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.