Pain and Proximal Leg Weakness: An Atypical Presentation of Guillan-Barré Syndrome

Guillain-Barré syndrome (GBS) is an acute, rapidly progressive immune-mediated polyradiculoneuropathy that is a neurologic emergency given the risk of mortality associated with respiratory failure. This report adds to the growing literature on atypical early presentations of GBS.  A 63-year-old man initially presented to the ED with a two-day history of diffuse myalgias and intermittent 'sharp zingers' lasting a few seconds. These started in his hands and quickly spread to his upper and lower extremities. He had recently recovered from an upper respiratory infection. Lab workup was notable for a mild elevation in creatine kinase (CK), and examination revealed no focal deficits. He was discharged home with a diagnosis of post-viral myalgias. The following day, he developed worsening back and leg pain, lower extremity weakness, and increased work of breathing and was readmitted to the hospital. Workup demonstrated areflexia, isolated hip flexor weakness (4/5), and diminished sensation to pin in distal extremities with no upper motor neuron signs. The patient was clinically diagnosed with GBS and started on intravenous immunoglobulin (IVIG) with improvement. A lumbar puncture (LP) subsequently confirmed GBS with cytoalbuminologic dissociation with CSF of 239 mg/dL (normal range: 15 to 60 mg/dL) and normal WBC count. The patient's hospital stay was complicated by the syndrome of inappropriate antidiuretic hormone secretion (SIADH). A challenge this case presented was the atypical description of sensory symptoms ('sharp zings' instead of the typical numbness/tingling) and the proximal rather than distal leg weakness. Patients who present with sensory and/or motor deficits require an exam including reflexes and a sensory pin exam to evaluate for GBS given the variable presentation of this condition.