Gliomatosis cerebri is a primary type brain tumor. Gliomatosis cerebri is characterized by diffuse infiltration in the tissues of the brain, which is also typical of other gliomas. Due to the extent of this infiltration, the solid structures of the tumor play a secondary role. Gliomatosis cerebri represents a very rare disease, with a high incidence of unreported cases suspected due to the low level of awareness of the disease.
What is gliomatosis cerebri?
In principle, gliomatosis cerebri occurs with a low prevalence. In part, clinical parallels of gliomatosis cerebri exist with certain types of encephalitis. It is a primary brain tumor characterized by diffuse infiltration. The physician Nevin described gliomatosis cerebri for the first time in 1938. So far, only about 200 cases of gliomatosis cerebri are known in the whole world. Gliomatosis cerebri usually occurs in adult patients. Here, gliomatosis cerebri occurs more frequently in people of advanced age. In individual cases, however, gliomatosis cerebri also affects people in childhood. In addition, the 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 abnormalities of gliomatosis cerebri are also seen in the cerebellum and brainstem, as well as the medulla of the back. According to WHO guidelines, gliomatosis cerebri belongs to the third-degree tumors affecting the central nervous system.
Causes
The exact causes of gliomatosis cerebri are currently unknown. Thus, according to WHO, it is a tumor of the brain with unclear pathogenesis. Gliomatosis cerebri develops as a result of proliferation affecting specific cell types in the gray and white matter of the brain. While the exact circumstances of gliomatosis cerebri are mostly unknown, physicians detect genetic mutations in some patients. Researchers currently assume that gliomatosis cerebri develops through continuously progressive de-differentiation processes. The cells themselves are hardly malignant in nature. However, gliomatosis cerebri belongs to the malignant tumors of the brain due to its typical growth behavior.
Symptoms, complaints, and signs
In most cases, gliomatosis cerebri is localized in the brainstem, both hemispheres of the cerebrum, the cerebellum, or the spinal cord. The tumor is characterized by diffuse infiltration in the nervous tissue. While the individual cells are hardly malignant, gliomatosis cerebri is a highly malignant tumor due to its overall structure. The symptoms of gliomatosis cerebri depend in individual cases mainly on the location of the tumor. In most cases, however, the symptoms of gliomatosis cerebri are relatively non-specific, so that the diagnosis of the malignant brain tumor is often delayed. For example, gliomatosis cerebri often causes patients to suffer from headaches that continuously increase in severity. Epileptic seizures due to gliomatosis cerebri are also possible. Usually, people with gliomatosis cerebri show significant mental and behavioral changes and are affected by memory disorders. Far less commonly, ischemic symptoms with paralysis, dyskinesias, and disturbances in sensibility develop as a result of gliomatosis cerebri.
Diagnosis
In many cases, the diagnosis of gliomatosis cerebri is made relatively late and at advanced stages of the disease. This is mainly due to the nonspecific symptoms that characterize gliomatosis cerebri at the onset of the disease. Patients often do not contact a physician until gliomatosis cerebri becomes noticeable through severe symptoms such as paralysis or severe memory problems. Thus, the patient interview initially focuses on the exact symptoms as well as the onset of the disease symptoms. Usually, the physician also takes a family history in order to obtain any indications of similar diseases in the relatives. The physician conducts the clinical examination primarily using imaging techniques, focusing on the patient’s brain. In most cases, an MRI and a CT scan are used to diagnose gliomatosis cerebri.Patients are often given contrast media before the examination, which makes various structures in the brain visible. In deceased individuals, a biopsy may be considered to determine the cause of death and identify gliomatosis cerebri.
Complications
Because gliomatosis cerebri is a tumor in the brain, the usual symptoms and complications of cancer occur. As with any other tumor, it can cause significant discomfort and, in the worst cases, death. As a rule, gliomatosis cerebri causes severe headaches, which can spread to the back or neck. In addition, seizures occur in various areas of the body, and epilepsy may further develop. The daily life of the affected person is significantly limited by the disease. Changes in behavior also occur, and memory may also be disturbed. In many cases, 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 discomfort. Since surgical treatment is not possible, the tumor can be removed with the help of radiation therapy. In most cases, however, it is not possible to remove the tumor completely. For this reason, life expectancy decreases to another year after diagnosis.
When should you see a doctor?
If the affected person suffers from recurrent headaches, there is cause for concern. If the pain increases in intensity or spreads further, a doctor must be consulted. Taking a pain medication should be completely avoided until a doctor is consulted. Further complications may occur and, in addition, gliomatosis cerebri continues to spread unhindered. This should be prevented if possible. If functional disturbances occur, this is considered unusual. A visit to the doctor is necessary to clarify the cause. If motor problems, paralysis or limitations of the musculoskeletal system occur, a doctor is needed. Epileptic seizures should always be examined comprehensively by a doctor. If there are problems with vision, hearing, or balance, medical attention is needed. Sensory problems should also be investigated and treated. A feeling of illness, general malaise, decreased performance or lightheadedness should be examined by a physician. Fatigue, an increased need for sleep or a feeling of pressure inside the head must be clarified by a doctor. A visit to the doctor is also necessary in the case of psychological problems. If anxiety, sleep disturbances or social withdrawal occur, a doctor should be consulted. Abnormalities of behavior, inner restlessness or personality disorders must be presented to a doctor.
Treatment and therapy
The options for therapy of gliomatosis cerebri are relatively limited. Surgical removal of the brain tumor is usually not possible because gliomatosis cerebri is inaccessible to such surgery. Although radiation therapy is an option, it usually requires irradiation of the entire brain as well as the spinal cord due to the severe local confinement of the brain tumor. Trials with chemotherapeutic approaches indicate that gliomatosis cerebri regresses temporarily. The agent temozolomide has been shown to be particularly successful. However, the general prognosis of gliomatosis cerebri is relatively unfavorable. A complete cure of gliomatosis cerebri is usually not possible. Patients live an average of 14.5 months after diagnosis.
Outlook and prognosis
Left untreated or in an advanced stage of disease, gliomatosis cerebri has a very unfavorable prognosis. The cancer causes severe impairment of lifestyle as well as well-being. In most cases, premature death of the affected person occurs. The mortality rate for the disease as a whole can be classified as high and is approximately 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 removed in a surgical procedure. Subsequently, cancer therapy is initiated so that recurrence can be excluded as far as possible. Characteristic for gliomatosis cerebri is an unfavorable position of the tumor. In this case, it must be weighed whether surgical intervention can be performed or whether the risks of lifelong impairment and dysfunction are too high. Often there is a risk that the personality of the affected person could be changed in a surgical removal of the diseased tissue. Therefore, surgery is not performed in most patients. Normally, the brain tumor is treated with radiation. Treatment is aimed at causing the tumor to regress as a result.
Prevention
Preventive measures regarding gliomatosis cerebri are not known. The causes of disease development are largely unexplored in gliomatosis cerebri.
Follow-up
In most cases of gliomatosis cerebri, there are no options for follow-up care. In this case, the affected person is primarily dependent on early detection and treatment of the disease, as this is the only way to prevent further complications. Self-healing cannot occur, so that without medical treatment the life expectancy of the affected person is significantly reduced. Even after successful treatment of gliomatosis cerebri, regular follow-up examinations should be performed to prevent the recurrence of tumors or to detect them at an early stage. Treatment in this case is usually radiation therapy, although medications may also be used to treat gliomatosis cerebri. The affected person is thereby dependent on the correct and regular intake of the medication in order to treat the disease properly. The support and care of the family and friends is also very important to support the affected person and to help him in his daily life. Psychological support is also very important in this process. In some cases, surgical interventions are also necessary. In this case, the affected person should always rest after such an operation and take care of his body in the process.
What you can do yourself
Gliomatosis cerebri is the development of a brain tumor according to WHO grade 3 with a course that cannot be clearly predicted. The impact on the patient and the limitations associated with the disease can also be quite different. Therefore, it depends very much on the form and severity of gliomatosis cerebri, which forms of self-help the patient can apply and how he manages his everyday life. In most cases, the help of caring relatives or a nursing service is necessary. Patients with gliomatosis cerebri should be guided and supported to be able to manage their daily lives independently and in a self-determined manner for as long as possible. This gives patients confidence in their own abilities and increases their 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 in enabling patients to remain mobile for longer. If areas of the brain that affect thinking and memory are affected, the patient can be supported by a person who takes care of administrative tasks and, for example, dealing with the authorities or health insurance. Simple mental games and thought exercises can improve the clinical picture. It is important to reassure the patient that he or she is being well cared for.
