Autor(es): Dilena Robertino; Strazzer Sandra; Esposito Susanna; Paglialonga Fabio; Tadini Laura; Barbieri Sergio; Giannini Alberto

Resumo: Guillain-Barré syndrome (GBS) may rarely manifest as a peripheral locked-in syndrome. Clinical and instrumental features of a fulminant form of infantile GBS were assessed. 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. 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.

Palavras-Chave: EMG, Guillain-Barré syndrome, herpes simplex virus 1, locked-in syndrome, plasmapheresis

Imprenta: Muscle & Nerve, v. 53, n. 1, p. 140-143, 2016

Identificador do objeto digital: 10.1002/mus.24908

Descritores: Guillain-Barre Syndrome - Cell ; Guillain-Barre Syndrome - Clinical examination ; Guillain-Barre Syndrome - Virus

Data de publicação: 2016