Silver Grain Disease

The Silver Grain Disease is a neurodegenerative dementia similar to Alzheimer's disease. In this disease in particular, tau proteins are deposited in the limbic system. The previously incurable disease is currently being treated primarily supportively.

What characterizes the silver grain disease?

The definitive diagnosis can usually only be made after death. In silver plating processes, during a post-mortem autopsy, the diagnosis can be made of silver grains of a certain shape.
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Under the Silver Grain Disease medicine understands 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. As a rule, the disease does not occur until the age of 80.

The clinical picture is similar to that of Alzheimer's disease and is associated with deposits in the brain. At the moment it is still being discussed whether the 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 systemic deposits of the silver grain disease are made visible with a histological silver plating process, which is also known as argyrophilia.

This procedure is partly responsible for the name of the disease. The disease is classified as one of the so-called Taupathias, as the characteristic deposits correspond to biochemically abnormally phosphorylated tau proteins.

causes

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, at least theories about the cause of the disease are currently in the room. For example, the ε4 allele of apolipoprotein E probably plays an increased role in the silver grain disease.

Apolipoprotein E is a component of many lipoproteins and, from a genetic point of view, is 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 [atherosclerosis atherosclerosis]].

Symptoms, ailments & signs

Personality changes, behavioral problems and cognitive deficits are considered early symptoms of silver grain disease. Above all, the behavioral problems and changes in personality include outbursts of anger and barely comprehensible social behavior. Tremors and restlessness can also occur. The cognitive abnormalities usually worsen in the further course.

Speech disorders are often one of the symptoms of the disease. Already at the beginning of the disease, spindle-shaped deposits appear in the frontal and temporal lobes of the brain. 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 conduction of excitation is greatly delayed or almost does not take place at all. Memory and psyche progressively lose their function. Usually Alzheimer's typical lesions are found in the brain in addition to the “silver grains”.

Diagnosis & course of disease

The silver grain disease is an extremely young and little researched disease. That makes the diagnosis more difficult. The definitive diagnosis can usually only be made after death. In silver plating processes, the diagnosis-confirming evidence of silver grains of a certain shape can be obtained during a post-mortem autopsy.

Usually, patients with silver grain disease will be diagnosed with a neurodegenerative disease in life. The differential diagnosis can hardly be made. Silver grains can also occur in combination with Alzheimer's. In the context of the silver grain disease, brain lesions typical of Alzheimer's can also be observed. Since it is still unclear whether the silver grain disease actually corresponds to an independent disease or possibly a special form of Alzheimer's, patients are most often diagnosed with Alzheimer's during their lifetime.

The course of silver grain disease depends heavily on the location of the protein deposits.In Alzheimer's disease, it is assumed that an early diagnosis has a positive influence on the course. If the silver grain disease corresponds to a special form of Alzheimer's, this could also apply to this disease.

Complications

As a rule, the cause of the silver grain disease cannot be treated. For this reason, only the symptoms and symptoms of this disease can be limited, so that in most cases the patients are dependent on lifelong therapy. Children in particular suffer from severe behavioral problems with the silver grain disease.

This can lead to impaired coordination and concentration, which can also have a negative impact 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 the silver grain disease.

Most of those affected also suffer from tremor and inner restlessness. Speech disorders or swallowing difficulties can also occur as a result of the disease and have a negative effect on everyday life and the quality of life of the person concerned. Many patients also suffer from memory lapses and mental health problems.

As a rule, the symptoms worsen as the disease progresses. The treatment takes place with the help of drugs and various therapies. There are no particular complications. However, there is no completely positive course of the disease.

When should you go to the doctor?

The silver grain disease should always be treated by a doctor. Only treatment can prevent further complications and complaints. In most cases, however, the affected person is dependent on lifelong therapy, as the silver grain disease cannot be completely cured.

A doctor should be consulted if the person affected shows signs of dementia. In addition, aggressiveness and dissatisfaction can become visible. They suffer from restlessness and tremor, and they find it difficult to speak. Often those affected with the silver grain disease do not recognize even known people and voices or cannot assign them correctly. If these symptoms occur, a doctor must be consulted in any case. Likewise, problems with memory in old age can point to the silver grain disease.

The diagnosis of the disease can be made 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 against the silver grain disease. Symptomatic therapies also turn out to be difficult. This is especially true with regard to the progressive loss of cognitive abilities. The therapy for the silver grain disease is above all supportive. The therapy is intended to alleviate the symptoms in order to make the life of the person affected and their relatives more bearable. The aim is to improve the quality of life, but the disease is not curable.

The course of the disease cannot be stopped either. The loss of cognitive abilities is often counteracted with cognitive training. In diseases such as Alzheimer's, this can at least delay the cognitive impairment. In the early stages of the disease, compensation strategies may serve 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 location of the protein deposits. Often, antidementia drugs, antidepressants and neuroleptics are also given. The patient's outbursts of violence and tantrums may need to be alleviated by administering calming medication. Neuroprotective substances are currently being researched and in the future may serve to preserve nervous systemic tissue.

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➔ Medicines against memory disorders and forgetfulness

prevention

Neurodegenerative diseases such as silver grain disease can probably not be prevented. Some studies suggest healthy lifestyles, activity, and mental training for the prevention of Alzheimer's and Alzheimer's-related diseases. The effectiveness of these prevention strategies remains controversial.

Aftercare

Since silver grain disease is an incurable disease, those affected usually have only very few and only very limited aftercare measures available. Therefore, a doctor should be contacted at the first symptoms or signs of this disease so that there are no other symptoms or complications in the further course. There can be no independent healing.

The earlier a doctor is consulted in the case of silver grain disease, the better the further course of the disease. In many cases the symptoms of the disease can be limited with the help of various drugs. The person concerned should always pay attention to the correct dosage and regular intake of the medication. 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, the silver grain disease does not reduce the life expectancy of the patient, although the further course depends very much on the severity of the disease.

You can do that yourself

Since the silver grain disease is characterized by a progressive loss of memory, patients suffer increasing cuts in their quality of life. First of all, those affected should accept that it is a previously incurable disease. Instead, active measures can be taken to alleviate symptoms and postpone major restrictions in everyday life.

For example, patients can actively train their memory, for example with suitable exercises or games. Even light sporting 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 engaging in stimulating, interest-generating activities rather than following the same 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 an active life as long as their mental and physical condition allows. Self-help groups can also be useful in order to exchange ideas with other people affected by the disease and thus to experience support and understanding. As the disease progresses, it may be necessary for those affected to take advantage of assisted living facilities and thus be able to cope with everyday life more easily.