Guillain-Barre syndrome is an acute, autoimmune polyradiculoneuropathy that
improves with immune-modulating treatment if instituted early in the illne
ss. Preliminary diagnosis relies on the clinician's recognition of the typi
cal symptoms and signs as supporting evidence of the illness, such as nerve
conduction studies, which may not be available emergently. We report eight
children with Guillain-Barre syndrome in whom the initial presentation was
atypical and suggested a primary central nervous system illness. In these
patients, the predominant clinical symptoms included drowsiness, headache,
irritability, and meningismus, although the classic features of Guillain-Ba
rre syndrome (weakness, hyporeflexia) were also present. The atypical prese
ntation caused delay in diagnosis in some cases. It is important to recogni
ze this variant of pediatric Guillain-Barre syndrome to ensure expeditious
diagnosis and treatment.