Neurological disorder breakthrough

Ataxia can leave patients in a wheelchair

A vitamin treatment has produced dramatic improvements in patients with a form of the crippling neurological condition ataxia.

An eight-year-old boy with the hereditary condition who had been confined to a wheelchair was able to walk independently after the treatment.

A second patient, a 20-year-old woman, was able to work outside the home for the first time.

All of the patients improved

Dr Salvatore DiMauro, Columbia University

Hereditary ataxia is a genetic neurological disorder that affects co-ordination.

Patients have difficulty with balance, co-ordination of arms and legs and speech. Some patients also develop seizures.

The disease often causes deterioration of the cerebellum, the area of the brain that controls co-ordination.

The researchers from Columbia University, New York, discovered that some patients with hereditary ataxia have a decreased level of a substance called CoQ10 in their muscles.

CoQ10, also called ubiquinone, is a vitamin-like substance that plays a key role in the production of energy within cells.

It is naturally present in small amounts in various foods.

The researchers identified six people with hereditary ataxia with no known genetic cause.

They found that CoQ10 levels in each of the patients were 70% lower than normal.

The patients were given daily supplements of CoQ10, ranging from 300 mg to 3,000 mg.

Lead researcher Dr Salvatore DiMauro said: "All of the patients improved with the CoQ10.

"They got stronger, their ataxia improved and their seizures either stopped or happened less often."

One year on

One year after they started taking CoQ10, the patients' scores improved by an average of 25 percent on an ataxia scale measuring their balance, speech and movement.

Five of the patients were unable to walk before receiving CoQ10, but after treatment all were able to walk with some assistance, such as a rolling walker.

There are many forms of hereditary ataxia, also called hereditary spinocerebellar ataxia, or SCA.

Dr DiMauro said: "Our findings suggest that CoQ10 deficiency is a potentially important cause of some forms of familial ataxia and it should be considered when diagnosing this condition.

"Where low levels are found, treatment to replace the missing CoQ10 should be aggressive and begin early."

The research is published in Neurology, the scientific journal of the American Academy of Neurology.