Locked‐in–like fulminant infantile Guillain–Barré syndrome associated with herpes simplex virus 1 infection. Issue 1 (1st October 2015)

• Record Type: Journal Article
• Authors: Dilena, Robertino; Strazzer, Sandra; Esposito, Susanna; Paglialonga, Fabio; Tadini, Laura; Barbieri, Sergio; Giannini, Alberto
• Is Part Of: Muscle & nerve. Volume 53:Issue 1(2016)
• Page Start: 140
• Page End: 143
• Publication Date: 2015-10-01
• Subjects: EMG -- Guillain–Barré syndrome -- herpes simplex virus 1 -- locked‐in syndrome -- plasmapheresis; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.24908
• Languages: English
• ISSNs: 0148-639X

Abstract

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/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. Muscle Nerve 53 : 140–143, 2016