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.
Locked-in-like fulminant infantile Guillain-Barré syndrome associated with herpes simplex virus 1 infection / R. Dilena, S. Strazzer, S. Esposito, F. Paglialonga, L. Tadini, S. Barbieri, A. Giannini. - In: MUSCLE & NERVE. - ISSN 0148-639X. - 53:1(2016 Jan), pp. 140-143.
|Titolo:||Locked-in-like fulminant infantile Guillain-Barré syndrome associated with herpes simplex virus 1 infection|
|Parole Chiave:||EMG; Guillain-Barré syndrome; Herpes simplex virus 1; Locked-in syndrome; Plasmapheresis; Neurology (clinical); Cellular and Molecular Neuroscience; Physiology (medical); Physiology|
|Settore Scientifico Disciplinare:||Settore MED/38 - Pediatria Generale e Specialistica|
|Data di pubblicazione:||gen-2016|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1002/mus.24908|
|Appare nelle tipologie:||01 - Articolo su periodico|