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Weber syndrome
Medical expert of the article
Last reviewed: 04.07.2025
A disease such as Weber 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 damage to the facial and hypoglossal nerves. Sometimes the disease is complicated by hemianopsia.
Weber syndrome is a rather complex and rare disease, and is one of the variants of neurological pathology from the category of peduncular alternating syndromes.
Causes Weber syndrome
The onset of the disease is associated with pathological changes occurring in close proximity to the cerebral peduncles. Such changes may be the result of cerebral circulation disorders (cerebral ischemia), disruption of the integrity of the brain vessels, and tumor processes.
In addition, the development of pathology may be associated with localized pressure of the neoplasm on the cerebral peduncles, even if the tumor is located at some distance from this area.
Pathogenesis
The pathogenesis of the syndrome may include:
- in a disorder of blood circulation within the main arterial vessel;
- in a large-scale expanding process in the temporal lobe of the brain;
- in the development of an inflammatory process in the meninges in the area of the base of the brain;
- in subarachnoid hemorrhage in the middle cranial fossa.
Symptoms Weber syndrome
The first signs of the disease are increasing paralysis of the facial muscles, tongue muscles, arms and legs in the central variant. Clinical symptoms are explained by absolute or partial immobilization of the oculomotor nerve. Muscle disorder leads to forced deviation of the eyeball to the temporal side. It looks as if the eye "looks" in the opposite direction from the affected side.
When the visual system pathways are simultaneously affected, hemianopsia occurs – bilateral blindness of half the visual field. The patient has wide strabismus, visual function declines, colors and shades are distinguished with great tension.
In addition, intense and rhythmic clonus-type movements caused by pushing muscle contractions may be detected. Over time, the patient's condition worsens: the function of flexion of the wrist at the level of the protective reflex is impaired.
Forms
Weber syndrome is an alternating syndrome, the essence of which is a functional disorder of the cranial nerves on the side of the injury, as well as a disorder of motor activity (in the form of paresis and paralysis), loss of sensitivity (conduction variant) and coordination of movements.
Depending on the location of the pathological focus, such syndromes are divided into the following types:
- peduncular syndrome (with damage to the base, or legs, of the brain);
- pontine syndrome (bridge pathology);
- bulbar syndrome (damage to the medulla oblongata).
Weber syndrome is classified as a peduncular type of the disease.
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Klippel-Trenaunay-Weber syndrome
Klippel-Trenaunay-Weber syndrome is similar to the Weber syndrome we are describing only in name. The essence of the disease is significantly different: the pathology is associated with a congenital defect in the vascular system, which is laid down in the embryonic period.
The disease is characterized by the appearance of a nevus on the limb of the telangiectasia type, against the background of varicose veins and venous-arterial anastomoses on the affected side. Cases of partial gigantism of the affected leg or (less often) arm are not uncommon. Some patients have curvature of the spine, hip dislocation, deformative changes in the joints and feet. The vessels of the visual organs, lungs, and kidneys also change.
Treatment of the pathology is carried out surgically.
Another name for Klippel-Trenaunay syndrome is Parkes-Weber-Rubashov syndrome, or simply Weber-Rubashov syndrome.
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Sturge-Weber-Krabbe syndrome
Another hereditary syndrome, Sturge-Weber-Krabbe, is characterized by the following symptoms that appear immediately after the baby is born:
- multiple angiomas (vascular formations) on the skin, sometimes nevi;
- damage to the vessels of the visual organs, which leads to an imbalance of fluid in the eye cavity and glaucoma. As a consequence - changes in the eye membranes, retinal detachment, blindness;
- damage to the vessels of the meninges, the appearance of angiomas on the pia mater, hemiparesis (immobilization of half of the body), hemianopsia (one-sided loss of vision), hyperactivity, cerebrovascular accidents, motor coordination disorders, convulsive syndrome, intellectual disabilities.
Treatment of the syndrome is symptomatic.
Otherwise, the syndrome is called encephalotrigeminal angiomatosis.
Weber-Osler syndrome
The exact name for Weber-Osler syndrome is Rendu-Weber-Osler disease.
The basis of this pathology is a deficiency in the transmembrane protein endoglin, which is an ingredient of the receptor system of transforming growth factor β. The disease is transmitted by autosomal dominant inheritance and is characterized by the following symptoms:
- numerous angioectasias of a purple-red hue, 1-3 mm in size;
- dilated cutaneous capillary vessels;
- frequent nosebleeds.
The syndrome manifests itself already in infancy, worsening after the onset of puberty.
Diagnostics Weber syndrome
Diagnosis of Weber syndrome can be difficult. Unfortunately, there are no specific methods that can determine the disease 100%. Therefore, it is necessary to use a full diagnostic complex in order to establish a correct diagnosis.
- Laboratory diagnostics are based on the use of various methods of examining brain functions. Weber's syndrome does not produce any changes in the composition of urine or blood, so the corresponding tests are considered uninformative. Sometimes a spinal puncture may be prescribed with the collection of fluid for further examination. At the same time, the pressure of the cerebrospinal fluid is determined.
- Instrumental diagnostics includes:
- assessment of the vascular network of the fundus (swelling, fullness, vascular spasm, presence of hemorrhages);
- neurosonography (ultrasound examination of the structural components of the brain, for example, the brain cavities - ventricles);
- computed tomography and NMR – a method of resonant absorption or emission by electromagnetic matter.
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Treatment Weber syndrome
Treatment of Weber syndrome should be aimed at eliminating the main cause of pathological changes in the base of the brain. Therefore, the focus of treatment is therapy of cerebral circulation disorders, vascular disorders, inflammatory processes of the meninges, removal of tumors, aneurysms, etc.
The following may be assigned:
- anticonvulsants or psychotropic drugs;
- medications to reduce intracranial and intraocular pressure.
If necessary, doctors resort to surgical intervention to correct vascular and structural disorders.
Currently, one of the most effective methods of treating alternating syndromes of any origin is considered to be stem cell transplantation.
Stem cells after transplantation into the brain activate tissue restoration (including nerve tissue), which creates extremely favorable conditions for the treatment and regeneration of damaged brain structures. After the final restoration of brain tissue, the course of Weber's syndrome is significantly alleviated.
Prevention
Weber syndrome is not an independent pathology: as a rule, it is 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 disorders of intracerebral circulation.
What recommendations does this type of prevention include?
- You should lead a healthy lifestyle, determining the optimal physical activity for yourself.
- It is important to give up smoking and alcohol abuse.
- It is necessary to support the body's immune system, periodically take multivitamin and mineral complexes, harden yourself and ensure the body's physical activity.
In addition, doctors recommend developing stress resistance and avoiding conflict situations. All these tips will help preserve the nervous system and prevent pathological changes.
Forecast
The prognosis with timely medical care and a small degree of pressure on the cerebral peduncles can be relatively favorable. It is worse if the lesion is extensive or caused by a tumor process. In this case, the impaired brain functions may not be restored.
The consequences of Weber syndrome can be varied:
- complete and irreversible blindness may occur;
- neuroses, psychoses, and even dementia may occur;
- a comatose state and even death may develop.
A patient with symptoms of such a disease as Weber's syndrome must be under constant medical supervision. Even with a relatively stable condition, without visible deterioration, one should not lose vigilance: negative consequences may arise over time.