Case Presentation:
A 64 year-old woman, with history of essential hypertension and hypothyroidism, presented to her primary care physician’s office with cold like symptoms and dry cough for 2 weeks associated with mild numbness in her fingers. She was clinically diagnosed with acute sinusitis and was prescribed levofloxacin. Few hours after she received the first dose of levofloxacin, she developed progressively worsening tongue swelling, dysphagia and difficulty breathing. The patient was evaluated in the ER and was diagnosed with angioedema. She received intravenous (IV) corticosteroid, antihistaminic and H2-blocker, but her symptoms failed to improve with subsequent worsening of her respiratory status that required intubation and she was transferred to ICU. She was treated initially for acute upper airway obstruction due to angioedema that seemed to improve clinically so it was decided to extubate her but the patient had increased respiratory distress with minimal air movement. The patient was then reintubated using the glide scope. Surprisingly, the epiglottis appeared normal and the glottis was open. A CXR showed evidence of right middle and lower lobes volume loss. A bronchoscopy was performed that showed mucus plugging involving the right bronchus intermedius, middle and lower lobes bronchi with successful suctioning. A thorough evaluation to look for other causes was initiated given the unexpected course with angioedema. Repeat physical exam was normal including detailed neurological exam with the exception of mildly decreased proximal muscle power in both upper extremities (4/5) which was not present on initial evaluation. Workup including blood work, CT head, MRI brain and spine were all normal. Nerve conduction studies of both upper extremities showed demyelinating polyneuropathy with extensive work up for plolyneuropathy including Lyme antibody screen, West Nile virus, serum protein electrophoresis and heavy metal screen, all were negative as well. Lumbar puncture was performed and CSF analysis showed evidence of albuminocytologic dissociation. The diagnosis of Guillain-Barré syndrome was made based on the clinical scenario and CSF finding. The patient responded well to IV immunoglobulin with successful extubation.
Discussion:
Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy affecting the peripheral nervous system. It is a syndrome with a number of variants. In most cases, GBS presents as an acute paralysis provoked by a preceding infection. Our case is unique as the patient presented initially with acute upper airway obstruction that was likely due to her bulbar palsy in addition to later proximal upper extremity muscle weakness. This was the sole presentation while she lacked the two most common features of GBS namely distal weakness and hyporeflexia. This case highlights a rare atypical presentation of GBS that led to initial misdiagnosis with subsequent delayed management. Clinicians should be aware that angioedema should respond to medical therapy within hours to a few days. Failure to see this response should direct the attention to look for other causes of upper airway obstruction.
Conclusions:
Considering this unusual presentation of GBS as the cause of the patient’s symptoms, providers will have the potential to diagnose and intervene early that ultimately benefit the patient’s outcome. We emphasize that early recognition of atypical presentations of GBS requires further evaluation.