull thicken. In some cases, the size of the Turkish saddle is even increasing.
Course of the disease
Morgagni-Morel-Stuart syndrome can affect the body in different ways. It often depends on the degree of obesity. Pronounced has a big impact on life expectancy:
- heart activity is difficult;
- due to the accumulation of fat, myocardium and pericardium arise;
- high aperture position;
- blood circulation is disturbed, and in combination with atherosclerosis and hypertension, heart failure develops, which is the cause of disability;
- if the patient with the syndrome also has diabetes, then a metabolic disorder begins , which leads not only to a decrease in working capacity, but also shortens life expectancy.
Treatment
Is it possible to eliminate Morgagni-Stewart-Morel syndrome? The treatment of the disease consists mainly in following a diet similar to that prescribed for diabetes and obesity. Food should be low in calories, rich in proteins, vitamins and mineral salts. In the diet, the amount of carbohydrates and fats is reduced. Basically, the Baranov diet is prescribed.
Additionally, during treatment, massage is performed and physical exercises are prescribed that reduce muscle weakness and improve peripheral blood circulation. If the patient has heart failure, then the treatment of the syndrome is carried out by diuretics, cardiopreparations, etc.