What Is Ataxia?

Overview

Ataxia is described as having difficulty controlling voluntary muscle movements, resulting in uncoordinated, clumsy, or awkward movements.¹ This uncoordinated movement can be seen in your limbs and even your eyes. Ataxia is typically a symptom of underlying conditions, such as a stroke or a genetic defect, and is not a disease in itself.²

There are different types of ataxia and therefore different causes. Lack of coordination when walking is usually the first sign of ataxia, other symptoms such as trouble swallowing and writing may also occur. Management and treatment options for ataxia largely depend on the cause of the ataxia. Management options may involve scheduling sessions with your physiotherapist who may focus on working on your balance and strength and may provide you with walking aids if necessary. 

Types of ataxia

The type of ataxia you may have depends on what part of your body has been affected. There are two main types of ataxia:

Cerebellar ataxia: This type of ataxia occurs when there has been damage or destruction to your cerebellum, the part of your brain that is involved in orchestrating the coordinated movement of your limbs and eyes. Therefore, damage to this area can lead to clumsiness. This type of ataxia affects your walking pattern, also referred to as gait, first.³ Causes of cerebellar ataxia include multiple sclerosis, meningitis, and tumours.⁴ 

Sensory ataxia: Not everyone with ataxia has an underlying disease; for others, ataxia is a result of a sensory issue.⁴ Coordination has a sensory component to it, it involves adequate proprioceptive senses. This involves knowing where your body parts are in relation to space at any given time and requires sensory input from your muscles. Therefore, damage to your sensory pathways may result in a lack of coordination of your voluntary muscle movements, and therefore ataxia.^2,3 

Causes of ataxia

The causes of ataxia can be acquired, inherited, or sporadic. 

Acquired ataxia is caused by damage to the brain or the nervous system.⁵ These problems include:

• Traumatic brain injury
• Tumours
• Infection
• Drugs
• Alcohol intoxication
• Vitamin deficiencies³

In contrast, inherited or hereditary ataxia is caused by a gene mutation and may develop over a number of years. The onset of this ataxia may be observed in childhood or adulthood depending on the gene that has the mutation.^2,3 

Common types of inherited ataxia:

• Ataxia telangiectasia: most common type of ataxia in children under 5 years old. 
• Friedreich ataxia: a result of a mutation of the FXN gene. Usually affects gait first and has its onset in early childhood. Later symptoms include difficulty with speech and  swallowing
• Ataxia with Vitamin E deficiency: is a result of a mutation in the gene that encodes for TTP1

Sporadic Ataxia: This is ataxia that is neither acquired nor inherited (due to genetic causes). It occurs spontaneously, without a known cause.¹ 

Signs and symptoms of ataxia

• Struggling with balance and coordination which affects gait (usually the first symptom)
• Impaired speech (e.g., slurring words)
• Trouble swallowing
• Difficulty writing or performing precise hand movements
• Slow/uncoordinated eye movements
• Double or blurry vision^4,5

Management and treatment for ataxia

Management and treatment options are largely dictated by the cause of ataxia. Some cases may resolve on their own and do not need any medical attention (e.g., alcohol intoxication). However, there is no cure for hereditary ataxias and in these scenarios, management options usually look at alleviating symptoms.

Treatment of acquired ataxia is achieved through treating the underlying condition.⁶ Like inherited ataxia, your healthcare professional may prescribe medication to help manage associated symptoms such as stiffness, spasticity, or pain. 

You also may be referred to a physiotherapist or occupational therapist who may work on your balance and coordination with you. They may also give you walking aids if walking is affecting your day-to-day activities.⁶ A speech therapist may help those who have difficulty swallowing and speaking. 

FAQs

How is ataxia diagnosed?

There are a few things your doctor may do to test for and subsequently diagnose ataxia. They will ask for your past medical history and family history to check for a genetic link.

Other tests your healthcare professional may run to check for the underlying cause of your ataxia include:

• Physical examination
• Neurological examination
• Blood tests
• Genetic testing
• MRI scan

How can I prevent ataxia?

From the causes listed in previous sections, you can observe that some of the causes are preventable if you avoid certain situations. Others, however, are more unpredictable and are not preventable.

Prevention methods:

• Drink in moderation or refrain from consuming alcohol 
• Wear helmets and protective gear when necessary (e.g., cycling) to prevent head injuries
• Stick to taking your prescription drugs in accordance with the instructions on your prescription and avoid taking recreational drugs
• Avoid infections, especially ear infections, by practicing good hygiene and seeking medical attention in a timely manner when you suspect an infection 
• Eat at a balanced diet to avoid vitamin deficiencies²

Who is at risk of ataxia?

There are certain things that put people more at risk of ataxia:

• Viral infections (e.g., chickenpox and HIV)
• Bacterial Infections (e.g., TB)
• Stroke 
• Multiple Sclerosis
• Tumour
• Cerebellar blood clot
• Obstruction of an artery
• Family history of inherited ataxia
• Vertigo
• Migraines

How common is ataxia?

According to Ataxia UK, the most common type of inherited ataxia is Friedreich ataxia and its prevalence in the population in Europe is between 1 in 20,000 and 1 in 50,000. The prevalence of childhood ataxia in Europe is estimated to be 26 in 100,000.

When should I see a doctor?

Ataxia can be an indicator that you have a more serious medical condition or problem that needs attention. Speak to your healthcare provider if you experience any of the following:

• Ataxia is getting worse
• Headache
• Vomiting
• Nausea
• Trouble swallowing or speaking
• Uncontrolled eye movements
• Affects your daily routines 

Summary

Ataxia is characterised by a lack of coordination in muscles when performing voluntary activities such as walking. It is commonly a symptom of other conditions such as stroke and traumatic brain injuries and is not a disease itself. Management options for ataxia focus on treating the underlying condition and/or alleviating symptoms. This can be through the treatment plans provided by your physiotherapist or occupational therapist or through medication prescribed by your healthcare professional.