title: Locked-in-like fulminant infantile Guillain-Barré syndrome associated with herpes simplex virus 1 infection.
abstract: BACKGROUND
  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/mm(3)) 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 217 mg/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.
authors.affiliation: Clinical Neurophysiology Unit, Department of Neuroscience and Mental Health, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza, 20122, Milano, Italy.
authors.forename: Robertino
authors.initials: R
authors.lastname: Dilena
authors.affiliation: Department of Neurorehabilitation 3, Scientific Institute "E. Medea," Bosisio Parini, Lecco, Italy.
authors.forename: Sandra
authors.initials: S
authors.lastname: Strazzer
authors.affiliation: Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Susanna
authors.initials: S
authors.lastname: Esposito
authors.affiliation: Pediatric Nephrology and Dialysis Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Paglialonga
authors.affiliation: Clinical Neurophysiology Unit, Department of Neuroscience and Mental Health, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza, 20122, Milano, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Tadini
authors.affiliation: Clinical Neurophysiology Unit, Department of Neuroscience and Mental Health, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza, 20122, Milano, Italy.
authors.forename: Sergio
authors.initials: S
authors.lastname: Barbieri
authors.affiliation: Pediatric Intensive Care Unit, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Giannini
mesh_terms: D004569:Electroencephalography; D004576:Electromyography; D020275:Guillain-Barre Syndrome / Q000150:complications* / Q000821:virology*; D006561:Herpes Simplex / Q000150:complications*; D018259:Herpesvirus 1, Human / Q000472:pathogenicity*; D006801:Humans; D007223:Infant; D008297:Male
keywords: EMG; Guillain-Barré syndrome; herpes simplex virus 1; locked-in syndrome; plasmapheresis