Ataxia is a brain disease of the cerebellum (along with its connections with other parts of the brain and spinal cord).
Ataxia is the broad description of a perplexing, sometimes fatal, condition that robs its victims of their ability to coordinate movement, the critical work of the cerebellum. Walking, talking, holding objects, eye contact, even swallowing become monumental and, eventually, insurmountable tasks. Cerebellar disease does not produce ataxia by making muscles weak, but rather by destroying muscle coordination and by making it difficult, and eventually impossible, to move any part of the body accurately.
Ataxia affects at least 150,000 Americans—three times the number of individuals affected by Lou Gehrig’s disease (ALS).
What is Ataxia?
There is currently no prevention or cure for the most prevalent forms of ataxia. There are several different types of ataxia. One-third of ataxias acquired after the age of 20 are hereditary. Ataxia strikes people from all walks of life. For some it appears as early as infancy. Certain forms develop during the adolescent years. Other forms of ataxia may suddenly affect individuals during the prime of life.
Most forms of Ataxia are hereditary, meaning that they do (or can) run in families. With some forms, it may only take the genes of one parent to pass along the disease. These are known as Dominant Ataxias. There are other forms of Ataxia that can only be contracted if both of the parents carry the gene for the disease. These are referred to as Recessive Ataxias.
All forms of hereditary Ataxias are caused by a defect in a certain gene present at the beginning of a person's life. Everyone has genes that have little mistakes or variations in them. Most variations do not cause disease. Occasionally, however, variations are significant enough to cause disease, including Ataxia.
Not all cases of Ataxia are genetically inherited, though. Environmental factors such as over-exposure to certain chemical toxins or head injuries can result in Ataxia also.
Furthermore, there are many cases of Ataxia without any known cause. These are known as Sporadic Ataxias. Many people diagnosed with Sporadic Ataxia have been re-diagnosed in the light of modern genetic testing techniques, as new genetic mutations causing Ataxia are discovered and named.
Symptoms and time of onset vary according to the type of Ataxia, and may also vary from person to person. Recessive disorders commonly cause symptoms to begin in childhood rather than adulthood (although for reasons that are not understood, symptoms are not necessarily present at birth or in infancy). Dominant Ataxias typically begin in the 20’s or 30’s or even later in life. Occasionally, individuals may not show symptoms until they are in their 60’s.
Balance and coordination are affected first. What may just seem like "clumsy" movements may not be diagnosed until symptoms become more pronounced, often over a period of years. Coordination affects both arms and legs. Walking becomes difficult. A wheelchair often becomes necessary as the Ataxia progresses.
Lack of coordination of the arms and hands affects the person's ability to perform tasks that require fine motor control such as writing and eating. Slow eye movements or limited range of eye movements can be seen in some forms of Ataxia.
Who is at risk?
What causes Ataxia?
What are common symptoms?
Since 2004, BAARC has allocated over $1 million in start-up money to help University of Minnesota researchers in the exploratory studies of Ataxia. This initial seed money was leveraged to secure basic science projects and clinical research grants of more than $6 Million from the National Institute of Health.
Your support of the Stand Up 2 Ataxia Benefit will directly fund research that is searching for treatment options and pursuing a cure for ataxia. Thank you.
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