Locked-in syndrome in three children with Guillain-Barré syndrome.

[locked-in syndrome]

Locked-in syndrome is a rare disorder in childhood . It resembles brain death , but patients are fully conscious , and incapable of communicating because of the complete paralysis of voluntary muscles . Although it can be caused by Guillain-Barré syndrome , it is rarely reported in pediatrics . We describe three pediatric cases of locked-in syndrome in patients with Guillain-Barré syndrome presenting acute tetraplegia , areflexia , cranial nerve involvement , and albuminocytologic dissociation in the cerebrospinal fluid . Electrophysiologic studies indicated acute motor axonal polyradiculoneuropathy in one patient , and acute motor sensory axonal polyradiculoneuropathy in the other two . Most Guillain-Barré syndrome patients with locked-in syndrome demonstrate nerve inexcitability in neurophysiologic studies , poor clinical outcomes , and increased risk of sequelae .