Morgan Stewart syndrome on post-mortem examination. Syndrome of persistent lactation and amenorrhea. Morgagni-Stuart-Morel syndrome. Morgagni-Adams-Stokes syndrome: symptoms, emergency care and treatment

Morgagni-Stuart-Morel syndrome(metabolic craniopathy) - a condition associated with a wide range endocrine problems, including diabetes and diabetes insipidus, and hyperparathyroidism. Other signs and symptoms include headache, dizziness, excessive male pattern hair, menstrual problems, galactorrhea, obesity, depression, and seizures. Thickening of the inner surface of the frontal part of the skull is usually initial stage disease known as hyperostosis frontalis interna ( English) .

Discovery history

The syndrome was first described in 1765 by the Italian pathologist Giovanni Battista Morgagni and later, in 1928, by the English neurologist Douglas Stewart. Swiss physician Ferdinand Morel (Polish) Russian (1888-1957) in 1930 added violations to the clinical picture menstrual cycle and impotence. The term "Morgagni-Stuart-Morel syndrome" was introduced in 1937 by Folke Henschen sv.

Etiology and pathogenesis

The etiology of the disease is not fully understood, but it is associated with hypertrophy of the adrenal cortex due to hypersecretion of adrenocorticotropic hormone, the influence of which causes secondary symptoms. Similar symptoms were noted in women with elevated prolactin against the background of hyperostosis of the frontal bone in 43% of cases. A number of studies have shown autosomal dominant inheritance of the disease. The disease is typical mainly for women.

Diagnostics

The diagnosis is made on the basis of complaints and the clinical picture; also use histological methods of research. X-ray shows thickening of the frontal bone. Morgagni-Stuart-Morel syndrome is differentiated primarily from Itsenko-Cushing's disease and adiposogenital dystrophy.

Treatment

Treatment is symptomatic. In rare cases, the overgrown inside of the frontal bone may be surgically removed.

Syndrome of Morgai and - Stewart - Morel, or hyperostosus frontalis interna. Morgagni-Stewart-Morel syndrome is a thickening of the inner side of the frontal bone, combined with obesity, arterial hypertension, virilism, sometimes with diabetes. Such patients experience headaches, weakening of vision, memory, severe asthenia, sometimes various mental disorders. The x-ray shows a thickening of the inner part of the frontal bone. In patients with Morgagni-Stuart-Morel syndrome, recurrent inflammation of the paranasal cavities is often observed. The pathogenesis of the disease has not been established; a particular dysfunction of the pituitary-hypothalamic mechanisms is assumed.

There are forms of the disease in which, along with hyperostosis of the frontal bone, an X-ray image shows a narrowing of the internal auditory canal, ossification of the petrosal-sphenoid ligament, a small fossa of the Turkish saddle; in some patients, mildly pronounced spondylosis of the cervical vertebrae is determined. With these forms of the syndrome, dizziness, tinnitus and dysfunction may occur. vestibular analyzer, predominantly of peripheral origin (labyrinth, nerve). Anosmia, paralysis can be observed at the same time facial nerve, diplopia, amblyopia.

marble disease. The basis of this disease is pathological condition skeletal system, manifested in the imbalance between the organization and resorption of the substance. Due to the suppression of the resorption process, the amount of spongy substance decreases sharply. In marble disease, a violation of the mineral metabolism of the skeletal system was established, which manifests itself in the form of an incorrect ratio between various types calorie compounds. An objective examination in patients with marble disease often shows an increase in the liver, spleen and lymph nodes. With severe marble disease, generalized osteosclerosis is observed, chronic anemia and pathological fractures. With marble disease, the development of the bones of the skull is disturbed: they become compacted (pyramids of the temporal bone, mastoid process). The consequence of this thickening is a significant reduction in size and even obliteration of the accessory cavities of the nose, the disappearance of pneumatization of the mastoid processes, as well as changes in the configuration of the Turkish saddle and all the openings of the skull.

A 40-year-old patient complains of headache and hearing loss.

Objective examination: a peculiar configuration of the skull with an overhang of the frontal-occipital regions, kyphoscoliosis lumbar spine. Peripheral paresis of the right facial nerve, minor paresis of the left hypoglossal nerve. The hard palate is high and narrow. The voice is peculiar, devoid of overtones. auricles small, their shape is irregular. ear canals very narrow. Sound in Weber's experience does not lateralize. Bone conduction from the top of the head is slightly shortened. Perception (air) of tuning forks with the right ear: Ci2s - 30%, C2o48 - 100%, with the left ear: Ci2s - 17%, C2048 - 0. Perceives whispered speech with the right ear at a distance of 1.5 m. Does not perceive whispered speech with the left ear, loud hears speech near the auricle.

X-ray examination: a sharp compaction of the bones of the base of the skull, to a lesser extent the vault of the skull. Adnexal cavities noses are missing. Pneumatization of the mastoid process is absent. Turkish saddle with a small lumen and club-shaped swelling of the posterior and anterior sphenoid processes. All openings of the base of the skull are significantly narrowed.

Marked changes bone tissue characteristic of marble disease. The very name "marble disease" is given on the basis of changes in long tubular bones, consisting in the picture of alternating areas of osteoporosis and osteosclerosis. In our case, we have atypical form of this disease: minor changes in long tubular bones with at the same time sharply expressed violations of the structure of the skull.

The lesion of the facial, hypoglossal, optic and both auditory nerves observed in the patient is apparently due to compression of these nerves in the region of the exit openings.

The 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 fall ill per million people. In addition, in practice, specialists mostly encounter “fragments” of the disease, because the disease itself develops imperceptibly for many years, and the symptoms manifest themselves at different speeds. Patients have to be examined by more than one doctor before the syndrome manifests itself completely and an accurate diagnosis is determined.
The critical state of the disease reaches when irreversible pathological changes in organism. 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 internal plate of the frontal bone. At the same time, he noted obesity and excessive hair growth. Morgagni considered the signs to be interconnected and described his observations in detail. Morel and Stewart later drew attention to the combination of symptoms and were able to add to clinical picture various neurological and mental manifestations.

What is a disease

Morgagni-Morel-Stewart 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 overwhelming majority of the disease occurs in women, mostly after forty years, but sometimes it manifests itself before thirty. Among the males, the syndrome practically does not occur, with rare exceptions.

Etiology and pathogenesis

Morgagni-Morel-Stewart disease is a syndrome whose pathogenesis and etiology do not have a single definition. Some authors believe that the pathology is genetically determined and is transmitted by a dominant autosomal type. There are opinions that the syndrome is closely related to the X chromosome. There were cases when diseases suffered in four generations, and predominantly women. There are isolated cases of manifestation of the syndrome in men. But they are sporadic, 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 menopause. But the data accumulated over time cast doubt on this assertion. The disease can occur due to infections (mainly sinusitis), neuroinfections and traumatic brain injuries. Morgagni-Stewart-Morel syndrome was found in female castrati who had undergone surgical operation. Separate signs of the syndrome may form after cancer treatment.
Cases were recorded when the disease manifested itself in patients with:

Morgagni-Morel-Stewart syndrome: pathology

With the disease, not only the internal frontal bone plate thickens, but sometimes hyperostosis is detected in the back of the head and fronto-occipital region. There are many microscopic changes in the endocrine glands. The number of eosinophilic and basophilic cells increases. Adenomatous growths begin ( thyroid gland, adrenal cortex, etc.).

Symptoms

When a person has Morgagni-Morel-Stewart syndrome, there are a number of symptoms that characterize him:

Course of the disease

Morgagni-Morel-Stewart syndrome can affect the body in different ways. It often depends on the degree of obesity. Pronounced has a huge impact on life expectancy:

Treatment

Can Morgagni-Stewart-Morel syndrome be eliminated? Treatment of the disease consists mainly in following a diet similar to that prescribed for diabetes and obesity. Food should be low-calorie, rich in proteins, vitamins and mineral salts. The diet reduces the amount of carbohydrates and fats. Basically, Baranov's diet is prescribed.
Additionally, during the treatment, massage is performed and prescribed physical exercise, which reduce muscle weakness and improve peripheral circulation. If the patient has heart failure, then the syndrome is treated with diuretics, cardio drugs, etc.

Morgagni-Adams-Stokes syndrome- fainting caused sharp decline cardiac output and cerebral ischemia due to acute cardiac arrhythmias (atrioventricular blockade of the 2nd degree or complete atrioventricular blockade, paroxysmal tachycardia, ventricular fibrillation, weakness and dullness syndrome of the sinoatrial node, etc.). Named for the Italian Giovanni Battista Morgagni (1682-1771) and the Irish Robert Adams (1791-1875) and William Stokes.

Etiology and pathogenesis

Morgagni-Adams-Stokes syndrome occurs due to cerebral ischemia with a sudden decrease in cardiac output due to heart rhythm disturbances or a decrease in heart rate. Their reason may be ventricular tachycardia, ventricular fibrillation, complete AV block, and transient asystole.

Morgagni-Adams-Stokes syndrome sometimes occurs with sick sinus syndrome, carotid sinus hypersensitivity, and brain steal syndrome. Symptoms of impaired consciousness appear 3-10 seconds after circulatory arrest

Clinical picture

Attacks usually come on suddenly, rarely last more than 1-2 minutes, and, as a rule, do not entail neurological complications. Acute myocardial infarction or cerebrovascular accident can be both a cause and a consequence of the Morgagni-Adams-Stokes syndrome.

At the beginning of the attack, the patient suddenly turns pale and loses consciousness; after the restoration of consciousness, severe hyperemia of the skin often appears. Outpatient ECG monitoring often allows determining the cause of seizures.

Treatment

If tachyarrhythmias are the cause of the syndrome, appropriate antiarrhythmic drugs should be prescribed. If attacks occur due to bradycardia (most often with complete AV block), permanent pacing is indicated. If the exacerbation of the syndrome is caused by complete AV block with a slow ventricular escape rate, for emergency treatment, you can use intravenous administration isoproterenol or adrenaline to increase heart rate. It is preferable to use isoproterenol, as it has a more pronounced positive chronotropic action, less often causes ventricular arrhythmias and does not lead to an excessive rise in blood pressure.

Patients with prolonged or recurrent bradyarrhythmias may require temporary or permanent pacing.

see also

Notes

Links

Wikimedia Foundation. 2010 .

See what "Morgagni Syndrome" is in other dictionaries:

Morgagni-Stewart-Morel-Henschen syndrome- See Morgagni's disease. Hyperostosis of the internal plate of the frontal bone, verilism, obesity, amenorrhea. It is observed only in women. Described by the Italian physician and anatomist Morgagni ...

Morgagni-Adams-Stokes syndrome- Fainting (see), which occurs against the background of a complete atrioventricular blockade, caused by impaired conduction in the bundle of His and provoking ischemia of the brain, in particular, the reticular structures of its trunk. It manifests itself as an instant general ... ... encyclopedic Dictionary in psychology and pedagogy

MORGANIA SYNDROME- (Morgagni-Stuart-Morel syndrome, described by the Italian physician and anatomist G. B. Morgagni, 1682-1771, later by the British neurologist R. M. Stewart, the Swiss doctor F. Morel, 1888-1957; synonym - internal frontal hyperostosis syndrome) - ... ... Encyclopedic Dictionary of Psychology and Pedagogy- (G. B. Morgagni, 1682 1771, Italian doctor and anatomist; R. Adams, 1791 1875, Irish doctor; W. Stokes, 1804 1878, Irish doctor; synonym: Adams Morgagni Stokes syndrome, Spence syndrome, Stokes syndrome) the occurrence of attacks of sudden loss of consciousness with ... ... Big medical dictionary

- (G. B. Morgagni, 1682 1771, Italian doctor and anatomist; R. M. Stewart, English neuropathologist of the 20th century; F. Morel, 1888 1957, Swiss psychiatrist) see Morgagni's syndrome ... Big Medical Dictionary

- (G. B. Morgagni, 1682 1771, Italian doctor and anatomist; S. E. Henschen, 1847 1930, Swedish doctor) see Morgagni's syndrome ... Big Medical Dictionary

SINUS WEAKNESS SYNDROME- honey. Sinus atrial node weakness syndrome (SSSPU) is the inability of the sinoatrial node (SAN) to adequately perform the function of the center of automatism. Partial or complete loss of the role of the central pacemaker of the heart leads to the emergence of ... Disease Handbook

In 1855 Chiari described persistent lactation and amenorrhea with atrophy of the uterus and ovaries after childbirth. The syndrome was further studied in detail by Frommel, Argonnz A. Del Castillo and Forbes.

According to modern ideas syndrome of persistent lactation and amenorrhea should be considered as a syndrome of damage to the hypothalamus by a hormonally inactive tumor or other pathological process.

pathological anatomy. An autopsy may reveal a chromophobe pituitary adenoma. The uterus is hypoplastic (atrophic changes in the uterine mucosa), ovarian atrophy is pronounced. The macro- and microscopic picture of the mammary glands can be different depending on the degree of hyperplasia and secretion of the organ.

Clinic. The Chiari-Frummel syndrome is characterized by a triad: 1) amenorrhea, 2) galactorrhea, 3) impaired hypothalamic-pituitary function (pituitary dysfunction). Often, lactation disorders can be combined with mental disorders, which confirms the neurogenic origin of the syndrome. Chiari-Frummel syndrome may be associated with obesity or malnutrition, diabetes insipidus. With compression or germination by a tumor of the chiasm optic nerves Bitemporal hemianopsia occurs.

Diagnosis and differential diagnosis. The diagnosis of the syndrome is not difficult. Similar symptoms are observed with chromophobic adenoma, craniopharyngioma.
Forecast depends on the severity of the disease.

Treatment. effective method treatment is prompt removal tumors or tumor treatment with radioactive drugs (radioactive gold, etc.). To reduce lactation, symptomatic treatment with preparations of female sex hormones (diethylstilbestrol, etc.) is carried out.

Morgagni-Stewart-Morel syndrome

This syndrome was first described in 1719. Italian pathologist Morgagni. Ashard and Thiers in 1921, Stewart in 1928, Morel about 1930 were engaged in further study of this syndrome. The syndrome is observed almost exclusively in women in adulthood (over 50 years old), sometimes occurs in younger women - up to 30 years old .
Etiology unclear. It is believed that the main cause of the disease are hypothalamic-pituitary disorders.

Pathogenesis disease has not yet been fully elucidated. Of certain importance, apparently, is the increased production of estrogens, as well as hormones produced by basophilic and eosinophilic cells of the anterior lobe.

pathological anatomy. At histological examination an increase in the number of eosinophilic and basophilic cells is found, as well as pituitary microadenomas, consisting of eosinophilic, basophilic and chromophore cells. Microscopic changes in other endocrine glands (adenomas of the thyroid gland, parathyroid gland and adrenal cortex) accompany this disease.

Clinic. Patients' complaints of severe and persistent headache are one of the earliest complaints. Headache likely due to hypertension and intracranial pressure. Patients are extremely excited, there is a tendency to depression. Sleep is poor, with frequent waking. Skin of normal color. Obesity resembling obesity in adipose-genital dystrophy. Often there is pyoderma, weeping eczema, acrocyanosis. Hypertrichosis. The boundaries of the heart are expanded. Heart sounds are muffled. Accent II tone over the aorta. Often observed arterial hypertension. Patients are prone to bronchopneumonia, acute respiratory infections.

Often can be observed disorder of the menstrual cycle, however, despite this, pregnancy sometimes occurs, which worsens the course of the disease, ROE is accelerated. Decreased tolerance to carbohydrates is noted. Sometimes Morgapy-Stewart-Morel syndrome is combined with diabetes insipidus. The main exchange is changeable. The Turkish saddle is usually not changed. There is a thickening of the inner plate of the frontal bone.
Meet erased forms of the syndrome, especially with menopause, which makes it difficult to make a diagnosis.

Morgagni-Stewart-Morel syndrome characterized by Hepshen's triad: obesity, male-pattern hair growth, and thickening of the inner plate of the frontal bone. In the differential diagnosis, one should first of all keep in mind adipose-genital dystrophy and Itsenko-Cushing's disease, in which there are similar signs.

Forecast and work capacity. The working capacity of patients mainly depends on a number of reasons, especially on the degree of obesity. With severe obesity, due to the deposition of fat in the pericardium and myocardium, the work of the heart becomes more difficult, which creates conditions for the development of heart failure and thus "aggravates the patient's condition, and this leads to complete disability.

Treatment. In mild forms of the disease, the ability to work is limited. Treatment comes down to diet. With complications of diabetes mellitus, treatment is carried out according to general scheme treatment diabetes. A diet rich in complete proteins is recommended, mineral salts and vitamins. Fats and carbohydrates should be limited. Physical education and massage are of additional importance. With complications from internal organs(heart failure, pneumonia, etc.) treatment is generally accepted in these cases.