PhD defence J. (Joyce) Roodbol

Brief summary:

The Guillain-Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy that presents as a rapidly progressive flaccid paresis of the limbs. GBS is usually triggered by a preceding infection and has a typical monophasic disease course. Approximately 20% of patients may also develop respiratory failure occurs for which ventilation is necessary. GBS may also affect the cranial, sensory and autonomic nerves. GBS is a clinical diagnosis but can be supported by examination of the cerebrospinal fluid (CSF) or nerve conduction studies (NCS). Standard treatment is with either intravenous immunoglobulins or plasma exchange, but the clinical response and outcome is highly variable. GBS in children may differ from GBS in adults in various ways. First, the age may influence the clinical presentation and differential diagnosis of acute flaccid paresis (AFP) and therefore the diagnostic work-up and accuracy. Second, the type of infection preceding GBS in children is unknown and may differ from adult patients. Third, children differ from adults with GBS with respect to the respond to treatment, clinical course and outcome. Fourth, prognostic models to predict the clinical course in individual patients are currently lacking for children with GBS.  

The overall scope of the thesis was to define GBS in children with respect to the type of preceding infection, clinical presentation, diagnostic features, occurrence of delayed or missed diagnosis, clinical course and long-term outcome. In addition, the aim was to develop a clinical prognostic model to predict respiratory failure and inability to walk in individual children with GBS.