Ataxia

Symptoms of ataxia

Always contact your GP if you (or your child) begin to experience unexplained problems with physical co-ordination, balance, walking, talking or swallowing.

Symptoms of ataxia

The first noticeable symptoms of ataxia are usually loss of balance and co-ordination in your hands, arms and legs.

Walking becomes increasingly difficult and many people walk with their feet further apart to compensate for their loss of balance.

Actions that require a good degree of physical control (motor control), such as writing, will become more difficult.

The condition then may affect the voice, mouth and tongue, which can cause:

  • speech to become increasingly slurred, slow and unclear – the medical term for this is dysarthria
  • difficulty swallowing – the medical term for this is dysphagia

Many people with hereditary ataxia experience increasing weakness in their legs, which means that they will require a wheelchair at some point.

Other symptoms of hereditary ataxia can include:

  • unusual and involuntary eye movements, such as moving the eyes from side to side or up and down – this is known as oscillopsia
  • shaking, usually in the hands when trying to use them

Some forms of ataxia have other symptoms, which can include:

  • disturbance of bladder and bowel function
  • memory loss
  • depression and anxiety
  • loss of feeling in the arms and legs
  • loss of strength in the arms and legs

Specific symptoms of hereditary ataxia

There are several signs and symptoms that are specific to certain types of hereditary ataxia. The symptoms and their associated types are explained below.

Friedreich’s ataxia

Friedreich’s ataxia is the most common type of hereditary ataxia with symptoms usually first developing during childhood between the ages of eight and 15 years.

Signs and symptoms specific to Friedreich’s ataxia include:

  • deformity of the feet, such as high arches or abnormal curvature of the toes (known as hammer toes)
  • abnormal curvature of the spine to one side (scoliosis)
  • damage to the optic nerve, which may result in the loss of some or all vision
  • hearing loss 
  • diabetes 
  • thickening of the muscles of the heart (hypertrophic cardiomyopathy), which causes breathlessness, chest pain and an irregular heartbeat

Ataxia-telangiectasia

Ataxia-telangiectasia (AT) is a rare type of hereditary ataxia. Around one in every 100,000 children are born with AT.

Symptoms usually begin in early childhood and then rapidly worsen after the age of five. Most children with AT will require a wheelchair by the time they are 10 years old.

Many children with ataxia-telangiectasia will develop small, spider-like clusters of red blood vessels in the corner of their eyes and on their cheeks. These are called telangiectases and it is from these that the condition derives its name. 

They also usually have a weakened immune system that makes them more vulnerable to infections, particularly infections of the lungs and airways, such as pneumonia.

An estimated one in five children with ataxia-telangiectasia will also develop cancer. This will usually be in the form of acute lymphoblastic leukaemia (cancer of the white blood cells) or lymphoma (cancer of the lymph nodes).

Ataxia with vitamin E deficiency

Ataxia with vitamin E deficiency is a rare type of ataxia that begins in childhood. It occurs when the body does not produce enough vitamin E, which causes nerve damage.

Ataxia with vitamin E deficiency has the same symptoms as Friedreich’s ataxia. However, unlike Friedreich’s ataxia, it is usually possible to control the symptoms by taking vitamin E supplements.

Spinocerebellar ataxias

Spinocerebellar ataxias (SCAs) are a range of different types of hereditary ataxias that affect around one in every 100,000 people.

Unlike other forms of hereditary ataxias, the symptoms of an SCA do not usually begin until adulthood. Depending on the type of SCA, this could be from the age of 30 up until the age of 70 (although, occasionally, some types of SCA begin in childhood).

The symptoms of an SCA can vary depending on the specific sub-type and can include:

  • muscle stiffness and cramps
  • reduced sensation in the hands and feet
  • mild cognitive problems, such as some memory loss or difficulties with spoken language
  • slow eye movement, which means people have to move their head to compensate
  • muscle cramps
  • reduced sensation in the hands and feet
  • urinary incontinence 
  • an uncomfortable feeling in the legs that makes someone want to move their legs (this combination of symptoms is known as restless legs syndrome)
  • episodes of vertigo (vertigo is the sensation that you, or the environment around you, are moving or spinning)
  • episodes of migraine (a severe headache that is often accompanied by feelings of nausea)
  • a progressive loss of vision that can often result in blindness

Ataxia UK has more detailed information on the most common sub-types of SCA.

Episodic ataxia

Episodic ataxia is a rare and unusual type of hereditary ataxia. In episodic ataxia, a person experiences periods of ataxia, while the rest of the time they have no symptoms.

The symptoms of episodic ataxia usually begin during the teenage years. The periods of ataxia can last from several minutes to six hours, and they are usually the result of certain triggers such as stress, exercise or excitement.

The symptoms of episodic ataxia often disappear as a person reaches middle age and do not affect a person’s life span.

Acquired ataxia

The symptoms of acquired ataxia are usually the same as the general symptoms of hereditary ataxia as discussed above, though they can often develop very quickly over the course of a few days, or in some cases, hours.

Idiopathic late onset cerebellar ataxia (ILOA)

The symptoms of idiopathic late onset cerebellar ataxia (ILOA) usually begin around the age of 50 and then slowly get worse over time. Most people with ILOA will require a walking aid or wheelchair 10 years after the onset of symptoms.

Symptoms specific to ILOA include:

  • double vision 
  • disturbed sleep often with associated snoring
  • urinary incontinence
  • some mild degree of learning difficulties
Last updated: 04 June 2013

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