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Locked-in syndrome in three children with Guillain-Barré syndrome
Conrado Medici1, Gabriel Gonzalez, Alfredo Cerisola
1Department of Pediatric Neurology, School of Medicine, Pereira Rossell Children's Hospital, Montevideo, Uruguay. conrado.medici@gmail.com
Insights
Locked-in syndrome in children, often caused by Guillain-Barré syndrome, presents with complete paralysis but preserved consciousness. This rare condition requires careful diagnosis and management due to potential poor outcomes.
Area of Science:
- Neurology
- Pediatrics
- Neuroimmunology
Background:
- Locked-in syndrome (LIS) is a rare neurological disorder characterized by complete paralysis of voluntary muscles while consciousness is preserved.
- While often associated with adult conditions, LIS in pediatric patients is exceptionally uncommon.
- Guillain-Barré syndrome (GBS) is an autoimmune disorder that can, in rare instances, lead to LIS in children.
Observation:
- This report details three pediatric cases of LIS secondary to GBS.
- Patients presented with acute tetraplegia, areflexia, cranial nerve deficits, and characteristic albuminocytologic dissociation in cerebrospinal fluid.
- Electrophysiologic studies revealed different subtypes of GBS, including acute motor axonal polyradiculoneuropathy and acute motor sensory axonal polyradiculoneuropathy.
Findings:
- The study highlights the rare occurrence of LIS in pediatric GBS cases.
- Clinical presentation included severe paralysis and specific neurological findings consistent with GBS.
- Neurophysiological assessments were crucial in diagnosing the underlying GBS subtypes.
Implications:
- Early recognition and diagnosis of LIS in pediatric GBS are critical for appropriate management.
- Understanding the neurophysiological underpinnings can inform prognosis and treatment strategies.
- Further research is needed to elucidate the mechanisms and optimize outcomes for children with GBS-associated LIS.
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.
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