Guillain‐Barre syndrome presenting as epiglottitis in a child

A previously healthy five-year-old boy presented to the emergency department with respiratory distress and drooling. His medical history was unremarkable except for symptoms of an upper respiratory tract infection five days earlier. Upon arrival at the emergency room, the child was lethargic, leaning forward, and in severe distress. Stretch reflexes were absent in the upper and lower extremities. His temperature was normal. The child was immediately taken to the operating room for emergency intubation with a presumptive diagnosis of epiglottitis. Laryngoscopy and bronchoscopy revealed a large pool of secretions in the postcricoid area and pyriforms, bilateral vocal cord paralysis, and tracheobronchial aspiration. Postoperatively, an electromyography (EMG) showed no motor or sensory response in the upper and lower extremities. Concentric needle examination of the right tibialis anterior muscle revealed normal insertional activity, no motor unit potential, and no recruitment of motor unit. These findings were consistent with the diagnosis of severe Guillain-Barre syndrome (GBS) with some demyelination and possibly some axonal damage. Two days later, the child had progressed to flaccid quadriplegia and facial dysplegia, and he had lost all reflexes except for the preservation of a weak brachioradialis reflex. He was alert and would respond by blinking and moving his eyes. The child was treated with pantoprazole, gabapentin, diphenhydramine, and a five-day course of intravenous immunoglobulin G (IVIg). A tracheostomy was performed after two weeks of intubation, and the child was hospitalized for six weeks. Three months after his discharge from the hospital, he was gaining strength, was decannulated, and went back to school part-time. One year later, he was walking normally and starting to run. This case report was approved by the Montreal Children's Hospital Institutional Review Board. GBS is an acute polyneuritis characterized by a rapidly progressing weakness and areflexia. It is the most common cause of acute-onset flaccid paralysis in children, with an incidence of 0.8 per 100,000 in children younger than 15 years.1 One third of childhood cases occur before the age of three years, and 73 percent of children suffering from GBS will initially present with weakness.2 Although onset of weakness is most frequently distal, 15 percent to 20 percent of children will initially present with proximal weakness. Cranial nerves are involved in approximately 45 percent of affected children, making it a more common occurrence than in adults.3 Maximal deficit will be reached within two weeks of onset of symptoms in about 80 percent of children and severity can vary from mild bilateral weakness to quadriplegia.3 Although GBS is a clinical diagnosis, it is characterized by elevated protein content in the cerebrospinal fluid without pleocytosis. However, this sign cannot be relied upon for diagnostic purposes because the elevation in protein content may be delayed for up to one week after onset of symptoms.3 Neurophysiological studies reveal slowing of nerve conduction attributable to demyelination, whereas EMG reveals loss of motor unit action potential.3 Although the mainstay of treatment is supportive, children with severe disease who cannot walk unaided can benefit from IVIg treatment to shorten recovery time. However, this treatment modality does not lessen the severity of the disease, and children who are quadriplegic and/or require mechanical ventilation do not seem to benefit from IVIg.4 Mechanical ventilation will be required in approximately 15 percent of patients suffering from GBS.5 Children with GBS tend to recover more rapidly and have more complete recovery than adults.3 More than 90 percent of children recover fully, and most children with incomplete recovery will have minimal residual deficits and will be able to walk unaided.4 Factors associated with poor prognosis include advanced age, fulminance, severity, presence of other significant illness, and primary axonopathy. The mortality rate of 1 percent to 2 percent in children suffering from GBS is attributable to respiratory failure and autonomic dysfunction.2 GBS is the most common cause of acute onset of paralysis in children. This diagnosis should be suspected in children with acute respiratory distress, vocal cord paralysis, and aspiration who also have associated neurological findings. Optimal management and supportive care in the acute stage are crucial, with severe cases requiring a tracheostomy and ventilatory support. Melanie Duval, participated in data collection and analysis, wrote manuscript, and approved final version; Sam J. Daniel, participated in design and interpretation of data, reviewed manuscript, and approved final version. Competing interests: Sam J. Daniel, speaker: Alcon, Abbott, Schering; research grant: Alcon. Sponsorships: None.

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