Case Report
A previously healthy 60-year-old man with pectus excavatum was brought in by ambulance for a 2-day history of rapidly progressive bilateral ascending weakness, bilateral paresthesia of the hands and feet, difficulty standing, and low back pain. Three days prior to presentation, he tested positive for COVID-19 and reported myalgias, fever and nausea. He was unvaccinated against COVID-19. Neurologic examination revealed weakness in neck extension, neck flexion, and leg extension. Furthermore, areflexia and flaccid paralysis were noted in all four extremities. No bulbar or respiratory symptoms were noted. Patient denied sensation below the level of the neck. The following day, the patient’s strength was 2/5 in the bilateral upper extremities, and 2/5 in the bilateral lower extremities. Additionally, there was mute plantar response bilaterally. He had length-dependent loss of temperature and pain in the bilateral lower extremities and hands. Absent proprioception at toes, ankles, and knees with sparing of the proximal interphalangeal joints was noted. Magnetic resonance imaging (MRI) of the spine with contrast was performed which revealed diffuse enhancement with thickening of the cauda equina nerve roots, mild enhancement of lower thoracic and few cervical nerve roots consistent with GBS. Lumbar puncture (LP) was significant for protein 295, white blood cell count 27, red blood cell count 1, and glucose 54 (Table 1).
Electromyography (EMG) performed several days later revealed severe axonal sensorimotor neuropathy. Differential diagnosis included GBS, myelitis, tick paralysis, toxin exposure, and arboviruses. Myelitis was ruled out based on the lack of motor and sensory symptoms localized to one or more contiguous spinal cord segments, as well as lack of MRI findings consistent with the diagnosis. Lyme disease was ruled out with negative Lyme serology and lack of recent travel to a tick infested territory or lack of rash or lesion. Toxin associated exposure was ruled out given no history of reported toxin exposure. Arbovirus exposure was ruled out on the basis of this case occurring in the winter season.
Given the rapidly progressive bilateral ascending weakness; sensory loss greater in the lower extremities compared to upper extremities; severe low back pain; and EMG, LP, and MRI findings, a presumptive diagnosis of GBS was made. The patient was started on plasma exchange (PLEX) treatment and corticosteroids for GBS. Despite medical management, his clinical condition deteriorated during the hospital course with hypotension, bradycardia and respiratory distress which was presumed to be secondary to dysautonomia and neuromuscular weakness. The patient was intubated and managed in the intensive care unit (ICU), followed by the neurological critical care service. On hospital day 12, the patient began to desaturate to 86%, developed fever at 39.1 degrees Celsius. The hospital course was complicated by sepsis from aspiration pneumonia requiring vasopressin and phenylephrine and treated with piperacillin, tazobactam, and vancomycin, which was later escalated to linezolid.
The patient subsequently developed acute respiratory distress syndrome secondary to bacterial pneumonia. Bronchial culture grew methicillin resistant staphylococcus aureus (MRSA). Therapeutic bronchoscopy was performed for MRSA pneumonia with poor secretion clearance. Worsening hypoxia warranted BIPAP, and he developed acute kidney failure requiring continuous renal replacement therapy. Repeat neurologic examination on hospital day showed no improvement: patient remained areflexic, flaccid quadriplegic, and with absent cough/gag reflex. On hospital day 29, computerized tomography (CT) of the chest showed persistent consolidation with evidence of engulfed emphysema versus necrosis of the right upper lung. The patient developed persistent fevers, vasodepressor dependent hypotension and lymphocytosis and was started on empiric cefepime for suspected septic shock. Interventional pulmonology attempted to perform a bronchoscopic assisted percutaneous tracheostomy tube placement, however the procedure was aborted due to hypoxia in the setting of mucus plugging. Subsequently, the patient required a surgical tracheostomy, and the patient was discharged to a long-term acute care hospital for continued management and ventilator weaning.