Title

Severe Pulmonary Hypertension Associated With the Acute Motor Sensory Axonal Neuropathy Subtype of Guillain-Barré Syndrome.

Publication/Presentation Date

1-1-2010

Abstract

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.

INTERVENTION: None.

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.

Volume

11

Issue

1

First Page

16

Last Page

19

ISSN

1529-7535

Disciplines

Medicine and Health Sciences | Pediatrics

PubMedID

20051787

Department(s)

Department of Pediatrics Faculty, Department of Pediatrics Residents

Document Type

Article