Silver grain disease is a neurodegenerative dementia similar to Alzheimer’s disease. In this disease, tau proteins are deposited particularly in the limbic system. The hitherto incurable disease is currently treated primarily supportively.
What is silver grain disease?
In medicine, silver grain disease is a degenerative disease of the central nervous system. It is a type of dementia in old age that focuses on the limbic system. The disease was first described in the 21st century by the German neuroanatomists H. and E. Braak. The disease usually occurs after the age of 80. For hemarthrosis definitions, please visit definitionexplorer.com.
The clinical picture is similar to that of Alzheimer ‘s disease and is accompanied by deposits in the brain. It is currently still being discussed whether silver grain disease should actually be regarded as an independent disease or whether it corresponds to a special form of Alzheimer’s. The central nervous system deposits of silver grain disease are visualized using a histological silvering procedure also known as argyrophilia.
This procedure is partly responsible for the name of the disease. The disease is counted among the so-called taupathies, since the characteristic deposits correspond biochemically to abnormally phosphorylated tau proteins.
So far, the cause of most taupathies is still unknown. Medicine today assumes a hereditary basis in which certain gene mutations are passed on. Although the triggers of the disease have not yet been adequately researched, there are at least theories about the cause of the disease. The ε4 allele of apolipoprotein E presumably plays an increased role in silver grain disease.
Apolipoprotein E is a component of many lipoproteins and is genetically located on chromosome 19. The three different alleles of apolipoprotein E differ in a single amino acid. The mutation of these alleles has already been identified as the genetic cause of hereditary diseases such as Alzheimer’s or [arteriosclerosis|atherosclerosis]].
Symptoms, Ailments & Signs
Personality changes, behavioral problems and cognitive deficits are considered early symptoms of silver grain disease. The behavioral problems and changes in personality include, above all, outbursts of anger and hardly comprehensible social behavior. Tremor and restlessness can also occur. The cognitive abnormalities usually get worse as the disease progresses.
Speech disorders are often among the course symptoms of the disease. Spindle-shaped deposits in the frontal and temporal lobes of the brain are already evident at the beginning of the disease. They usually appear as four to nine µm large inclusions in the processes of the nerve cells or in the oligodendroglia. Because of these inclusions, the transmission of excitation is greatly delayed or does not take place at all. Memory and psyche progressively lose their function. In addition to the “silver grains”, there are usually lesions typical of Alzheimer’s in the brain.
Diagnosis & course of disease
The silver grain disease is an extremely young and little researched clinical picture. That makes the diagnosis difficult. The definitive diagnosis can usually only be made after death. In the silver-plating process, a post-mortem autopsy can be used to provide diagnostic evidence of silver grains of a specific shape.
Usually, patients with silver grain disease are diagnosed with a neurodegenerative disease in life. However, the differential diagnosis is difficult to make. Silver grains can also appear in combination with Alzheimer’s. In the context of silver grain disease, brain lesions typical of Alzheimer’s can also often be observed. Since it is still unclear whether silver grain disease is an independent disease or a special form of Alzheimer’s, patients are most often diagnosed with Alzheimer’s during their lifetime.
The course of the disease of silver grain disease depends strongly on the location of the protein deposits. In the case of Alzheimer’s, it is assumed that an early diagnosis has a positive influence on the course. If silver grain disease corresponds to a special form of Alzheimer’s, this could also apply to this disease.
As a rule, silver grain disease cannot be treated causally. For this reason, only the symptoms and complaints of this disease can be limited, so that in most cases patients are dependent on lifelong therapy. Children in particular suffer from severe behavioral problems with silver grain disease.
This can lead to coordination and concentration disorders, which can also have a negative effect on performance at school. Many patients suffer from unfounded outbursts of anger or from severe irritability. Other psychological complaints or depression can also occur with silver grain disease.
Furthermore, most of those affected suffer from tremor and inner restlessness. Speech disorders or swallowing difficulties can also occur as a result of the disease and have a negative impact on everyday life and the quality of life of those affected. Many patients also suffer from memory lapses and psychological limitations.
As a rule, the symptoms worsen as the disease progresses. The treatment is carried out with the help of medication and various therapies. There are no particular complications. However, the course of the disease is not completely positive.
When should you go to the doctor?
Silver grain disease should always be treated by a doctor. Only treatment can prevent further complications and symptoms. In most cases, however, the person affected is dependent on lifelong therapy, since silver grain disease cannot be completely cured.
A doctor should be consulted if the person concerned shows signs of dementia. In addition, aggressiveness and dissatisfaction can become visible. They suffer from inner restlessness and tremors, and they find it difficult to speak. Those affected with silver grain disease often do not recognize well-known people and voices or cannot correctly identify them. If these symptoms occur, a doctor must be consulted in any case. Memory problems in old age can also point to silver grain disease.
The disease can be diagnosed by a general practitioner. Further treatment depends on the exact symptoms and their severity, although complete healing is not possible.
Treatment & Therapy
So far, there is no causal therapy for silver grain disease. Even symptomatic therapies turn out to be difficult. This is especially true with regard to the progressive loss of cognitive abilities. The treatment path for silver grain disease is primarily supportive. The symptoms should be alleviated by the therapy in order to make the life of those affected and their relatives more bearable. The aim is to improve the quality of life, but the disease cannot be cured.
The course of the disease has also not been stopped so far. The loss of cognitive abilities is often counteracted with cognitive training. In the case of diseases such as Alzheimer’s, the cognitive impairment can at least be delayed. In the early stages of the disease, compensation strategies can be used to compensate for certain deficits. Depending on the area of degeneration, occupational therapy, speech therapy and physiotherapy can take place.
Which compensation strategies are learned depends on the localization of the protein deposits. Antidementia, antidepressants and neuroleptics are often given in addition. The patient’s outbursts of violence and tantrums may need to be reduced by the administration of sedative medication. Neuroprotective substances are currently being researched and may in future serve to preserve nervous system tissue.
Neurodegenerative diseases such as silver grain disease can probably not be prevented. Some studies suggest healthy lifestyle, activity, and mental training to prevent Alzheimer’s and Alzheimer’s-related diseases. However, the effectiveness of these prevention strategies remains controversial.
Since silver grain disease is an incurable disease, those affected usually only have very few and only very limited aftercare measures available. Therefore, a doctor should be contacted as soon as the first symptoms or signs of this disease appear, so that other symptoms or complications do not arise in the further course. It cannot heal on its own.
The sooner a doctor is consulted for silver grain disease, the better the further course of the disease. In many cases, the symptoms of the disease can be reduced with the help of various medications. The person concerned should always ensure that the dosage is correct and that the medication is taken regularly. If you have any questions or are unclear, it is advisable to contact a doctor.
In some cases, physiotherapy measures are also very useful to limit the symptoms. Many of the exercises can also be repeated at home to speed recovery. As a rule, silver grain disease does not reduce the patient’s life expectancy, although the further course depends very much on the severity of the disease.
You can do that yourself
Since silver grain disease is characterized by a progressive loss of memory, patients suffer increasing cuts in quality of life. First of all, those affected should accept that it is a disease that has not yet been cured. Instead, active measures can be taken to relieve the symptoms and delay major restrictions in everyday life.
For example, patients can actively train their memory, for example with suitable exercises or games. Even light physical activity has a beneficial effect on cognitive performance and overall well-being. In general, people with silver grain disease are advised to spend their free time in stimulating, interest-inducing activities rather than following a consistent daily routine. Because new impressions stimulate the ability to learn and can also have a positive effect on memory performance.
Overall, patients should strive to lead as active a life as possible for as long as their mental and physical condition allows. Self-help groups can also be useful for exchanging information about the disease with other sufferers and thereby gaining support and understanding. As the disease progresses, it may be necessary for those affected to make use of offers for assisted living and thus be able to cope with their everyday life more easily.