Response

Abstract

Regarding the remarks by Drs. Wang and Zhu on our report about three patients with Guillain-Barré syndrome presenting with locked-in syndrome, we offer two comments. First of all, Drs. Wang and Zhu considered locked-in syndrome a consequence of brain damage caused by a brainstem lesion in the central nervous system. By definition, locked-in syndrome is a state in which the patient is de-efferented, with paralysis of the four limbs and cranial nerves. Although it resembles coma or brain death, insofar as patients are incapable of communicating or responding to stimuli because of a complete paralysis of voluntary movements, they are not unconscious [ [1] Posner J. Saper C. Schiff N. Plum F. Pathophysiology of signs and symptoms of coma. in: Plum and Posner’s diagnosis of stupor and coma. 4th ed. Oxford University Press, New York2007: 3-37 Google Scholar ]. Most frequently, locked-in syndrome is attributable to an interruption of the corticospinal and corticobulbar tracts as a consequence of ischemic, hemorrhagic, traumatic, tumoral, or infectious brainstem lesions, usually in the ventral region of the pons, proximal to the reticular formation [ [2] Bruno M. Schnakers C. Damas F. et al. Locked-in syndrome in children: Report of five cases and review of the literature. Pediatr Neurol. 2009; 41: 237-246 Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar ]. In severe cases, patients with Guillain-Barré syndrome can become completely de-efferented [ [1] Posner J. Saper C. Schiff N. Plum F. Pathophysiology of signs and symptoms of coma. in: Plum and Posner’s diagnosis of stupor and coma. 4th ed. Oxford University Press, New York2007: 3-37 Google Scholar ] because of the compromise of all cranial and limb nerves. Locked-In Syndrome in Three Children With Guillain-Barré SyndromePediatric NeurologyVol. 46Issue 4PreviewWe read with interest the case report by Dr. Medici et al. [1], in which locked-in syndrome owing to Guillain-Barré Syndrome was diagnosed in three patients. However, we should raise some concerns about the diagnosis of locked-in syndrome in Guillain-Barré syndrome. Here we refer to the first case as an example to argue against their diagnosis. Full-Text PDF