Ataxia: Causes, Symptoms & Treatment

Ataxias are disorders of movement coordination for which different diseases are the trigger. There is a loss of function of certain parts of the nervous system. The cerebellum is usually affected, but damage to the spinal cord or peripheral nerves can also lead to ataxias.

What is ataxia?

Ataxia is derived from the Greek word ataxia, which means disorder or irregularity. The term ataxia is used to describe various disorders of movement coordination that can manifest themselves in different ways. Ataxias are classified according to etiology, according to the affected section of the nervous system or according to the affected form of movement. In the latter case, a distinction is made between trunk ataxia, stance ataxia, gait ataxia and pointing ataxia. Affected individuals with trunk ataxia are unable to sit or stand straight without support. In stance ataxia, patients are only able to stand and walk with assistance. Gait ataxia manifests as a wide-legged and unsteady gait. In pointing ataxia, patients are unable to coordinate their movements properly, which leads to fine motor difficulties such as pointing beside the hand, overshooting and extending movements, or fluid and shaky movements. If only one half of the body is affected by ataxia, it is called hemiataxia. In addition to impaired movement coordination, other signs may be present. Speech may be disturbed, eye movements may no longer be coordinated, or problems with swallowing may occur. Accompanying symptoms such as incontinence, pain or muscle spasms are often observed.

Causes

Diseases involving loss of function of certain parts of the nervous system can cause ataxias. The most common cause is damage to the cerebellum, which is responsible for coordinating information from the spinal cord, the vestibular system, and other sensory input. The cerebellum translates this information into motor movements. If this no longer functions, ataxias occur. Brain tumors or metastases in the cerebellum can lead to the symptoms. Likewise, a stroke that results in circulatory problems or bleeding in the cerebellum can trigger ataxia. Inflammatory diseases of the nervous system that damage the cerebellum or spinal cord can also be considered as a cause. An example of this is multiple sclerosis. Infectious diseases, such as measles, can damage nerves and cause ataxia if severe. Acute cerebellar dysfunction caused by excessive alcohol consumption or the overdose of certain medications, such as antiepileptic drugs, benzodiazepines or certain antibiotics, also trigger disturbances in movement coordination. Ataxias can also be genetic, called hereditary ataxias. They are triggered by various rare, inherited disorders that usually affect the cerebellum or spinal cord.

Symptoms, complaints, and signs

Definite symptoms and signs of ataxia include gait disturbances, coordination problems, restricted movement, speech problems, and difficulty swallowing. When a patient has ataxia, he or she is no longer able to move at a normal rate. In gait ataxia, movements appear unsteady with a comparatively wide-legged gait. Because of these shaky and uncoordinated movements, sufferers try to compensate for balance problems. Trunk ataxia is noticeable by the inability to sit up straight and an associated tendency to fall to the right or left side. In the case of pointing ataxia, complaints occur in the sequence of fine motor movements. Pointing aside, extending and excess as well as uncoordinated and shaky movements are the result. In standing ataxia, the affected person is only able to stand and walk with outside help. The ataxia can vary in severity and can affect both or only one half of the body. Due to the multiple symptoms, there is also an increased risk of accidents. Due to the disturbed movement sequences, it often happens that the affected person not only stumbles, but simply falls over. In addition to these complaints of the musculoskeletal system, a loss of control of the eyes is possible, which leads to the fact that distances and targets are not correctly estimated. Pronunciation is unclear and difficult to understand, while food intake is limited due to swallowing difficulties. Likewise, muscle spasms, pain and incontinence may occur.

Diagnosis and course

Ataxia may begin gradually or symptoms may appear suddenly, depending on the cause. Initial signs include difficulty with coordination, uneven gait or frequent stumbling as well as fine motor difficulties. Problems speaking, loss of control of eye movements, and difficulty swallowing also occur. If there is loss of balance, loss of limb control, spongy speech, or difficulty swallowing, affected individuals should consult a neurologist to determine the cause. If movement disorders suddenly occur, a doctor should also be contacted immediately. If the symptoms occur before the age of 25, a molecular genetic examination is performed. This will determine whether an inherited disorder is causing the symptoms. In adulthood, extensive physical and neurological examinations may be required to determine the cause of the disease. Laboratory tests, involving blood or urine, may provide information about other diseases. An X-ray, computer tomography or spinal fluid sampling may also be necessary in some cases. The further course of symptoms and whether they worsen or recede depends on the causative disease.

Complications

In ataxia, the patient is unable to move properly on his or her own. In most cases, this person relies on help from others or on walking aids to walk properly. In ataxia, it may also occur that the affected person can no longer stand alone. The gait pattern appears relatively wide-legged and unsteady. Targets and the distance to targets can also no longer be correctly estimated. The patient often misses and cannot easily reach certain things. Rapid and sudden movements can also usually no longer be performed. The patient’s everyday life is severely restricted by the ataxia and the person is usually dependent on the help of other people. In many cases the ataxia is caused by the abuse of alcohol or other drugs. Its extension can be prevented by stopping these drugs. However, the ataxia usually will not completely go away, even if the drugs are stopped. Physical therapy can be equally helpful and encourages the patient to move. However, treatment with drugs is not necessary.

When should you see a doctor?

If ataxia is suspected, the primary care physician should be consulted quickly. If coordination disorders, gait disturbances or other movement restrictions occur quite suddenly that cannot be attributed to any other cause, medical advice is required. Acute speech disorders or swallowing difficulties may also be due to a disease that needs to be clarified. Stroke patients should talk to their doctor if they show signs of ataxia. The same applies to people who have already suffered a brain hemorrhage or have had measles in the past. Tumors and nerve diseases can also cause ataxia. Patients with a corresponding medical history should therefore definitely have said symptoms investigated. If disturbances in movement coordination occur after taking a new medication, the patient should talk to his or her family doctor before the symptoms increase further. Patients with hereditary ataxias should regularly inform themselves about the possible symptoms and consult a physician at the first signs. Comprehensive therapy can usually avert further complications.

Treatment and therapy

Thus, therapy for ataxia also depends on the underlying disease. In some cases, when this disease is cured, the symptoms of ataxia also disappear. Similarly, ataxia caused by measles or viral infections usually improves on its own. In case of excessive alcohol consumption or overdose of medications, abstaining from these substances helps for the improvement of the symptoms. Only chronic alcohol abuse can cause irreversible damage to movement coordination. In other cases, such as multiple sclerosis, a cure is not possible and permanent limitation may result. Affected persons are dependent on walking sticks or other aids. Supportive movement and speech therapies can be used with ataxias to improve or maintain these abilities.

Outlook and prognosis

The prognosis of ataxia depends on the underlying disease present. In Friedreich’s ataxia or a genetic disease such as multiple sclerosis, there is an unfavorable prognosis. The course of the disease is progressive and there is a shortening of life. Many patients are dependent on the help of others, walkers or a wheelchair for locomotion. If viral or bacterial infections are present, there is a much better chance of recovery. Once the original disease has been cured, the symptoms of ataxia disappear. The supply of the muscles as well as nerves takes place as usual and the coordination of movement is possible without complaints. In the case of alcoholism, substance abuse or due to excessive consumption of medications, a cure is also possible in most patients. As soon as the active substances have been completely and permanently removed from the organism, regeneration occurs after some time. The possibilities of movement start as usual and remain. In the case of a stroke, a heart attack or a tumor in the brain, the prospects for recovery are individual, but still not favorable. With a lot of training to build up the muscles and the coordination of movement, as well as good medical care, the symptoms can be alleviated with little damage. A complete cure is usually not possible.

Prevention

Prevention of ataxias is not possible in many cases and depends on the original disease. Avoiding obesity, high blood pressure, and cholesterol levels in the normal range and abstaining from nicotine will lower the risk of stroke. Abstaining from excessive alcohol, drug, or medication use is also useful.

Here’s what you can do yourself

If ataxia is suspected, a physician must initiate the appropriate treatment steps. Accompanying this, dealing with the disease can be made easier by some self-help measures. First, the condition should be diagnosed at the first signs so that treatment can be given at an early stage. In this way, severe secondary symptoms can still be avoided in many cases. Movement exercises such as yoga or physiotherapy can help against the Parkinson’s-like movement disorders and muscle cramps. The accompanying pain can be alleviated by painkillers, but also by pain-relieving teas. Depending on the type of ataxia, massages and sauna sessions can also help against the pain. If changes occur in the speech apparatus, these must be compensated for at an early stage by speech therapy so that good communication is still possible. Accompanying symptoms such as incontinence or swallowing difficulties should be alleviated by appropriate aids, depending on the clinical picture. Since the disease cannot be completely cured, it is necessary to learn how to deal with the limited movement in the long term. Self-help groups and discussions with friends and family members are recommended for this purpose. In addition, appropriate precautions must be taken to create an environment that is suitable for the disabled.