Locked-In Syndrome in Three Children With Guillain-Barré Syndrome

• Published in: Pediatric neurology Vol. 45; no. 2; pp. 125 - 128
• Main Authors: Medici, Conrado, MD, Gonzalez, Gabriel, MD, Cerisola, Alfredo, MD, Scavone, Cristina, MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.08.2011; Elsevier
• Subjects: Biological and medical sciences; Brain; Cerebrospinal fluid; Child; Child, Preschool; Children; Complications; Cranial nerves; Electroencephalography; Female; Guillain-Barre syndrome; Guillain-Barre Syndrome - complications; Guillain-Barre Syndrome - diagnosis; Guillain-Barre Syndrome - physiopathology; Humans; Infant; Male; Malformations of the nervous system; Medical sciences; Muscles; Neurology; Paralysis; Pediatrics; Quadriplegia - complications; Quadriplegia - diagnosis; Quadriplegia - physiopathology; Human; Nervous system diseases; Locked-in syndrome; Peripheral nerve disease; Child; Guillain-Barré syndrome; Inflammatory disease
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2011.03.005

Abstract 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.