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Abnormal brain circuits may prevent movement disorder
Date:8/5/2009

e it hard to process sequential information. Nonetheless, mutation carriers have a characteristic circuit disorder involving a motor system that is revved up and idling at high speed, making it difficult to integrate the information needed to plan movements and to learn new motor skills. It was not suspected that these otherwise healthy individuals had such difficulties since doctors only saw those who presented with the uncontrollable movements.

While their brains show the same abnormal network, only approximately 30% of people who carry the mutated gene called DYT1 will develop the involuntary movements that can prevent them from living a normal life, according to Dr. Eidelberg.

The puzzle was why.

In the latest study, the new advances in diffusion imaging allowed them to see something for the first time. They saw that there were two places along the motor pathway that seemed to stop the flow of neural signals from one part of the circuit to the other. Those with only one lesion in the circuit developed the debilitating movements and those with two lesions did not. "There is something about this second lesion that is protective," the authors concluded. "We found a consistent cerebellar pathway problem in all DYT1 carriers. When we went back and looked at those without symptoms, we saw that they had an additional lesion downstream in the portion of the pathway connecting directly to the motor cortex." This second area of pathway disruption abrogated the effects of the first lesion.

Normally, the cerebellum (a region that controls movement) puts the breaks on the motor cortex by potentiating inhibition at the cortical level. It is likely that mutation carriers have a developmental problem in the flow of neural signals along this circuit such that the brain can't inhibit an unwanted movement. With the second pathway lesion, Dr. Eidelberg explained, "the flow is shut off and the abnormal activity stops."


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Contact: Jamie Talan
jtalan@nshs.edu
516-562-1232
North Shore-Long Island Jewish (LIJ) Health System
Source:Eurekalert

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