We report 11 patients with the locked in syndrome (LIS). The functional outcome was good in four patients with notable motor recovery, but motor deficit remained seriously disturbed in seven patients. All of the patients regained some distal control of finger and toe movements, often allowing functional use of a digital switch. The independence thereby gained is worthwhile, in some patients allowing environment control, communication by means of a computer, and electric wheelchair ambulation. When motor recovery occurs, the progression is disto-proximal with dramatic axial hypotonia. In five patients clinical insomnia was noted and polysomnography showed a reduction of REM sleep. The implications of systems other than the pyramidal tracts in the physiopathology of LIS are discussed.