Contrary to contention that Onuf nucleus is spared in Werdnig-Hoffmann disease (WHD) as it is in ALS, we found central chromatolysis in the Onuf neurons of the sacral cord from all five WHD patients. They were less depopulated but the central chromatolysis appeared more frequently than in anterior motor neurons. In contrast, the sacral autonomic neurons were not affected in any of the five cases. This argues against the view that the Onuf nucleus is autonomic and is spared with other autonomic neurons.