Progressive Weakness and Dyspnea in a 14-Year-Old Boy

Danielle D. DeCourcey, MD; Mark Wainwright, MD, PhD; Jason M. Kane, MD


April 02, 2018

The main modalities of therapy for Guillain-Barré syndrome include plasma exchange and intravenously administered immunoglobulin (IVIG). Corticosteroids have not been shown to be beneficial.[5] The American Academy of Neurology issued a practice parameter regarding immunotherapy for Guillain-Barré syndrome that concluded IVIG and plasma exchange are options for children with severe disease and should be reserved for those with the following findings:[6]

  • Rapidly progressing weakness

  • Worsening respiratory status or need for mechanical ventilation

  • Significant bulbar weakness

  • Inability to walk unaided

Several trials have demonstrated that IVIG is at least as effective as plasma exchange in the treatment of Guillain-Barré syndrome and is associated with a lower rate of complications.[7] IVIG administered at 0.4 g/kg/day for 5 days has been shown to hasten recovery and lower the relapse rate. Doses of 1 g/kg/day over 2 days have also been demonstrated to hasten recovery time, but early relapses are more prevalent.[8] The combination of plasma exchange and IVIG does not improve outcomes or shorten the duration of illness.[6]

Complications associated with Guillain-Barré syndrome include arrhythmia, sepsis, pneumonia, ileus, deep venous thrombosis and pulmonary embolism. The risk of sepsis and infection may be decreased by aggressive physiotherapy and mechanical ventilation with positive end expiratory pressure (PEEP). Administration of anticoagulant therapy and intermittent pneumatic compression devices may lower the risk of deep venous thrombosis and pulmonary embolism. Cardiac telemetry is useful to monitor for arrhythmias, which are a common cause of morbidity and mortality in Guillain-Barré syndrome. In addition, physical and occupational therapy should be initiated early and may be beneficial in helping patients to regain their baseline functional status.[9]

More than 90% of patients reach the nadir of their function within 4 weeks of the onset of symptoms, with return of normal function occurring slowly over the course of weeks to months. Most patients with Guillain-Barré syndrome achieve a full and functional recovery within 6-12 months. The clinical course of Guillain-Barré syndrome in children is shorter than it is in adults, and recovery is more complete.[10]

Upon transferring this patient to the ICU, a dialysis catheter was placed and plasmapheresis was initiated. His hypertension was controlled with a nicardipine drip. Prophylaxis for deep venous thrombosis was started. On hospital day 3, the patient had improved strength in his lower extremities and pressure support trials on the ventilator were initiated. On hospital day 6, he was extubated successfully to room air after receiving a total of 5 sessions of plasmapheresis. He was transferred to a pediatric ward 1 week after admission with intensive physical and occupational therapies. At the time of the transfer, the patient's bulbar symptoms were resolved, and his strength was 3/5 in his lower extremities and 4/5 in his upper extremities.


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