Case Presentation: A 29-year-old male patient with no past medical history was admitted initially with bilateral lower extremities (LE) weakness and right side facial paralysis. Before the onset of the symptoms, he reported that he developed round red spots with fever after being in the woods. His neurologic exam was positive for absent reflexes in both ankles and 4/5 power in both LE. Electromyogram showed diffuse neuropathic abnormality of multiple nerve roots. Head computed tomography was normal and brain magnetic resonance imaging was also negative. Lyme disease IgG/IgM antibody screen was positive but B Burgdorferi Ab IgG/IgM western blot was negative. Based on these findings, Lyme disease was considered to be the cause so he was started on IV ceftriaxone. His symptoms got slightly better and he was discharged. Shortly after that, his bilateral LE weakness started to get worse and was barely able to raise his legs. His weakness was ascending and progressed till the nipple line. He noted bilateral fascial muscle weakness and had problems chewing but no changes in swallowing, speech or breathing. Physical exam revealed normal vital signs. Neurologic exam was positive for bilateral cranial nerve VII palsy as he was unable to close his eyes, could not show teeth and could not blow cheeks. Power was 2/5 in both LE, 4/5 in both upper extremities (UE). Sensation was intact to pin prick and touch in both LE and UE. Both ankle and knee reflexes were absent (0/2) bilaterally. The rest of exam was normal. Negative inspiratory force on admission was normal at -70 cm H2O and remained stable thereafter. Vital capacity was normal at 1.5-2 L throughout his stay.

On this second presentation, lumbar puncture was done, showing markedly elevated CSF protein: 830 mg/dL, with relatively normal cell count consistent with albumino-cytologic dissociation. The diagnosis of Guillain-Barré syndrome (GBS) was made based on the whole clinical scenario and CSF findings. The patient was started immediately on IV immunoglobulin 400 mg/kg/day for 5 days and responded very well. The patient was seen 2 months later with complete resolution of his neurologic symptoms.

Discussion: GBS is an acute immune-mediated polyneuropathy affecting the peripheral nervous system. It is a syndrome of multiple variants. The most common variants are acute inflammatory demyelinating polyradiculoneuropathy, Miller Fisher syndrome, acute motor axonal neuropathy and acute sensorimotor axonal neuropathy. In most cases, GBS presents as an acute paralysis provoked by a preceding infection that may pass unnoticed. Our patient presented initially with an incomplete picture of GBS that was confounded by presence of rash and fever as well as positive Lyme antibody screen. This led to an initial misdiagnosis of neurologic Lyme disease that did not respond to antibiotic therapy. Clinicians should be made aware that the diagnosis of Lyme disease with neurologic manifestations depends on proper interpretation of a confirmatory positive western blot test.  

Conclusions: This case highlights a confounding unusual presentation of GBS as the cause of the patient’s symptoms. By considering this unusual picture, providers will have the potential to diagnose and intervene early which would ultimately benefit the patient’s outcome and decrease morbidity, mortality as well as health care costs. We emphasize that early suspicion of unusual presentation of GBS requires further evaluation and immediate initiation of proper therapy.