Clinical Course:
48-year-old gentleman known case of moderate-severe chronic rhinosinusitis and mild asthma who initially presented to an outside hospital with a progressive 4-week history of paresthesia and weakness of all four extremities, more prominently on the right compared to the left. He reports testing positive for COVID-19 infection one month prior, for which he was treated with a 5-day course of favipiravir. He noted after taking the first dose of medication, he started to develop paresthesia involving right hand that spread to his forearm then soon developed weakness and pain as well. The paresthesia and weakness progressed to involve the right leg, left arm and left leg after a few days.
During his stay at the outside hospital, he was diagnosed with Guillain-Barre syndrome based on nerve conduction study that showed demyelinating motor neuropathy and conduction block of the right median, right ulnar, right tibial and left peroneal nerves. Additionally, he underwent an electromyogram that confirmed absence of motor nerve activity in a similar distribution. He was treated with IVIG and pulse steroid therapy for 5 days with mild improvement and was discharged afterwards. Unfortunately, his symptoms continued to worsen to the point he developed right wrist and bilateral foot drop, prompting his return back to the hospital for further management.
He underwent further testing including laboratory work up that revealed leukocytosis of 19.7 x 109/L with neutrophilia of 8.9 x 109/L and eosinophilia of 7.23 x 109/L, elevated C3 C4 levels, negative ANA, positive pANCA, positive cryoglobulin IgG, IgE of 1625 IU/mL, CRP of 135 mg/L, creatinine of 69 micromol/L, microscopic hematuria and underwent a lumbar puncture that yielded WBC of 2, high IgG, normal protein and normal glucose levels. Imaging including MRI spine that revealed a mild disc bulge at C3-C4 and C4-C5, MRI brachial plexus that revealed no brachiopathy. He started on prednisolone 60 mg daily and transferred from the outside hospital for evaluation of presumed eosinophilic granulomatosis with polyangiitis.
On admission to our facility, physical examination revealed 0/5 power of the distal right upper extremity (right wrist drop), 5/5 of the proximal right upper extremity, 4/5 power of the distal left upper extremity, 5/5 power of the proximal left upper extremity, 0/5 power of the distal left lower extremity (left foot drop), 4/5 power of the right lower extremity and marked loss of sensation in the lower extremities bilaterally up to the mid-shins in a stocking distribution.
He underwent nerve biopsy of the left sural nerve that later revealed focal angiocentric acute and chronic inflammation (figure.2). He was promptly started on mepolizumab in addition to prednisolone 60 mg in the interim. His stay was complicated by worsening severe neuropathic pain that required consultation of our pain management team. He was started on a strong pain regimen. Further progression of his neurological deficits was evidenced by a new onset right foot drop. In view of his worsening symptoms, he was started on rituximab but unfortunately there continued to be an increase of inflammatory markers despite this. It was felt that he was not responding to the treatment thus far and the decision to start on a course of IVIG with an increase of his prednisolone from 60 mg to 90 mg was made.
After these changes, he showed both biochemical and clinical improvement and was discharged with close follow up with Allergy and Immunology as an outpatient (figure.1).
He was continued on mepolizumab for 2 months and tapering dose of prednisolone but due to financial constraints, he was unable to continue on mepolizumab. In view of his clinical improvement, he was started on azathioprine to maintain remission of the disease and his disease has been stable.