Locked-in state due to Epstein–Barr virus primary infection in an immunocompetent patient

Sir: Guillain–Barre syndrome (GBS) can occasionally present with acute quadriparesis and abolition of brain stem reflexes [1]. We report a fulminant presentation of GBS mimicking a locked-in state during an Epstein–Barr virus (EBV) primary infection in an immunocompetent patient. A previously healthy 59-year-old woman was hospitalized because of weakness and dysaesthesia in the extremities for 24 h. One week before admission, she had had isolated fever for 72 h which had spontaneously resolved. At admission, the physical examination showed only a symmetric weakness of the legs (strength, 4/5), and preserved deep tendon reflexes. Laboratory tests showed an increase of aminotransferase plasma concentration (3N), no inflammatory syndrome, and a complete blood count normal without atypical leukocytes. The cerebrospinal fluid was normal. Magnetic resonance imaging (MRI) of the brain and the entire spine showed no abnormality. Over the next 24 h, the patient developed a flaccid quadriparesis, hyporeflexia, diplopia, difficulty in swallowing, and respiratory failure. She required intubation and mechanical ventilation. The electromyography (EMG) showed motor and sensory abnormalities in all four limbs consistent with a severe axonal polyneuropathy. A diagnosis of GBS was made and intravenous immunoglobulin (IVIg) (0.4 g/kg/day) was started. On the 4th hospital day, the patient presented full flaccid quadriplegia and absence of brain stem reflexes, in particular dilated pupils unresponsive to bright light, complete ophthalmoplegia, and no corneal reflex. An electroencephalogram (EEG) showed symmetrical 7–8 Hz activity responsive to visual and sound stimuli. Serologic tests for HIV, B and C hepatitis, Campylobacter jejuni and anti-ganglioside antibodies were negative. The results of serologic tests for EBV are shown in Table 1. EBV was not detected by PCR in CSF. After 5 days of IVIg, corticosteroids (1 mg/kg/day) were administrated for 4 weeks. The patient recovered progressively, with ocular motricity on day 9, head movement on day 15, and motricity of the arms beginning on day 30. She was extu-