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. [10.1002/mus.24908]

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

R. Dilena;S. Esposito;F. Paglialonga;
2016-01

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.
EMG; Guillain-Barré syndrome; Herpes simplex virus 1; Locked-in syndrome; Plasmapheresis; Neurology (clinical); Cellular and Molecular Neuroscience; Physiology (medical); Physiology
Settore MED/38 - Pediatria Generale e Specialistica
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/369726
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