Research helps physicians understand rare form of ataxia that causes patients to appear drunk at times
Scientists at Oregon Health & Science University are the first to produce an animal model for episodic ataxia. The condition causes patients to suffer bouts of extreme clumsiness where they have balance, speech and motor difficulties. The research helps scientists better understand this rare and intriguing disorder. It may also help provide valuable information for improved, targeted drugs for treatment. The research is printed in the April edition of the journal Nature Neuroscience. It was conducted in conjunction with researchers at the Portland Veterans Affairs Medical Center.
"By developing a mouse model for episodic ataxia, we now have a valuable tool to better understand and treat the disease," said James Maylie, Ph.D., a professor of obstetrics and gynecology in the OHSU School of Medicine. "We have already used this animal model to observe and learn more about cellular mechanisms behind the disease. These disease-linked cells are located in the cerebellum, a portion of the brain involved in motor coordination."
The research also helps explain how a medication commonly used to treat patients works. "Acetazolamide is often given to patients with episodic ataxia," Maylie said. "Using these mice models, we were able to establish how acetazolamide acts at a cellular level to combat the disorder, something that was previously unknown."
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