The Locked-In Syndrome refers to a patient with retained consciousness and cognition who is "locked in" by severe paralysis of voluntary muscles.
Pathogenesis: An abnormality in the descending corticospinal and/or corticobulbar pathways at or below the pons. It may be associated with a severe peripheral neuropathy.
Features of the locked-in syndrome:
(1) full consciousness and self-awareness
(2) sleep and wake cycles present
(3) severe paralysis of voluntary muscles with quadraplegia, pseudobular palsy and aphonia
(4) vertical eye movements and blinking are usually preserved sufficiently to allow for limited communication
(5) preserved auditory and visual function
(6) preserved emotions
(7) respiratory function normal
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