Ataxia

Ataxia means ”a lack of order”; people with ataxia experience a failure of muscle control in their arms and legs, resulting in a lack of balance and coordination or a disturbance in their gait. Ataxia may also affect the movement of your fingers, hands and entire body, and it may also compromise your speech and alter your eye movements.    

Ataxia results when cells in the cerebellum degenerate or atrophy; the spine and the peripheral nerves that connect the spine to the cerebellum may be affected too.
 
The symptoms and time of onset of ataxia vary according to the underlying cause of the disease and type of ataxia you have (and with the inherited forms, can vary from person to person even with the same type of the condition). The most common symptoms of ataxia across all forms include loss of balance and coordination, slurred speech, slow eye movement, difficulty in swallowing and trouble with walking. The loss of balance can interfere with your ability to stand and walk and the loss of fine motor control can make writing and eating a trying task.

Types of Ataxia 
Ataxia occurs when the nerve cells in the cerebellum (the part of your brain that controls motor function) suffer damage, degeneration or death. Ataxia can be acquired as a symptom of another condition, inherited through variants of a number of genes or appear without a verifiable cause (also known as sporadic ataxia).

• Acquired ataxia conditions include trauma or injury to the brain or spinal cord , stroke, a transient ischemic attack (TIA), cerebral palsy, multiple sclerosis (MS), the chickenpox virus, tumors in the cerebellum, paraneoplastic syndromes (rare, degenerative disorders caused by your immune system’s response to cancerous tumors), a toxic reaction to medications, drugs or chemicals (especially barbiturates, sedatives, heavy metals, solvents, alcohol and recreational drugs) and a deficiency in vitamin E or B-12. 
• Hereditary ataxia occurs when you inherit either a dominant gene variant from one parent (called autosomal dominant ataxia) or a pair of recessive gene variants from both parents (autosomal recessive ataxia).
  
  • The autosomal dominant form includes the spinocerebellar ataxia group, a progressive degenerative condition that may be caused by 1 of 20 identified genes. Some symptoms, as well as age of onset, differ depending on the gene mutation involved. This group is also represented by episodic ataxia (in which the episodes are triggered by stress and sudden, startling movements. Seven versions of this type of ataxia exist, labeled as EA1 through EA7. Some of these forms resolve on their own in later life and the symptoms may respond to medication.
  • The autosomal recessive form includes Friedreich's ataxia (a common, progressive form of hereditary ataxia), ataxia-telangiectasia (a rare, progressive form that appears in childhood), congenital cerebellar ataxia (damage to the cerebellum at birth) and Wilson disease (caused by the accumulation of copper in brains, livers and other organs). 
• Sporadic ataxia may appear in a number of forms, including multiple system atrophy.

Diagnosis of Ataxia
At Houston Methodist, we will evaluate your medical and family history and perform a neurological examination, including an MRI scan (magnetic resonance imaging). Our team may recommend various blood tests to discover or rule out other possible disorders that may present similar symptoms. 
Genetic blood tests are now available for some types of hereditary ataxia to confirm a diagnosis or as a predictive test to determine if someone has inherited an ataxia gene known to affect other family members.

Treatment Options for Ataxia  
Although there is no cure for a hereditary ataxia, an ataxia caused by another condition may be cured by treating the underlying condition. For example, ataxia caused by a metabolic disorder may be treated with medications and a controlled diet; ataxia due to vitamin deficiency is treated with vitamin therapy. 

A variety of drugs may be used to either effectively prevent ataxia symptoms or reduce the frequency with which they occur. Physical therapy can strengthen muscles, while special devices or appliances can assist in walking and other activities of daily life.

Ataxias are complex disorders that require a comprehensive approach by specialists devoted to their evaluation and management. The comprehensive team approach we take at Houston Methodist involves experienced neurologists, neurosurgeons, neuropsychologists, physical therapists, speech therapists and others as appropriate. We use the latest treatment and in technology and diligently listen to our patients and their families. Our team evaluates and treats all these aspects in order to maximize the quality of our patients' lives.