Weber Syndrome

Such a disease, as Weber's syndrome, was first described by the English doctor Herman David Weber in the 19th century. The main characteristics of the syndrome are: unilateral oculomotor paralysis, hemiplegia and hemiparesis, as well as lesion of the facial and sublingual nerve. Sometimes the disease is complicated by hemianopsia.

Weber's syndrome is a rather complicated and rare disease, and represents one of the variants of neurological pathology from the category of peduncular alternating syndromes.

Epidemiology

The epidemiology of Weber's syndrome has not been investigated.

[1], [2]

Causes of the weber's syndrome

The appearance of the disease is associated with pathological changes occurring in the immediate vicinity of the legs of the brain. Such changes can be the result of cerebral circulation disorder (cerebral ischemia), disruption of the integrity of the brain vessels, tumor processes.

In addition, the development of pathology may be associated with localized neoplastic pressure on the legs of the brain, even if the tumor is located some distance from this region.

[3], [4],

Risk factors

We can distinguish the following risk factors for Weber's syndrome:

• ischemic or hemorrhagic stroke;
• benign or malignant neoplasms of the brain;
• craniocerebral trauma.

[5], [6]

Pathogenesis

The pathogenesis of the syndrome can be:

• in the disorder of blood circulation inside the main arterial vessel;
• in a large-scale growing process in the temporal lobe of the brain;
• in the development of the inflammatory process in the meninges in the region of the base of the brain;
• in a subarachnoid hemorrhage into the middle cranial fossa.

[7], [8], [9], [10], [11]

Symptoms of the weber's syndrome

The first signs of the disease - is the growing paralysis of the facial musculature, muscles of the tongue, hands and feet according to the central variant. The clinical symptomatology is explained by the absolute or partial immobilization of the oculomotor nerve. Muscular dysfunction leads to the forced deflection of the eyeball on the temporal side. It looks like the eye "looks" in the opposite direction from the affected side.

With simultaneous damage to the visual system, hemianopsia occurs - a double blindness of half the field of vision. The patient has wide strabismus, the visual function falls, colors and tints differ with greater stress.

In addition, intensive and rhythmic movements of the clonus type can be detected, caused by pushing muscle contractions. Over time, the patient's condition worsens: the function of flexion of the hand is violated at the level of the protective reflex.

Forms

Weber's syndrome refers to alternating syndromes, the essence of which is the functional breakdown of the cranial nerves on the part of the lesion, as well as in the disturbance of motor activity (in the form of paresis and paralysis), loss of sensitivity (conductor variant), and coordination of movements.

Depending on the location of the pathological focus, these syndromes are divided into the following types:

• peduncular syndrome (with damage to the base, or legs, brain);
• Pontine syndrome (bridge pathology);
• bulbar syndrome (lesion of the medulla oblongata).

Weber's syndrome is referred to as peduncular varieties of the disease.

[12], [13], [14], [15]

Clippel-Trenone-Weber Syndrome

The syndrome of Klippel-Trenone-Weber is similar to the Weber syndrome described by us only by name. The essence of the disease is significantly different: pathology is associated with an inborn defect in the vascular system, which is laid in the embryonic period.

The disease is characterized by the appearance on the extremity of the nevus by the type of telangiectasia, against the background of varicose veins and by venous-arterial anastomoses from the side of the lesion. Cases of partial gigantism of the affected leg or, more rarely, of the hand are not uncommon. In some patients, there is a curvature of the spine, hip dislocation, deformative changes in the joints, and feet. Vessels of visual organs, lungs, and kidneys also change.

Treatment of pathology is performed promptly.

The second name for the Klippel-Trenone syndrome is Parkes-Weber-Rubashov syndrome, or simply Weber-Rubashov syndrome.

[16], [17], [18], [19]

The Sturge-Weber-Crabbe Syndrome

Another hereditary Sturge-Weber-Crabbe syndrome is characterized by such signs that appear immediately after the birth of the baby:

• multiple angiomas (vascular formations) on the skin, sometimes - nevuses;
• damage to the vessels of the visual organs, which leads to an imbalance of fluid in the eye cavity and glaucoma. As a consequence - a change in the ocular membranes, retinal detachment, blindness;
• damage to the vessels of the meninges, the appearance of angiomas on the soft membrane, hemiparesis (immobilization of the half of the trunk), hemianopia (one-sided loss of vision), hyperactivity, disorders of cerebral circulation, motor coordination disorders, convulsive syndrome, intellectual development disorders.

Symptomatic treatment is symptomatic.

Otherwise, the syndrome is called encephalotrigeminal angiomatosis.

Weber-Osler Syndrome

The exact name of the Weber-Osler syndrome is Rundu-Weber-Osler disease.

The basis of this pathology is the lack of endogenous transmembrane protein, which is an ingredient in the receptor system of the transforming growth factor β. The disease is transmitted by autosomal dominant inheritance and is characterized by the following symptoms:

• numerous angioectasies of violet-red hue, 1-3 mm in size;
• dilated capillary vessels;
• frequent nasal bleeding.

The syndrome manifests itself already in infancy, aggravated after the onset of puberty.

Diagnostics of the weber's syndrome

Diagnosis of Weber's syndrome may present certain difficulties. Unfortunately, there are no specific methods that completely determine the disease. Therefore, you have to use a full diagnostic complex in order to correctly establish the diagnosis.

• Laboratory diagnosis is based on the use of various methods of examination of brain functions. Weber's syndrome does not give any changes in the composition of the urinary fluid or blood, so the corresponding analyzes are considered uninformative. Sometimes a spinal puncture can be prescribed with taking a fluid for further investigation. At the same time, the pressure of the CSF is determined.
• Instrumental diagnostics includes:
  • assessment of the vascular network of the fundus (puffiness, fullness, vascular spasm, the presence of hemorrhages);
  • neurosonography (ultrasound examination of the structural components of the brain, for example, cerebral cavities - ventricles);
  • computer tomography and NMR - a method of resonant absorption or radiation by an electromagnetic substance.

[20]

Differential diagnosis

Differential diagnosis is carried out with ophthalmic diseases, acute violation of cerebral circulation, tumor neoplasms.

[21], [22], [23], [24]

Treatment of the weber's syndrome

Treatment of Weber's syndrome should be aimed at eliminating the main cause of pathological changes in the area of the base of the brain. Therefore, the focus of treatment is the therapy of cerebral circulation disorders, vascular disorders, inflammatory processes of the meninges, removal of tumoral tumors, aneurysms,

Can be appointed:

• anticonvulsant or psychotropic drugs;
• drugs to reduce intracranial and intraocular pressure.

If necessary, doctors resort to surgical intervention - correction of vascular and structural disorders.

At present, one of the most effective ways of treating alternating syndromes of any origin is stem cell transplantation.

Stem cells after transplantation in the brain activate the restoration of tissues (including nerve tissues), which creates extremely favorable conditions for the treatment and regeneration of damaged brain structures. After the final restoration of the brain tissue, the syndrome of Weber is greatly facilitated.

Prevention

Weber's syndrome is not an independent pathology: it is usually a consequence or complication of other diseases or injuries associated with circulatory disorders in the brain and spinal cord. For this reason, preventive measures to prevent the development of the syndrome should be aimed at preventing various violations of the cerebral circulation.

What recommendations does this prophylaxis include?

• You should lead a healthy lifestyle, determining for yourself the optimal physical activity.
• It is important to stop smoking and abuse of alcoholic beverages.
• It is necessary to maintain the body's immune forces, periodically take multivitamin-mineral complexes, temper and provide motor activity of the body.

In addition, doctors recommend developing a resilience in themselves, avoid conflict situations. All these tips will help to save the nervous system and prevent pathological changes.

[25], [26], [27]

Forecast

The prognosis with timely rendered medical care and with a small degree of pressure on the legs of the brain can be relatively favorable. Worse, if the lesion is extensive, or caused by a tumor process. In this case, impaired brain functions may not recover.

The consequences of Weber's syndrome may be different:

• full and irreversible blindness may occur;
• can attach neuroses, psychoses, up to dementia;
• can develop a coma, and even death.

A patient with signs of a disease such as Weber's syndrome should always be under constant medical supervision. Even with a relatively stable condition, without any apparent deterioration, one should not lose vigilance: negative consequences can arise even after a while.