Locked-in-like fulminant infantile Guillain-Barré syndrome associated with herpes simplex virus 1 infection

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Abstract

Introduction: Guillain-Barré syndrome (GBS) may rarely manifest as a peripheral locked-in syndrome. Methods: Clinical and instrumental features of a fulminant form of infantile GBS were assessed. Results: After 2 days of rhinitis, a 6-month-old infant was intubated in the emergency room for sudden-onset respiratory failure. Neurological examination showed generalized areflexic flaccid paralysis with no detectable interaction, which resembled a coma. Brain MRI was normal. Lumbar puncture showed pleocytosis (43 cells/mm3) and herpes simplex virus 1 (HSV1) PCR positivity. EEG showed normal sleep-wake cycles, and EMG demonstrated nerve inexcitability. Acyclovir and immunoglobulins provided no benefit. After 1 week, lumbar puncture showed albuminocytological dissociation (protein 217mg/dl). Plasmapheresis was then started, and progressive improvement occurred. At age 1 year, the child had recovered well with residual distal lower limb hyporeflexic weakness. Conclusions: A fulminant infantile GBS variant presenting as peripheral locked-in syndrome can be associated with HSV1 infection likely due to autoimmune cross-reactivity.

Original languageEnglish
Pages (from-to)140-143
Number of pages4
JournalMuscle and Nerve
Volume53
Issue number1
DOIs
Publication statusPublished - Jan 1 2016

Keywords

  • EMG
  • Guillain-Barré syndrome
  • Herpes simplex virus 1
  • Locked-in syndrome
  • Plasmapheresis

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

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