Severe Locked-In-Like Guillain-Barré's Syndrome: Dilemmas in Diagnosis and Treatment

Authors: Korinthenberg, R, Eckenweiler, M, and Fuchs, H.

Publication: Neuropediatrics; November 2020

Affiliations: Department of Neuropediatrics and Muscular Disorders, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany; Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

Abstract: Guillain-Barré’s syndrome in childhood can follow an atypical course, increasing the challenges in diagnosis and decisions regarding immunomodulatory treatment. Here, we report the case of on a 13-year-old boy with acute onset Guillain-Barré’s syndrome progressing over 40 days to a very severe, locked-in-like syndrome despite intensive immunomodulatory treatment. After a plateau phase lasting 3 months and characterized by fluctuating signs of ongoing inflammatory disease activity, we were prompted to perform repeated and maintenance immunomodulatory treatment, which resulted in a continuous and nearly complete recovery of function. Atypical features at disease onset, the severe « total » loss of all peripheral and cranial nerve function, and an apparent late response to treatment give rise to reviewing the dilemmas of diagnosis and treatment in such severe and protracted courses of Guillain-Barré syndrome.