Neuropediatrics 2013; 44 - FV11_01
DOI: 10.1055/s-0033-1337709

Central nervous system demyelination complicating Guillain-Barré syndrome

M Häusler 1, K Claeys 2, M Schoberer 1, V Busch 3, K Rostásy 4, K Nolte 5
  • 1Kinder-und Jugendmedizin, UK/RWTH Aachen, Aachen, Germany
  • 2Department of Neurology, Institute of Neuropathology, Aachen, Germany
  • 3Kinderklinik und Poliklinik der TU München, München, Germany
  • 4Departement of Pediatrics I, Division of Pediatric Neurology, Medical University Innsbruck, Austria
  • 5Institut für Neuropathologie, UK Aachen, Aachen, Germany

Aims: Additional peripheral nervous system (PNS) demyelination was reported in 22 to 33% of the patients with central nervous system (CNS) demyelination. The prevalence and clinical characteristics of CNS demyelination among patients with Guillain-Barré syndrome (GBS), in contrast, is unknown.

Methods: We retrospectively studied 64 adults and 16 children with GBS for clinical and/or magnetic resonance imaging (MRI) signs of additional CNS demyelination.

Results: MRI studies were available from 11 children. Demyelination was present in three out of nine brain MRI studies and one out of nine spinal MRI studies. Analysis of 9 brain and 19 spinal MRI studies deriving from 24 adults revealed one male with combined supra- and infratentorial CNS demyelination. The child and the adult with combined CNS and PNS involvement had presented with severe encephalopathy, including impaired consciousness (n = 2) and seizures (n = 1). Treatment included steroids (n = 1), immunoglobulins (n = 2), and plasmapheresis (n = 2). In the boy, physiotherapy and long-term intravenous methylprednisolone pulse therapy established slow continuous amelioration. After 18 months, he still shows moderate distal muscle weakness.

None of the remaining 63 adults and 15 children developed encephalopathic signs or seizures, so that symptomatic CNS demyelination seems rare among patients with GBS.

In the severely affected child, acute M. pneumoniae infection was found, whereas autoantibodies to gangliosides and to myelin oligodendrocyte glycoprotein were not detected. Analysis of a sural nerve biopsate was in line with combined peripheral demyelination plus secondary axonal degeneration.

Conclusion: Combined CNS and PNS demyelination expands the spectrum of demyelinating disorders and should be suspected in patients with GBS developing encephalopathic symptoms. The distinct mechanism may differ from classical GBS or ADEM as autoantibodies typically detected in demyelinating CNS disorders were not found. Further studies should focus on autoantibodies directed to myelin epitopes shared by Schwann cells (PNS) and oligodendrocytes (CNS).