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review

Multifocal Motor Neuropathy Review

Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 […]

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discovery

Gene defect for muscle atrophy discovered

Researchers at the ANZAC Research Institute (University of Sydney and Concord Hospital) have found the cause of a hitherto mysterious disorder causing degeneration of motor nerves that leads to severe muscle wasting in the limbs.

The disease is manifest almost exclusively in men but inherited through their mothers as carriers, a pattern that arises because the disorder is linked to a gene on the X chromosome.

Affected men cannot run properly and show loss of strength in the hands and feet becoming increasingly disabled through later life. The gene defect causes a slow, but progressive degeneration of the ends of the long motor neurons which drive the limb muscles.

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discovery

Prion protein is not all bad

A molecular Dr. Jekyll finally has a day job—as an electrical lineman. A new study suggests that the normal form of prion protein helps maintain the insulation that speeds electrical signals along nerve fibers. In its twisted Mr. Hyde form, the prion protein causes fatal brain-wasting diseases, such as mad cow disease in cattle and […]