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Published online before print June 14, 2006, doi:10.1212/01.wnl.0000231139.26253.d0)
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Received January 31, 2006
Accepted March 27, 2006

Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy

Conrad C. Weihl MD, PhD, Anne M. Connolly MD, and Alan Pestronk MD*

From the Department of Neurology, Washington University School of Medicine, St. Louis, MO.


* To whom correspondence should be addressed. E-mail: pestronka{at}neuro.wustl.edu.

Abstract-- Spinal muscular atrophy (SMA) is a recessively inherited motor neuron disease caused by deficient survival motor neuron (SMN) protein. Valproate increases SMN protein in vitro by increasing transcription of SMN2 genes. The authors treated seven adult patients with SMA type III/IV with valproate for a mean duration of 8 months. The treated patients with SMA had increased quantitative muscle strength and subjective function. Further trials of valproate treatment for SMA type III/IV are warranted.




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