How Ataxia Progresses
Ataxia is a loss of control of body movements. It can be a symptom of progressive neuromuscular diseases, such as multiple sclerosis, or a symptom of an acute injury to the brain via a traumatic head injury or stroke. The word ataxia is also used to describe a group of inherited degenerative diseases of the nervous system.
If ataxia is caused by an acute injury or infection, it doesn’t usually worsen. In fact, the symptoms of ataxia—balance problems, difficulty with standing or walking, swallowing difficulty—may improve as the body heals.
Other cases of ataxia progress over time. Learn more about ataxia progression, how treatment changes over time, and how the prognosis varies across ataxia types.
Episodic ataxia is a rare, hereditary type of ataxia that’s characterized by occasional episodes of ataxia. During an episode, an affected individual may wobble while walking, slur their speech, have difficulty controlling eye movements, or experience involuntary muscle movements. The rest of the time, they can walk, move and function normally or with only minor difficulty.
The first symptoms of episodic ataxia usually appear in the teenage years. Unlike most hereditary ataxias, episodic ataxia may disappear as the person grows older. In other cases, the symptoms of ataxia become progressively worse.
Symptoms of Friedreich’s ataxia, an inherited form of ataxia, typically show up before the child turns 15 and as early as 5 years old. In approximately 15% of cases, symptoms don’t appear until after age 25.
Early symptoms of Friedreich’s ataxia include difficulty walking, decreased sensation in the arms and legs, poor balance, and slurred speech. As the disease progresses, muscle weakness increases and walking becomes more difficult. Affected individuals may also experience involuntary spasms of the arms and legs. Most people with Friedreich’s ataxia are wheelchair-dependent within 10 to 20 years of the onset of symptoms.
Eventually, many people with Friedreich’s ataxia also develop scoliosis (a curving of the spine) and heart disease. Approximately 30% of sufferers develop diabetes. Friedreich’s ataxia often leads to a shortened life span. Some affected individuals live into their 60s, but many die sooner.
Ataxia telangiectasia (A-T) is a rare, hereditary form of ataxia. Symptoms usually become apparent in the toddler years, when children are learning to sit, stand and walk. All children are wobbly when they first learn these skills, but children with A-T have pronounced and persistent problems with balance and coordination.
School-age children may have difficulty controlling their eye movements; speech may become slurred as well. Eventually, A-T children lose the ability to walk and even read (because they can no longer control their eye movements). By age 10, most children with ataxia telangiectasia need a wheelchair.
A-T significantly shortens the lifespan. Most people with A-T only live into their late teens or early 20s. A few people survive into their 40s or 50s.
Spinocerebellar ataxia (SCA) is a group of hereditary ataxias. Though there are some differences between subtypes, each is a progressive disease. Symptoms—clumsiness, lack of coordination, poor balance—often begin in adulthood and worsen over time.
Like other ataxias, treatment teams focus on muscle strength and adaptive devices to help SCA patients maintain their independence for as long as possible. Canes, walkers, scooters and wheelchairs help with walking. Speech-generating devices help with communication.
The cerebellum is the part of the brain that controls movement. Damage to the cerebellum—via an injury, chronic disease or infection—can cause difficulty with movement, repetitive eye movements, slurred speech, and difficulty swallowing (dysphagia).
If cerebellar ataxia is caused by an infection, symptoms usually resolve over a period of months as the body recovers. Ataxia may also resolve after healing from a brain injury or after surgical treatment of an underlying cause.
In other cases, cerebellar ataxia progresses over time. Still, therapeutic interventions, such as physical therapy, occupational therapy, and speech therapy are an important part of the care plan because they condition and train the muscles to make them more effective. Therapists also add assistive devices, including walkers, wheelchairs, eating tools, and speech generators to treatment as necessary.
Doctors use a variety of medications to control ataxia and other symptoms, but they are not a cure. Many of the medicines are prescribed ‘off label,’ which means they are not specifically approved for ataxia. There are medicines to help with balance, tremor, eye movements, muscle cramps, muscle spasticity, and more. Research is ongoing for new, more effective medications, and some are in clinical trials. Recently, the medication dalfampridine (Ampyra) was approved for improving walking in multiple sclerosis patients.
In late stages of a progressive ataxia, difficulty swallowing becomes a serious problem because of the risk of choking and aspirating food into the airways. Tube feeding prevents this life-threatening complication associated with ataxia.
Supportive and palliative medical care to prevent discomfort is essential towards the end of life. If you or a loved one has a progressive ataxia, connect with a support group through the National Ataxia Foundation.