Gliomatosis cerebri is a primary type of brain tumor. Gliomatosis cerebri is characterized by a diffuse infiltration in the tissue of the brain, which is also typical for other gliomas. Due to the extent of this infiltration, the solid structures of the tumor play a subordinate role. According to abbreviationfinder.org, gliomatosis cerebri is a very rare disease, although a high number of unreported cases can be assumed due to the low level of awareness of the disease.

What is cerebrospinal gliomatosis?

In principle, gliomatosis cerebri occurs with a low prevalence. There are some clinical parallels between gliomatosis cerebri and certain types of encephalitis. It is a primary brain tumor characterized by diffuse infiltration. Physician Nevin first described gliomatosis cerebri in 1938.

So far only about 200 cases of gliomatosis cerebri are known all over the world. Gliomatosis cerebri usually occurs in adult patients. Gliomatosis cerebri occurs more frequently in people of advanced age. In individual cases, however, gliomatosis cerebri also affects people in childhood. In addition, an occurrence of gliomatosis cerebri in dogs is possible.

Gliomatosis cerebri is characterized by the fact that both hemispheres of the cerebrum are affected by the disease. In addition, typical anomalies of gliomatosis cerebri also appear in the area of ​​the cerebellum and brainstem as well as the spinal cord. According to the guidelines of the WHO, gliomatosis cerebri is a third-degree tumor that affects the central nervous system.

Causes

The exact causes of gliomatosis cerebri are currently unknown. According to the WHO, it is a brain tumor with an unclear pathogenesis. Gliomatosis cerebri develops as a result of proliferation affecting certain cell types in the gray and white matter of the brain.

While the exact circumstances of the origin of gliomatosis cerebri are mostly unknown, doctors have found genetic mutations in some patients. Researchers currently assume that gliomatosis cerebri is caused by continuously progressing dedifferentiation processes. The cells themselves are hardly malignant in nature. However, due to its typical growth behavior, gliomatosis cerebri is one of the malignant tumors of the brain.

Symptoms, Ailments & Signs

Most cases of cerebellar gliomatosis are located in the brainstem, the two hemispheres of the cerebrum, the cerebellum, or the spinal cord. The tumor is characterized by a diffuse infiltration in the nervous tissue. While the individual cells are hardly malignant, the gliomatosis cerebri is a highly malignant tumor due to its entire structure.

The symptoms of gliomatosis cerebri depend on the location of the tumor in the individual case. In most cases, however, the symptoms of gliomatosis cerebri are relatively unspecific, so that the diagnosis of the malignant brain tumor is often delayed. Patients with gliomatosis cerebri often suffer from headaches that continuously increase in severity. Epileptic seizures due to gliomatosis cerebri are also possible.

Most people with gliomatosis cerebri show significant mental and behavioral changes and are affected by memory disorders. Much less frequently, ischemic symptoms with paralysis, dyskinesia and sensory disturbances develop as a result of cerebrospinal gliomatosis.

Diagnosis

In many cases, the diagnosis of gliomatosis cerebri occurs relatively late and in advanced stages of the disease. This is mainly related to the non-specific symptoms that characterize cerebrospinal gliomatosis at the beginning of the disease. Patients often only turn to a doctor when the gliomatosis cerebri becomes noticeable through serious symptoms such as paralysis or severe memory problems.

The discussion with the patient initially concentrates on the exact complaints and the onset of the symptoms of the disease. Usually, the doctor also takes a family history to get any indications of similar diseases in the family. The doctor carries out the clinical examination primarily by means of imaging methods, whereby he focuses on the patient’s brain.

MRI and CT scans are usually used to diagnose cerebrospinal gliomatosis. Before the examination, patients are often given contrast media that make various structures in the brain visible. In deceased people, a biopsy can be used to determine the cause of death and to identify cerebrospinal gliomatosis.

Complications

Since gliomatosis cerebri is a tumor in the brain, it comes with the usual symptoms and complications of cancer. As with any other tumour, this case can also lead to considerable symptoms and, in the worst case, even death. Gliomatosis cerebri usually causes severe headaches, which can spread to the back or neck.

In addition, seizures occur in different parts of the body, which can also lead to epilepsy. The everyday life of those affected is significantly restricted by the disease. Behavioral changes also occur, and memory can also be impaired. In many cases, the patients can no longer remember events correctly, so that they are dependent on the help of other people in everyday life.

Sensitivity can also be disturbed by the tumor. In most cases, the relatives also suffer from psychological problems. Since surgical treatment is not possible, the tumor can be removed with the help of radiotherapy. In most cases, however, it is not possible to remove the tumor completely. For this reason, life expectancy drops to another year after diagnosis.

When should you go to the doctor?

If the sufferer suffers from recurring headaches, there is cause for concern. If the pain increases in intensity or spreads further, a doctor must be consulted. You should refrain from taking any pain medication until you have consulted a doctor. Further complications can occur and, in addition, the gliomatosis cerebri spreads unhindered. This should be prevented if possible.

If malfunctions occur, this is considered unusual. A doctor’s visit is necessary to clarify the cause. If motor problems, paralysis or limitations of the musculoskeletal system occur, a doctor is required. Epileptic seizures should always be examined comprehensively by a doctor. If your vision, hearing or balance is impaired, medical attention is required. Sensitivity issues should also be investigated and treated.

A feeling of illness, general malaise, reduced performance or drowsiness should be examined by a doctor. Exhaustion, an increased need for sleep or a feeling of pressure inside the head must be clarified by a doctor. A doctor’s visit is also required in the case of psychological problems. If you experience anxiety, sleep disorders or social withdrawal, you should consult a doctor. Behavioral abnormalities, inner restlessness or personality disorders must be presented to a doctor.

Treatment & Therapy

The options for treating cerebrospinal gliomatosis are relatively limited. Surgical removal of the brain tumor is usually not possible, since the gliomatosis cerebri is inaccessible to such an operation. Radiation therapy is an option, but due to the severe localization of the brain tumor, radiation of the entire brain and spinal cord is usually required.

Experiments with chemotherapeutic approaches indicate that the gliomatosis cerebri regresses temporarily. The active ingredient temozolomide has proven to be particularly successful. However, the general prognosis of gliomatosis cerebri is relatively unfavorable. Complete healing of cerebral gliomatosis is usually not possible. Patients live an average of 14.5 months after diagnosis.

Outlook & Forecast

If left untreated or in an advanced stage of the disease, gliomatosis cerebri has a very unfavorable prognosis. Cancer leads to severe impairments in lifestyle and well-being. In most cases, the victim dies prematurely. The overall mortality rate for the disease is high and is around 14 months after diagnosis. In addition to the limited treatment options, the usually very late diagnosis is responsible for this.

If the tumor is detected in time, the prognosis is linked to the location of the tissue changes. In rather rare cases, the tumor is in a favorable position and can be surgically removed. Cancer therapy is then initiated so that a recurrence can be ruled out as far as possible.

An unfavorable position of the tumor is characteristic of gliomatosis cerebri. Here it must be weighed up whether an operation can be carried out or whether the risks for life-long impairments and functional disorders are too high.

There is often a risk that the personality of the person affected could be changed by surgical removal of the diseased tissue. Therefore, most patients do not undergo surgery. Normally, the brain tumor is irradiated. The treatment is aimed at causing the tumor to regress.

Prevention

Preventive measures regarding gliomatosis cerebri are not known. The causes of the development of the disease are still largely unexplored in the case of gliomatosis cerebri.

Aftercare

In most cases, aftercare is not possible for gliomatosis cerebri. The person concerned is primarily dependent on the early detection and treatment of the disease, since this is the only way to prevent further complications. Self-healing cannot occur in this case, so that without medical treatment the life expectancy of the person affected is significantly reduced.

Even after successful treatment of gliomatosis cerebri, regular follow-up examinations should be carried out in order to prevent the recurrence of tumors or to detect them at an early stage. Treatment is usually radiation therapy, although medication can also be used to treat cerebrospinal gliomatosis.

The affected person is dependent on the correct and regular intake of medication in order to treat the disease properly. The support and care of family and friends is also very important in order to support those affected and to help them in everyday life. Psychological support is also very important. In some cases, surgical interventions are also necessary. The person concerned should always rest after such an operation and protect their body.

You can do that yourself

Gliomatosis cerebri is the development of a brain tumor according to WHO grade 3 with a course that cannot be clearly determined in advance. The effects on the patient and the limitations associated with the disease can also be very different.

It therefore depends very much on the form and severity of the gliomatosis cerebri, which forms of self-help the patient can use and how he goes about his everyday life. In most cases, the help of caring relatives or a nursing service is necessary.

Patients with gliomatosis cerebri should be instructed and supported in order to be able to manage their everyday life in a self-determined and independent manner for as long as possible. This conveys confidence in one’s own abilities and increases the quality of life, which often has a positive effect on the course of the disease and therapy. In order to maintain the body’s performance and motor skills, rehabilitation-supported exercises are very helpful, as they enable the patient to remain mobile for longer.

If areas in the brain are affected that affect thinking and memory, the patient can be supported by a person who takes care of administrative tasks and, for example, administrative procedures or dealing with the health insurance company. Simple mind games and mind exercises can improve the clinical picture. It is important to reassure the patient that they are being well cared for.