• Ataxia

Pediatric Ataxia

Ataxia is a family of neurological conditions which share the primary symptom of severely uncoordinated movement. Symptoms can also include slurred speech and difficulties eating and swallowing.  

Ataxia has many root causes and occurs when the brain’s cerebellum begins to degenerate. The cerebellum, located near the base of the skull, is sometimes called the “second brain,” due to its resemblance to the cerebrum, the primary structure of the brain. It is the center of unconscious coordination and allows us to learn to walk in a balanced and coordinated way, to run, to eat with utensils and perform all manner of everyday movement.The condition can be acquired through viruses, develop congenitally, or result from a traumatic event or genetic condition.  

In children, ataxia is very rare. The diseases and conditions which cause it are myriad, including cerebral palsy, multiple sclerosis, and stroke. Addressing the underlying condition is the first step to recovery, which is dependent upon the level of damage done to the cerebellar-spinal area. In some cases, it is temporary (as in some post-infectious cases), but even in those, children will usually require physical therapy to recover their coordination.

Types of Ataxia:

  • Post-infection, Post-Trauma, and Other Acquired Ataxias

Pediatric ataxia is most commonly a result of infection or injury to the brain, causing damage to the cerebellum or sensory pathways of the spinal cord that allow a person to sense themselves in space (proprioception). Stoke, traumatic brain injury, tumors, vitamin deficiencies (e.g. folate), poisoning (e.g. lead, alcohol, some anti-seizure medications), and infections (e.g., chicken pox, Coxsackie virus, influenza, and Epstein-Barr viruses) are the most common causes of acquired ataxia in children. Ataxias that are caused by infection and injury generally have a sudden, acute onset, but progression can often be arrested. In many cases ataxic symptoms may disappear with treatment of the underlying disease.

  • Congenital Ataxias

Some ataxias are the result of problems during prenatal development. Chiari malformations, Dandy-Walker syndrome, and encephalocele are three of the most common congenital ataxias. While these ataxias are present at birth and chronic, they are not progressively degenerative.

  • Hereditary Ataxias

Genetic disorders may also cause ataxia. Ataxia telangiectasia and Friedreich’s ataxia are chronic and progressively degenerative diseases caused by two different recessive gene mutations, meaning that a child must inherit the gene from both parents for it to cause the disease.

  • Friedreich’s ataxia is the most common inherited type of ataxia and affects 1 in 30,000 children. A gene mutation in Friedreich’s ataxia causes damage to mitochondria, the organelles which provide energy to cells. Friedreich’s ataxia appears late in childhood, usually between the ages of 5 to 15. While Friedreich’s ataxia is a progressively degenerative disease, it does not typically affect mental ability. While research for drug therapies are active, rigorous physical therapy protocols are being developed that slow the loss of coordination and function. There is currently no cure. 
  • Ataxia Telangiectasia is a very rare disease that may affect as few as 1 in 100,000 children. The gene mutation that causes ataxia telangiectasia results in cells' failure to repair cellular DNA, creating a cascade of symptoms over time. Signs of ataxia telangiectasia may be noted as early as the stage when walking and standing begin to develop, and may often be misdiagnosed at that time as cerebral palsy. Children with ataxia telangiectasia have trouble standing and walking without assistance. Gross and fine motor movements, as well as the ability to control eye and vocal movement, deteriorate over time. Between ages 5 to 8 children may develop permanent “blood-shot eyes” and prominent small broken blood vessels on the face and neck--called telangiectasia. Over time, the immune system also begins to weaken. Mental abilities are sometimes negatively affected. Ataxia telangiectasia is definitively diagnosed with genetic testing to distinguish it from cerebral palsy and Friedrich's ataxia, which may be done after positive clinical exams and brain scans. Currently, no cure exists for ataxia telangiectasia. Most therapies focus on supportive care and symptomatic treatment, with specific treatment for related conditions.

The diseases and conditions that cause ataxia are myriad. Addressing the underlying condition first leads to treatment of the cause of ataxia.

Conventional Treatment

A physical exam is the first stage of ataxia diagnosis. A wide, staggering gait while walking is a hallmark sign of the condition. Many children with ataxia are unable to walk in a straight line, and their movements are entirely uncoordinated. Abnormal eye movements, called nystagmus, as well as the inability to judge depth, are other key signs of ataxia that can be seen in a physical exam. A definitive diagnosis is usually made with brain scans, blood work, and sometimes genetic testing.

Treatment plans for all ataxias almost always call for as much exercise as possible in addition to physical and occupational therapy. Addressing the emotional needs of children with ataxia is often paramount, especially if they have a chronic, progressive form of the condition. If speech has been affected, speech therapy is also prescribed. Depending upon the root cause of ataxia, additional specific treatments are available. 

Support

At The Brain Possible, our goal is to empower you to take a holistic approach to your child’s treatment. Below are ways in which you can support several aspects of your child’s recovery; before embarking on any, be sure to discuss them with your trusted health care providers.

Physical

Courses of physical and occupational therapy are recommended for most patients. If the condition progresses, supportive devices such as canes, hiking sticks, walkers, and electric scooters may be prescribed. Manual therapies such as craniosacral and scalp acupuncture, which encourages the flow of energy through meridians in the head and body, and massage modalities such as lymphatic drainage may ease symptoms of ataxia.

Emotional/Social

As children’s symptoms progress, they may suffer from teasing at school and need additional emotional support to process their feelings of not fitting in or having the ability to do “normal” activities. In addition, their siblings may suffer from feelings of guilt or resentment. To read first-person accounts of how pediatric ataxia impacts families and individuals, read this collection of stories from the National Ataxia Foundation

Sensory

Sensory therapies may be carefully tailored to the patient’s type of ataxia. In a recent study, children benefited greatly from physical therapy that was directed by video games, enabling them to coordinate their physical skill building and sensory intake. Hydrotherapy, which uses water as a setting for occupational therapy and/or as a means of transferring heat or cold to the body, may help with relaxation and neurological integration of sensory experiences.

Intellectual

Some ataxias cause intellectual impairment; others do not. If a child with ataxia is presenting cognitive or psychiatric challenges, it’s important to learn how her neurological condition may be impacting these aspects of her development. Then, a detailed treatment plan can be put in place by her care team, which may include behavior therapy and educational therapy. 

Nutritional/Environmental

Some ataxias are directly related to nutritional deficiencies, including deficiencies of vitamin E and coenzyme Q10. A 2015 study found that patients with cerebellar ataxia benefitted from high doses of vitamin E and gluten-free diets. The Ketogenic Diet is a high-fat diet that changes the brain metabolism and has been shown to improve many neurological conditions. In their dietary guidelines, The National Ataxia Foundation also discourages eating foods containing aspartame, sulfites, tyramine, MSG and chocolates.

Physiological

Antibiotics are usually prescribed for virus-induced ataxias. Ensuring that your child is up-to-date on vaccines is recommended to reduce risk to viruses that result in ataxia. Caregivers have reported reduced symptoms using homeopathy. Homeopathic physician Vikas Sharma, MD recommends Belladonna to reduce the unsteady gait and Zincum Met to improve muscle coordination. Immunotherapy has greatly improved some ataxia patients, as has the antiviral drug Amantadine.