Cerebellar Ataxia Due to CoQ10 deficiency

June 12, 2003--A disorder known as cerebellar ataxia may be due to a deficiency of coenzyme Q10 (CoQ10), according to a new study in Neurology (2003;60:1206–8). Cerebellar ataxia is a genetic condition that primarily affects coordination of muscles, causing problems with walking, writing, speaking, and standing up straight. The study suggests that supplementation with CoQ10 may improve the symptoms associated with cerebellar ataxia.

In addition to the symptoms described above, cerebellar ataxia may also be associated with seizures, muscle weakness, delayed motor development, and mental retardation. The most striking symptom of cerebellar ataxia is an altered walking pattern, observed as a staggering, uncoordinated gait with the legs spread wide apart. Studies suggest that different genetic defects may be involved in causing cerebellar ataxia in different people, so it is unlikely that all forms of cerebellar ataxia are due to any single factor. CoQ10 deficiency may represent one of these genetic variants.

In the new study, muscle biopsies were taken from 135 people between the ages of 6 and 35 years with genetically undefined cerebellar ataxia. Most of the participants also had other neurological conditions, such as seizures, developmental delay, and mental retardation.

The results showed that 3% of those with adult-onset and 10% of those with childhood-onset cerebellar ataxia had decreased levels of CoQ10 in their muscles. The lowest levels were found in those with childhood-onset cerebellar ataxia (those in whom symptoms began before the age of ten). Those with CoQ10 deficiency had muscle levels at least 35% lower than the levels found in other individuals with cerebellar ataxia who did not have CoQ10 deficiency.

Each person identified with CoQ10 deficiency was started on supplemental CoQ10, with amounts ranging from 200 to 900 mg per day. The amount of CoQ10 taken varied due to availability and cost. Supplementation with CoQ10 led to improvement in symptoms in more than 75% of those treated. Some of the improvements observed included better posture and gait, clearer speech articulation, and decreases in seizure activity. None of the adults with low muscle CoQ10 concentrations responded to CoQ10 treatment, suggesting the benefits of CoQ10 supplementation may be limited to children.

CoQ10 as a treatment for cerebellar ataxia appears to be most appropriate for people who are under the age of 12. The authors believe that cerebellar ataxia in some individuals may be due to a defect in CoQ10 metabolism that interferes with normal coordination. However, since CoQ10 supplementation was only given to those with low amounts in their muscles, it is unknown whether the treatment would also be beneficial for others with cerebellar ataxia whose muscle CoQ10 concentration is considered normal. It is also unclear why children responded well to the treatment and the adults did not, considering that all of these individuals had low muscle levels of CoQ10. More research is necessary to determine whether CoQ10 levels in muscles are predictive of how well a person will respond to CoQ10 therapy.

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Darin Ingels, ND, MT (ASCP), received his bachelor’s degree from Purdue University and his Doctorate of Naturopathic Medicine from Bastyr University in Kenmore, WA. Dr. Ingels is the author of The Natural Pharmacist: Lowering Cholesterol (Prima, 1999) and Natural Treatments for High Cholesterol (Prima, 2000). He currently is in private practice at New England Family Health Associates located in Southport, CT, where he specializes in environmental medicine and allergies. Dr. Ingels is a regular contributor to Healthnotes and Healthnotes Newswire.

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