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Guillain-Barré Syndrome and Atypical Variants in Children: A Croatian Single Tertiary Center Experience

Aim: To depict heterogeneous clinical features of atypical Guillain-Barré syndrome (GBS) variants overlapping between different GBS types and subtypes. Methods: Retrospective analysis of data comprising neurological features, cerebrospinal fl uid (CSF) analysis, ganglioside antibody testing results, electromyography (EMG) fi ndings, brain and spinal magnetic resonance imaging (MRI) in all pediatric patients with GBS treated during a 10- year period at a tertiary center. Results: Twenty- three children were treated for GBS during the study period. Atypical variants were found in fi ve patients and included bifacial and severe pharyngocervicobrachial weakness of descending type, sixth nerve lesion accompanied with lower extremity paresthesias, sensory atactic neuropathy and facial nerve lesion, acute ptosis with mydriasis and incomplete Miller Fisher syndrome, and bilateral facial nerve paresis (one case each). Initial CSF analysis revealed mostly normal proteinlevel in atypical variants. MRI evaluation was normal in all atypical variants except for enhancement of the cervical nerve roots in a patient with pharyngocervicobrachial subtype. EMG performed in the fi rst two weeks showed prolonged distal latency and proximal conduction block in 3/5 patients, in elicitable nerves and axonal loss on upper extremities in a patient with pharyngocervicobrachial subtype, and absent F- waves and neural potentials in 3/5 patients. Slight decrease of motor conduction velocity was present in 2/5 patients in distal nerve segments. Antiganglioside antibodies were positive in 4/5 patients. Conclusion: Clinical manifestations of GBS are very variable, whereas atypical variants/overlaps are not so uncommon. This study supports the proposed hypothesis of continuous spectrum of GBS requiring reconsideration of the existing diagnostic criteria for classic GBS in pediatric population supported by recently proposed (published) diagnostic guidelines.

Guillain-Barré syndrome ; polyneuropathy ; atypical ; children

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