Weber syndrome is a form of brainstem syndrome. It is often due to ischemic stroke secondary to thromboembolism. Typical consequences are hemiplegia, restricted eye motor function and other neurological damage.
What is Weber Syndrome?
Weber syndrome is one of the brainstem syndromes, all of which can be traced back to damage in the brain area of the same name. Doctor David Weber was the first to describe the condition, also known as midbrain foot syndrome. Damage to the substantia nigra in the midbrain and the disruption of certain nerve pathways are characteristic of Weber syndrome. For hydrocele overview, please visit homethodology.com.
The most affected are the corticopontine tract, which is connected to the pons and the cerebral cortex, the oculomotor nerve, which controls eye movements, and the pyramidal tract, which transmits motor signals to the body. The substantia nigra is a core area in the midbrain that has numerous connections to other brain areas.
These include, for example, the striatum, thalamus, globus pallidus, subthalamic nucleus and the cerebral cortex. The substantia nigra also plays an important role in its function within the extrapyramidal motor system. This system, like the pyramidal tract, controls movements.
Various causes can underlie Weber’s syndrome. In the majority of cases, the clinical picture is caused by an ischemic stroke, in which one or more blood vessels leading to the brain become blocked. As a result, the cells behind the closure receive little or no oxygen and nutrients, eventually causing them to die.
Since the brain is only able to regenerate nerve cells to a very limited extent, it cannot replace the dead cells: the brain is irreversibly damaged. Clogging of various blood vessels can contribute to the development of Weber syndrome. In the vertebral artery, blood flows not only to the brain but also to the dura mater and spinal cord.
The basilar artery represents one of its branches, which also divides into different branches. One of these, the posterior cerebral artery, can also cause Weber’s syndrome if it becomes blocked. Regardless of the blood vessel involved, the occlusion is often caused by blood clots.
Deposits in the blood vessels can form thrombi, which often tear loose and get stuck in constricted areas or in blood vessels that are becoming finer. Such embolisms can also be based on fat droplets blocking the blood vessel.
Symptoms, Ailments & Signs
In Weber syndrome, an oculomotor nerve palsy typically develops on the side of the brain damage (ipsilateral) : The oculomotor nerve is interrupted and can therefore no longer transmit nerve signals to the innervated eye muscles. In complete oculomotor palsy, the ipsilateral eye hangs down with the gaze directed outward.
Visual disturbances include forced dilation of the pupil ( mydriasis ), drooping of the eyelid ( ptosis ), impaired pupillary response, and accommodation disorders. There are two subtypes of partial oculomotor nerve palsy: internal and external paralysis.
The former manifests in mydriasis and accommodation disorders, while in external paralysis the affected eye is turned down and out. Hemiparesis on the side opposite to the brain damage (contralateral) is also one of the characteristic symptoms of Weber syndrome.
Depending on how badly the brain is damaged, the paralysis can take on different forms. It often occurs as spastic paralysis and is accompanied by dystaxia. Other symptoms depend on the affected nerves at the midbrain base.
Diagnosis & course of disease
As part of the diagnosis, doctors use imaging methods, such as computed tomography (CT) or magnetic resonance imaging (MRI). Both allow the affected tissue to be precisely localized and the extent of the damage to be assessed. In many cases, neurological tests carried out beforehand can already provide initial indications of the type of disease. Depending on the cause, the doctor treating you will also clarify whether other organs are affected or at risk.
Sturge-Weber syndrome favors malformations in the facial area. Those affected suffer from severe aesthetic losses, which leads to psychological problems. Inferiority complexes are common among patients and self-esteem declines rapidly. The level of suffering is particularly high at a young age, because many patients become victims of bullying.
Furthermore, the syndrome entails a limited sensitivity: numbness, indefinable pain and a tingling and burning sensation that cannot be localized aggravate the suffering. This is made even more difficult by paralysis in various parts of the body. Cataracts often form in connection with Sturge-Weber syndrome and the lens of the eye becomes significantly clouded.
A strong sensitivity to light is also a sign that the person is suffering from a cataract. Glaucoma often occurs, increasing the pressure in the eye to such an extent that the patient can go blind. Mental disabilities and developmental delays are also not uncommon, regardless of whether Sturge-Weber syndrome is treated or not. Emotional behavior disorders and learning disabilities are added.
It is rarely possible to carry out physical activities without outside help, which limits the independence and mobility of the patient. Severe headaches are common in Sturge-Weber syndrome and further reduce the quality of life of those affected. The blood vessels in the brain develop abnormally, and severe seizures and epilepsy further aggravate the health condition.
When should you go to the doctor?
If you have Weber syndrome, you should definitely see a doctor. It is a serious condition that definitely needs to be checked and treated by a doctor. The earlier the disease is recognized and treated, the better the further course of the disease will usually be. In most cases, aesthetic impairments or facial deformities indicate Weber’s syndrome. The disease can also significantly increase the risk of a stroke. If you have a stroke, you should call an ambulance or go to a hospital immediately.
Intellectual impairments of the affected person can also indicate this syndrome and must be examined by a doctor. The tumors on the face often lead to defects in the field of vision or to very severe headaches. First and foremost, a general practitioner can be consulted for Weber syndrome. The further examination is usually carried out in a hospital. Since the syndrome can lead to severe psychological upsets or depression, psychological treatment should also be carried out in any case. This disease may also reduce the life expectancy of those affected.
Treatment & Therapy
As a first therapeutic measure, doctors ensure the immediate care of the patient. An imaging procedure also shows whether intravenous thrombolysis or another immediate measure is possible. In some cases, this procedure allows the blockage of the blood vessel to be removed shortly after an ischemic stroke, thereby preventing further tissue death.
However, numerous factors influence its success and there is a risk of bleeding from intravenous thrombolysis. Intra-arterial lysis with a catheter (catheter intervention) may also be considered. If the cause of Weber syndrome is not a blocked blood vessel but bleeding, surgical interventions are an option that help to relieve the tissue.
After an ischemic stroke, about 40 percent of those affected die in the first year. In the long term, patients undergo extensive rehabilitation that includes occupational therapy and physiotherapy, speech therapy, (neuro)psychological and psychiatric treatments. The aim of these measures is to maintain the patient’s independence and remaining abilities as far as possible.
Some brain cells from other areas that are still intact can take over the tasks of the dead cells if the therapy actively stimulates and promotes them. Long-term treatment also includes preventing further strokes.
The prevention of Weber’s syndrome essentially corresponds to the general prevention of a stroke, since such a stroke is the cause of the brain damage in most cases. In this context, the treatment of other diseases such as diabetes mellitus, dyslipidemia, high blood pressure and others is particularly crucial.
Lifestyle factors that each person can influence include alcohol consumption, smoking habits, diet and exercise. Avoiding stress and obesity can also prevent strokes.
Those affected with Weber syndrome usually only have limited and only a few measures of direct follow-up care available, so that those affected with this disease should see a doctor very early on. The sooner a doctor is contacted, the better the further course of this disease is usually.
It is also a congenital disease, so it cannot be completely cured. For this reason, the person concerned should have a genetic examination and counseling carried out even if they wish to have children, in order to prevent the recurrence of Weber syndrome in their descendants. As a rule, various surgical interventions are necessary to limit the symptoms properly and permanently.
The person concerned should definitely rest and rest after such an operation, whereby no strenuous or stressful activities should be carried out. The support and care of one’s own family is also very important with this disease. This can also prevent mental upsets and depression. The further course of the syndrome depends very much on the exact severity of the disease, whereby in some cases the life expectancy of the person affected is reduced.
You can do that yourself
If there is a suspicion of Weber syndrome, the doctor must first be consulted. The rare disease occurs as a result of severe brain disorders, which is why rapid clarification is necessary. It is best to see a doctor as soon as the first typical symptoms appear.
After the diagnosis, depending on the symptoms, an individual therapy can be developed, which can be supported by those affected and their relatives. Physiotherapy can be supported at home by independent training. Endurance sports and stretching are important, although the exact measures depend on the respective symptoms. The training plan should be drawn up together with the responsible physiotherapist.
Weber syndrome can lead to a wide variety of secondary diseases. For example, epileptic seizures can occur. The relatives and the person concerned must take the necessary measures to reduce the risk of a fall or injury to a minimum. The tissue damage can also lead to motor disorders, which in turn can be treated with comprehensive physiotherapy. The self-help measures must be regularly adapted to the varying course of the disease in order to continue to improve the state of health.