Neurodegenerative diseases are diseases whose main characteristic is the progressive death of nerve cells. Among the best known are Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (ALS). In addition, rarer diseases such as Creutzfeldt-Jakob disease and Huntington’s disease fall into this group.
What are neurodegenerative diseases?
Neurodegenerative diseases usually occur at an older age – unlike the physiological aging process, the degradation of nerve cells progresses faster and to a greater extent. As a result, massive impairments of mental and physical abilities occur and increase. The pathological degradation processes of the nerve cells are usually limited to certain parts of the brain, but can also affect the entire central nervous system. Due to increasing life expectancy, neurodegenerative diseases are becoming more and more important; despite intensive research, a cure is not yet possible.
Causes
The causes of pathological neuronal degeneration are not yet clearly understood. Genetic factors play a role, as do disorders in protein metabolism, as a result of which protein deposits lead to the death of nerve cells in the brain. Unlike other cells in the human body, brain cells are normally very long-lived, but have only a limited ability to regenerate. Premature cell death is therefore difficult for the organism to compensate. Infections and inflammatory processes, environmental toxins and traumatic brain damage are also discussed as triggers. Whether risk factors such as obesity, hypertension, and diabetes mellitus also promote the development of a neurodegenerative disease has not yet been completely clarified.
Symptoms, complaints, and signs
The symptoms of each disease depend on the type of nerve affected. In Parkinson’s disease, for example, the nerve cells that produce the hormone dopamine, which is necessary for the coordination of movements, die: This results in the typical tremor, stiff gait and slowed movements. In Huntington’s disease, which is hereditary, involuntary movements of the head and extremities are noticeable at first, followed by speech and swallowing disorders. Alzheimer’s disease is characterized by increasing forgetfulness that goes far beyond normal levels – even temporal and spatial orientation becomes increasingly difficult. In amyotrophic lateral sclerosis, which can also occur at a young age, only the nerve cells responsible for muscle movements (motoneurons) are affected, which becomes noticeable through spastic paralysis and increasing muscle weakness. As with Parkinson’s disease, intellectual abilities are not usually affected in this disorder, but depression, sleep disturbances, and anxiety often occur as a result of the physical symptoms.
Diagnosis and course of the disease
The diagnosis is preceded by a detailed interview and examination of the patient and his relatives: conspicuous movement disorders or significant impairments of mental abilities already provide initial information about the clinical picture. If dementia is suspected, psychological tests provide further clues. Technical examination procedures include computer tomography, magnetic resonance imaging and magnetic resonance imaging, which can be used to visualize pathological changes in the brain. In order to rule out other diseases, comprehensive blood tests are carried out – an examination of the cerebrospinal fluid (CSF) can confirm the suspicion of Alzheimer’s disease or Parkinson’s disease. Genetic tests are used to detect hereditary diseases such as Huntington’s disease. Electrical muscle activity and nerve conduction velocity are measured to confirm amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy. In some neurodegenerative diseases, such as Creutzfeldt-Jakob disease, changes in brain waves are noticeable on the electroencephalogram (EEG). The loss of mental and/or physical abilities progresses steadily over years in all neurodegenerative diseases. In the advanced stage, independent living is usually no longer possible.
Complications
Neurodegenerative diseases always progress and often lead to severe complications in the late stages. Therefore, the most important task is to slow down the process of degeneration. The complications that can occur also depend on the particular disease. For example, Alzheimer’s disease is characterized by a progressive decline in cognitive abilities. Like many other neurodegenerative diseases, Alzheimer’s is not a fatal disease. In the late stages of the disease, however, the affected patient must be constantly cared for by nursing staff, as the increasing inability to care for oneself can lead to death by starvation or thirst. It is also no longer possible to ensure that vital medications are taken. Furthermore, the neurodegenerative diseases such as Alzheimer’s or Parkinson’s can also lead to other complications in later stages, such as life-threatening infections of the respiratory tract (pneumonia), dysphagia up to the complete cessation of swallowing, or life-threatening falls. One of the most severe diseases of this type is Huntington’s disease. Huntington’s disease itself always leads to death, which usually occurs 15 years after diagnosis. During the course of this disease, energy consumption constantly increases and problems with eating occur. As with many neurodegenerative diseases, Huntington’s disease also has an increased risk of suicide. There is currently no causal therapy for any neurodegenerative disease. Only the symptoms can be alleviated.
When should you see a doctor?
If trembling hands or restless limbs can be noticed by fellow patients in an affected person, the observation should be openly discussed and followed up. If the shaking persists or increases in intensity, a visit to the doctor should be made to clarify the symptoms and make a diagnosis. If there are changes in habitual movements, slowed locomotion or a stiff gait, investigation of symptoms is indicated. Disturbances in coordination, problems in performing usual sports activities and an increased risk of accidents are signs of an irregularity that must be discussed with a physician. If unusual head movements are evident, there is cause for concern and a physician should be consulted. If memory problems develop, forgetfulness or disturbances in the recall of learned skills occur, a doctor is needed. If the affected person complains of discomfort in swallowing, loss of appetite, or shows a change in weight, a physician should be consulted. Changes in mood, depressive behavior, apathy, and withdrawal from social life should be discussed with a physician. Sleep disturbances, diffuse anxiety as well as a decrease in physical performance indicate a disease for which action is required. Paralysis or general musculoskeletal complaints should be investigated.
Treatment and therapy
Neurodegenerative diseases are not yet curable, despite intensive research. Therefore, the goal of therapy is to slow progression. The course of PD can be positively influenced by drugs that compensate for the dopamine deficiency underlying the disease: In many cases, the symptoms remain stable for years, although unpleasant side effects are not uncommon. Good results can also be achieved by inserting a brain pacemaker for deep brain stimulation – since the operation is not free of risks, it is only performed after exhausting the possibilities of medication. Targeted coordination and movement exercises counteract muscle weakness and muscle tension that occur in neurodegenerative diseases. Voice and speech therapy may also be indicated. If, as in Alzheimer’s disease, the focus is on a decline in mental abilities, psychotherapy and memory training are used in addition to drug treatment. A feeding tube ensures food intake in the advanced stage of amyotrophic lateral sclerosis (ALS), in which mechanical support of respiratory activity may also be necessary. In addition to conventional medical therapy, the use of alternative treatment methods – such as osteopathy or acupuncture – can also help alleviate symptoms in some cases.
Outlook and prognosis
Patients diagnosed with neurodegenerative disease receive an unfavorable prognosis.Although the intensity of the disease and the progress of the underlying disease must be assessed individually, the decay of the nerve cells is common to all. The cognitive degradation processes can be slowed down if the diagnosis is made early and therapy is started in the early stages of the disorder. However, they are not completely prevented. At the same time, there is no possibility that the already damaged neurons will regenerate. The focus of the basic disease is basically to improve the current quality of life and to delay further degradation processes. The general life expectancy is reduced in affected individuals. If medical care is not sought, a more rapid deterioration in general health is seen. It is often no longer possible to cope with everyday life without help. In addition to disturbances of mental capacities, the further course of the disease also leads to a loss of mobility. States of confusion, disorientation and an increased risk of accidents are given. The underlying diseases place a heavy emotional burden on the patient as well as his or her relatives. Therefore, when making a prognosis for the further development of the disease, the probability of developing a mental illness must be taken into account. These lead to further deterioration of the overall situation, as they also take an unfavorable course on the development of physical possibilities.
Prevention
The causes of neurodegenerative diseases are not yet fully understood. At least some diseases in this group are certainly based on genetic factors: targeted prevention is therefore only possible to a limited extent. At least on the occurrence of Alzheimer’s disease, a healthy lifestyle with plenty of exercise, mental challenges, but also the necessary recovery phases seems to have a positive effect. Certain environmental toxins (pesticides, heavy metals) are suspected of promoting PD – contact with such products should therefore be avoided as far as possible. Early detection plays an important role: if treatment begins in the early stages of the disease, its progression can often be significantly delayed.
Follow-up care
Neurodegenerative diseases are fundamentally incurable. Moreover, they progress inexorably and deprive patients of their independence in the long term. Therefore, affected individuals are always dependent on long-term aftercare until the end of their lives. Thus, the quality of aftercare also determines the quality of life of those affected. The type of aftercare depends in turn on the corresponding disease and the phase of the disease. In mild cases, exercise and mental training can help to compensate for certain deficits in addition to drug treatment. The more intensively the patient is cared for in this regard, the longer he can maintain his independent lifestyle. However, when dementia and immobility are more advanced, those affected often need professional support in all situations and at all times of the day. In many cases, this assistance can no longer be provided in the home environment by family members alone. A great deal of help in caring for their sick family member is then provided by well-trained caregivers who look after the patient day and night. In other cases, only placement in a nursing facility can guarantee the affected person a dignified life. Neurodegenerative diseases without dementia, such as ALS among others, also require permanent care due to the increasing restriction of mobility and organ functions.
What you can do yourself
In dealing with the patient in everyday life, you should refrain from correcting, reprimanding or pointing out deficits. Instead, recognition and praise for successful things lead to positive success in the relationship with the ill person. With regard to his resources and abilities, he should be given smaller tasks or simple activities. The aim is not to perform a task perfectly. Rather, the aspect of being useful and still being able to accomplish something is in the foreground. Relatives must learn to enter into the world of the patient and to treat him with appreciation. Naomi Feil, founder of integrative valitation as a form of communication with dementia patients, called it: “Walking in the shoes of the other.”With this, she explained that the sick person must be picked up where he is at the moment in terms of his psychological, emotional and spiritual state. Only at this level is communication with the patient possible with a great deal of empathy and compassion. Withdrawal due to the disease should be avoided. Rather, the social environment must be informed and educated about the case of illness, the clinical picture and the accompanying changes in living together. Acceptance and recognition by the social environment are equally important for the ill person as well as for the family member in everyday life.