Cerebellar Ataxia Can Be Treated | News | Loyola Medicine
Monday, March 23, 2015

Cerebellar ataxia can't be cured, but some cases can be treated

Vitamin E, gluten-free diets among the effective treatments for certain ataxias

MAYWOOD, Ill. (March 23, 2015) –  No cures are possible for most patients who suffer debilitating movement disorders called cerebellar ataxias.

But in a few of these disorders, patients can be effectively treated with regimens such as prescription drugs, high doses of vitamin E and gluten-free diets, according to a review article in the journal Movement Disorders.

“Clinicians must become familiar with these disorders, because maximal therapeutic benefit is only possible when done early. These uncommon conditions represent a unique opportunity to treat incurable and progressive diseases,” first author Adolfo Ramirez-Zamora, MD, co-author José Biller, MD, and colleagues report. Dr. Ramirez-Zamora is an assistant professor of neurology and the Phyllis E. Dake Endowed Chair in Movement Disorders at Albany Medical Center Department of Neurology, and a former chief neurology resident at Loyola University Chicago. Dr. Biller is chair of the Department of Neurology of Loyola University Chicago Stritch School of Medicine.

The word ataxia means without co-ordination. Ataxia symptoms include poor coordination, unsteady walk, difficulty speaking and swallowing, involuntary back-and-forth eye movements and difficulty performing fine-motor skills such as writing or buttoning a shirt. Hereditary ataxias are degenerative and progress over time. Ataxias usually are due to damage to the cerebellum, a part of the brain that controls muscle coordination.  

Chronic cerebellar injury due to alcohol or other commonly used drugs such as lithium can be treated by discontinuing the offending drugs, the review article said.

Below is a sampling of other effective treatments for cerebellar ataxias detailed in the review article:

Ataxia with vitamin E deficiency (AVED) impairs the body’s ability to use vitamin E, resulting in ataxia and other debilitating symptoms. High doses of vitamin E (800 mg/d) typically stop disease progression and lead to neurological improvement – although recovery may be slow and incomplete. “The results of vitamin E supplementation also seem to be most beneficial if started in patients with less than 15 years of disease duration; the sooner after diagnosis the supplementation is begun, the better,” the article said.

Cerebrotendinous xanthomatosis (CTX) is treated with chenodeoxycholic acid, which is made from naturally occurring bile acid. Beginning treatment early is crucial to preventing neurological deterioration.

Gluten ataxia can be treated with a strict gluten-free diet. Glutose transporter type 1 deficiency can be treated with a high-fat, low-carb ketogenic diet. Glutamic acid decarboxylase ataxia can be treated with intravenous immunoglobulin (IVIG) or steroids.

The review article is titled “Treatable Causes of Cerebellar Ataxia.” In addition to Drs. Ramirez-Zamora and Biller, co-authors are Warren Zeigler, MD, and Neeja Desai, MD, both of Albany Medical Center.

About Loyola Medicine and Trinity Health

Loyola Medicine, a member of Trinity Health, is a quaternary care system based in the western suburbs of Chicago that includes Loyola University Medical Center (LUMC), Gottlieb Memorial Hospital, MacNeal Hospital and convenient locations offering primary and specialty care services from 1,877 physicians throughout Cook, Will and DuPage counties. LUMC is a 547-licensed-bed hospital in Maywood that includes the William G. and Mary A. Ryan Center for Heart & Vascular Medicine, the Cardinal Bernardin Cancer Center, a Level 1 trauma center, Illinois's largest burn center, a certified comprehensive stroke center and a children’s hospital. Having delivered compassionate care for over 50 years, Loyola also trains the next generation of caregivers through its teaching affiliation with Loyola University Chicago’s Stritch School of Medicine and Marcella Niehoff School of Nursing. Gottlieb is a 247-licensed-bed community hospital in Melrose Park with 150 physician offices, an adult day care program, the Gottlieb Center for Fitness, the Loyola Center for Metabolic Surgery and Bariatric Care and the Loyola Cancer Care & Research at the Marjorie G. Weinberg Cancer Center at Melrose Park. MacNeal Hospital is a 374-bed teaching hospital in Berwyn with advanced inpatient and outpatient medical, surgical and psychiatric services, advanced diagnostics and treatments. MacNeal has a 12-bed acute rehabilitation unit, a 25-bed inpatient skilled nursing facility, and a 68-bed behavioral health program and community clinics. MacNeal has provided quality, patient-centered care to the near west suburbs since 1919.

Trinity Health is one of the largest multi-institutional Catholic healthcare systems in the nation, serving diverse communities that include more than 30 million people across 22 states. Trinity Health includes 94 hospitals, as well as 109 continuing care locations that include PACE programs, senior living facilities and home care and hospice services. Its continuing care programs provide nearly 2.5 million visits annually. Based in Livonia, Mich., and with annual operating revenues of $18.3 billion and assets of $26.2 billion, the organization returns $1.1 billion to its communities annually in the form of charity care and other community benefit programs. Trinity employs about 133,000 colleagues, including 7,800 employed physicians and clinicians. Committed to those who are poor and underserved in its communities, Trinity is known for its focus on the country's aging population. As a single, unified ministry, the organization is the innovator of Senior Emergency Departments, the largest not-for-profit provider of home health care services—ranked by number of visits—in the nation, as well as the nation’s leading provider of PACE (Program of All Inclusive Care for the Elderly) based on the number of available programs.