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.