OP72 – 2501: Atypical Guillain-Barré (GBS) syndrome variants – Spectrum or developing continuum of the same disease

Abstract

Objective To present very heterogeneous and complex clinical features of atypical GBS variants and overlaps between different GBS types and subtypes. Methods Clinical exam, cerebrospinal fluid (CSF) exam, ganglioside antibodies, EMG, brain and spinal MRI. Results We report 5 children at the age 13 months to 15 years with atypical presentation of GBS and GBS variants, out of 33 patients with typical GBS, treated at Department of Paediatrics in the period from 2005 to 2014. Clinical features included: bifacial and severe pharyngocervicobrachial weakness of descending type, lesion of n. VI with paresthesias of lower extremities, acute ptosis and mydriasis and incomplete Miller Fisher syndrome, bilateral dominant cervicobrachial weakness, and mild asymmetric paraparetic GBS with priapism. First cerebrospinal fluid analysis revealed normal protein content in 3/5, while increased (range 0.39–0.42 g/L) during follow up in second week of disease in 2/5. MRI of the brain and spinal cord was normal in all patients except enhancement of the cervical nerve roots in patient with pharyngocervicobrachial subtype. EMG performed in first 2 weeks showed prolonged distal latency and proximal conduction block in 3/5, inelicitable nerves and axonal loss on upper extremities in a patient with pharyngocervicobrachial subtype, absent F-waves and neural potentials in 4/5 patients. Mild decreasing (38–41 m/s) of motor conduction velocity was present in 2/5 in distal nerve segments. Ganglioside antibodies were positive in 3/5 patients. The clinical course was more severe and prolonged in a patient with descending type of pharyngocervicobrachial GBS subtype. Conclusion Spectrum of GBS presents with very variable clinical manifestations requiring reconsideration of existing diagnostic criteria for GBS in paediatric population. Appropriate diagnosis based on criteria and diagnostic guidelines are important for rational and early immunotherapy in patients with atypical GBS variants.