Severe Pulmonary Hypertension Associated With the Acute Motor Sensory Axonal Neuropathy Subtype of Guillain-Barré Syndrome.
OBJECTIVE: To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome. Guillain-Barré syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain-Barré syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems.
DESIGN: Case report.
SETTING: Tertiary care pediatric intensive care unit.
PATIENT: Three-yr-old female patient.
MEASUREMENTS AND MAIN RESULTS: Serial measurements of pulmonary artery pressure. We report the case of a young girl with acute motor sensory axonal neuropathy who presented with severe cardiovascular collapse secondary to severe pulmonary hypertension. In this patient, multiple factors may have played a role in the development of pulmonary hypertension including autonomic dysfunction, hypoventilation, and immobility as a risk for thrombosis and pulmonary emboli.
CONCLUSION: It is possible that many other individuals suffering from severe forms of Guillain-Barré syndrome, especially those with significant autonomic dysfunction, may actually have undiagnosed and therefore untreated pulmonary hypertension. Therefore, it is recommended that clinicians caring for critically ill children with Guillain-Barré syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.
Published In/Presented At
Rooney, K. and Thomas, N. (2010). Severe pulmonary hypertension associated with the acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome. Pediatric Critical Care Medicine. 11 (1): e16-9. doi:10.1097/PCC. Ob013e3181b0133d.
Medicine and Health Sciences | Pediatrics
Department of Pediatrics Faculty, Department of Pediatrics Residents