Episodic Ataxia

Episodic ataxia explained

Ataxia refers to a lack of coordination in muscle movement. When we use our muscles, our brain sends complex signals to the affected areas. By coordinating our muscles, the body is able to perform tasks, such as walking or running.

When ataxia occurs, however, this voluntary movement is limited. While patients may intend for their muscles to move, the signal is not properly received and, therefore, movement is inhibited.

Ataxia itself isn’t linked to a particular disease. Instead, it can occur as a result of numerous conditions. While most people find that ataxia affects both sides of their body, on occasion patients may just experience ataxia on one side. This form of the condition, known as hemiataxia, can be just as debilitating for sufferers.

When does episodic ataxia occur?

Episodic ataxia means that the condition occurs periodically, rather than continuously. When a patient is affected by an episode, they may experience poor coordination and balance. In addition to this, episodic ataxia can result in extreme dizziness, nausea and vertigo.

This combination of symptoms means that patients can struggle to perform their daily activities when episodic ataxia strikes. As a result, people may need to take time off work or school due to the condition.

However, episodic ataxia can vary and there are currently seven different types of the condition which are recognized. Despite seven varying types of episodic ataxia, the condition is normally split into types one and two when determining which treatment may be appropriate.

What causes ataxia?

In general, ataxia can have a number of causes. Focal lesions, depression of the central nervous system and even B12 deficiency can cause ataxia to occur. However, some forms of the condition, such as episodic ataxia, are hereditary.

If a hereditary disorder affects the cerebellum or the spine, the patient may suffer from mobility issues. If both damage of the cerebellum and spine is present, ataxia may occur. Episodic ataxia, along with spinocerebellar ataxia, dentatorubropallidoluysian atrophy and Friedreich’s ataxia are all forms of hereditary ataxia.

Can episodic ataxia be treated?

Although there are treatments for ataxia, the effectiveness of medical interventions depends on the type of ataxia present and its underlying cause.

In cases of episodic ataxia, medications can be used to limit the impact of the condition. In addition to this, genetic testing and counseling can be arranged if a patient wishes to have a child. Generally, if someone is affected by episodic ataxia, there will be a genetic basis and a subsequent 50% chance that they will pass the condition on to their child, assuming the other parent does not carry the affected gene.

Traditionally, carbamazepine, valproic acid and acetazolamide have had beneficial effects when used to treat type one episodic ataxia, while 4-aminopyridine has been used in the treatment of type two.

It is believed that medications which activate calcium-activated potassium channels could also be effective in treating type two episodic ataxia but, as yet, these have not been made readily available.

Generally, attacks of episodic ataxia can be managed well with the use of established medication patterns. Once a therapeutic dose has been reached, the attacks of episodic ataxia should become less frequent and less severe.

However, there are lifestyle changes which could help to prevent attacks from occurring. Patients may wish to incorporate these changes into their treatment regime, in addition to their prescribed medications.

Stress, exertion, caffeine, alcohol consumption, heat and fever have all been associated with episodic ataxia. While medication can stop the attacks, regardless of their cause, if patients manage stress well and reduce exposure to potential triggers, attacks of episodic ataxia may reduce further.

Management of episodic ataxia

Although episodic ataxia can be a debilitating condition, it can be well managed. As the condition is fairly rare, it’s important for patients to seek help from a medical professional with a particular interest in ataxia. Once the patient has been assessed and a diagnosis made, an experienced physician with extensive knowledge of the condition will be able to provide an effective treatment plan.

While patients may need monitoring at the start of treatment, once the correct medication and dose has been established, patients with episodic ataxia may need no more than a yearly check-up to ensure that the treatment continues to be effective.

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Last Reviewed:
July 12, 2017
Last Updated:
October 24, 2017