P0887 - Hiding Behind a Cough: Guillain-Barré Syndrome Masquerading as Community-Acquired Pneumonia

Introduction:
Guillain-Barré syndrome (GBS) is the most common form of acute flaccid paralysis in pediatric patients. Classically, an antecedent illness provokes an autoimmune response against the myelin sheath of peripheral nerves, leading to ascending paralysis that can cause respiratory failure. Although associated with significant morbidity and mortality, it is treatable. Here, we present a case of GBS that manifested as respiratory distress and was initially diagnosed as simple community acquired pneumonia (CAP).

Case Description:
A 16 year old male with a history of Sotos syndrome, stage III CKD, autism spectrum disorder, nonverbal status, seizure disorder, GERD, and global developmental delay presented to the emergency department with respiratory distress in the setting of three days of progressive fatigue and one day of cough. On arrival, he was afebrile, tachycardic to 132, tachypneic to 27, and hypoxic to 83%. He appeared generally unwell and was unwilling to ambulate, but moved all extremities equally. He had diminished aeration to the bases bilaterally with scattered rhonchi, and was started on BiPAP for work of breathing. Chest x-ray showed bibasilar airspace opacities. He was started on ceftriaxone for presumed CAP and was admitted to the PICU. His respiratory status deteriorated, and he was noted on hospital day two to be areflexic in his bilateral lower extremities, with voluntary movement limited to minimal hip flexion and slight movements of upper extremities. Due to cognitive impairment, he was unable to participate in a negative inspiratory force (NIF) test. On further history, his mother reported several falls prior to admission; these had initially been attributed to general illness, but were newly concerning for neurological weakness and possible GBS in the setting of areflexia. He was started on empiric IVIG and intubated for hypoxia. A lumbar puncture was performed, which demonstrated a normal differential, glucose, gram stain, and culture, with elevated protein of 93 mg/dL. MRI brain and spine showed diffuse enhancement of the cauda equina nerve roots and EMG demonstrated extensive psychomotor polyneuropathy, both consistent with GBS. The patient’s weakness improved after two rounds of IVIG, and he was successfully extubated. He also completed a 10 day course of antibiotics.

Discussion
GBS is a dangerous but potentially treatable neurological condition, and accurate diagnosis is crucial to prevent morbidity and mortality. While respiratory failure can occur due to involvement of the phrenic nerve, it classically occurs after other neurological symptoms have become obvious.

Conclusion:
This case is unusual in that respiratory distress was the chief complaint and the patient’s underlying weakness was more difficult to elucidate, possibly due to his global developmental delay. It highlights the need for a high index of suspicion for neurological etiologies in rapidly progressive respiratory distress, and the danger of anchoring bias.